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==Differentiating neck masses from other Diseases==
==[[Schwannoma overview|Overview]]==
Neck masses must be differentiated from [[Congenital disorder|congenital abnormalities]], [[Inflammation|inflammatory]], and [[malignant]] lesions.  
Schwannomas are benign tumors of Schwann cell origin and are the most common tumor of peripheral nerves, including cranial nerves.  


{|
==Classification ==
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Category
 
! colspan="2" rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
Schwannoma may be classified according to pathology into 4 subtypes: conventional schwannoma, cellular schwannoma, plexiform schwannoma, and melanotic schwannoma.<ref name="pmid19574706">{{cite journal| author=Touzri RA, Errais K, Zermani R, Benjilani S, Ouertani A| title=Schwannoma of the eyelid: apropos of two cases. | journal=Indian J Ophthalmol | year= 2009 | volume= 57 | issue= 4 | pages= 318-20 | pmid=19574706 | doi=10.4103/0301-4738.53063 | pmc=2712707 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19574706  }} </ref><ref name="pmid27018477">{{cite journal| author=Sharma S, Rai G| title=Schwannoma (Neurilemmoma) on the Base of the Tongue: A Rare Clinical Case. | journal=Am J Case Rep | year= 2016 | volume= 17 | issue= | pages= 203-6 | pmid=27018477 | doi=10.12659/ajcr.897063 | pmc=4815988 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=27018477  }} </ref><ref name="pmid19130068">{{cite journal| author=Cohen M, Wang MB| title=Schwannoma of the tongue: two case reports and review of the literature. | journal=Eur Arch Otorhinolaryngol | year= 2009 | volume= 266 | issue= 11 | pages= 1823-9 | pmid=19130068 | doi=10.1007/s00405-008-0907-2 | pmc=2758150 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19130068  }} </ref>
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Benign/
Malignant
! colspan="6" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Clinical manifestation
! colspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Paraclinical findings
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
|-
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |History
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Symptoms
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Signs
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab findings
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! rowspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Pain
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Dysphagia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mass exam
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Others


{| class="wikitable"
|+
! colspan="2" |Classification
|-
|-
! rowspan="5" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Neoplasm
|'''Pathology'''
! rowspan="5" align="center" style="background:#DCDCDC;" |[[Salivary gland neoplasm]]
|
! align="center" style="background:#DCDCDC;" |[[Pleomorphic adenoma]]<ref name="pmid22190789">{{cite journal |vauthors=Debnath SC, Adhyapok AK |title=Pleomorphic adenoma (benign mixed tumour) of the minor salivary glands of the upper lip |journal=J Maxillofac Oral Surg |volume=9 |issue=2 |pages=205–8 |date=June 2010 |pmid=22190789 |pmc=3244097 |doi=10.1007/s12663-010-0052-5 |url=}}</ref><ref name="pmid29845358">{{cite journal |vauthors=Kato H, Kawaguchi M, Ando T, Mizuta K, Aoki M, Matsuo M |title=Pleomorphic adenoma of salivary glands: common and uncommon CT and MR imaging features |journal=Jpn J Radiol |volume=36 |issue=8 |pages=463–471 |date=August 2018 |pmid=29845358 |doi=10.1007/s11604-018-0747-y |url=}}</ref>
*Conventional schwannoma
| align="left" style="background:#F5F5F5;" |
*Cellular schwannoma
* [[Benign]]
*Plexiform schwannoma
| align="left" style="background:#F5F5F5;" |
*Melanotic schwannoma
* More common in females
* [[Incidence]] increase with [[age]]
* [[Incidence]]: 2-3.5 cases per 100,000 [[population]]
| align="left" style="background:#F5F5F5;" |
* History of [[swelling]]
* [[Dysphagia]]
* [[Hoarseness]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | +
| align="left" style="background:#F5F5F5;" |
* Palpable [[mass]] of deep [[lobe]] of [[parotid gland]]
* Firm
* Mobile
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
* Proliferation of [[epithelial cells]] and [[stromal]] [[matrix]] in the [[ducts]]
| align="left" style="background:#F5F5F5;" |
* [[MRI]]: Homogenous on T1
* Abundant myxochondroid [[stroma]] on T2
| align="left" style="background:#F5F5F5;" |
* [[Biopsy]]
| align="center" style="background:#F5F5F5;" | −
|-
! align="center" style="background:#DCDCDC;" |[[Warthin's tumor]]<ref name="pmid24376295">{{cite journal |vauthors=Chulam TC, Noronha Francisco AL, Goncalves Filho J, Pinto Alves CA, Kowalski LP |title=Warthin's tumour of the parotid gland: our experience |journal=Acta Otorhinolaryngol Ital |volume=33 |issue=6 |pages=393–7 |date=December 2013 |pmid=24376295 |doi= |url=}}</ref><ref name="urlWarthin tumor | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program">{{cite web |url=https://rarediseases.info.nih.gov/diseases/8569/warthin-tumor |title=Warthin tumor &#124; Genetic and Rare Diseases Information Center (GARD) – an NCATS Program |format= |work= |accessdate=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Benign]]
| align="left" style="background:#F5F5F5;" |
* [[Male]] to female ratio: 4:1
* More common in people aged 60-70 years old
| align="left" style="background:#F5F5F5;" |
* History of [[Swelling|swollen]] [[salivary gland]]
* [[Jaw]] pain
* [[Tinnitus]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | +
| align="left" style="background:#F5F5F5;" |
* Non tender
* Mobile
* Firm
* [[Solitary]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
* [[Papillae]]
* [[Fibrous]] [[capsule]]
* [[Cystic]] spaces
| align="left" style="background:#F5F5F5;" |
*[[CT|Neck CT]]: [[Cystic]] lesion posteriorly within the [[parotid gland]]
*[[MRI]]: B/L heterogeneous lesions
| align="left" style="background:#F5F5F5;" |
* [[Biopsy]]
| align="center" style="background:#F5F5F5;" | −
|-
! align="center" style="background:#DCDCDC;" |[[Oncocytoma]]
<ref name="pmid277220032">{{cite journal |vauthors=Chen B, Hentzelman JI, Walker RJ, Lai JP |title=Oncocytoma of the Submandibular Gland: Diagnosis and Treatment Based on Clinicopathology |journal=Case Rep Otolaryngol |volume=2016 |issue= |pages=8719030 |date=2016 |pmid=27722003 |pmc=5045990 |doi=10.1155/2016/8719030 |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Benign]]
| align="left" style="background:#F5F5F5;" |
*Race: [[Caucasian pop|Caucasian]] patients predilection
*Gender: No gender preference
*Age: 50–70 years
| align="left" style="background:#F5F5F5;" |
*Growing [[palpable]] painless mass
*[[Facial swelling]]
*[[Lymphadenopathy]] (if transformed to malignant)
| align="center" style="background:#F5F5F5;" | ±
| align="center" style="background:#F5F5F5;" | ±
| align="left" style="background:#F5F5F5;" |
* [[Firm]], multilobulated
* Mobile [[mass]]
| align="left" style="background:#F5F5F5;" |
*Normal
*Redness
*[[Swelling]]
*Skin [[ulceration]]
| align="left" style="background:#F5F5F5;" |
*Normal
*[[Anemia]]
| align="left" style="background:#F5F5F5;" |
* [[Epithelial cells]] with [[eosinophilic]]
* Granular [[cytoplasm]]
* Rich in [[mitochondria]]
| align="left" style="background:#F5F5F5;" |
*CT:
**Isodense expansive mass
**Enhancement after intravenous contrast
**Hypodense areas
*[[MRI]]:
**Isodensties on T1
**Mass is hyperintense on T2
**Enhancement on contrast
| align="left" style="background:#F5F5F5;" |
* [[Biopsy|Incisional biopsy]] and [[histopathological]] examination
| align="center" style="background:#F5F5F5;" |-
|-
! align="center" style="background:#DCDCDC;" |[[Monomorphic adenoma]] <ref name="pmid10889498">{{cite journal |vauthors=Kim KH, Sung MW, Kim JW, Koo JW |title=Pleomorphic adenoma of the trachea |journal=Otolaryngol Head Neck Surg |volume=123 |issue=1 Pt 1 |pages=147–8 |date=July 2000 |pmid=10889498 |doi=10.1067/mhn.2000.102809 |url=}}</ref><ref name="pmid24431845">{{cite journal |vauthors=Pramod Krishna B |title=Pleomorphic Adenoma of Minor Salivary Gland in a 14 year Old Child |journal=J Maxillofac Oral Surg |volume=12 |issue=2 |pages=228–31 |date=June 2013 |pmid=24431845 |pmc=3681990 |doi=10.1007/s12663-010-0125-5 |url=}}</ref><ref name="pmid30546932">{{cite journal |vauthors=Kessler AT, Bhatt AA |title=Review of the Major and Minor Salivary Glands, Part 2: Neoplasms and Tumor-like Lesions |journal=J Clin Imaging Sci |volume=8 |issue= |pages=48 |date=2018 |pmid=30546932 |pmc=6251244 |doi=10.4103/jcis.JCIS_46_18 |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Benign]] or [[malignant]]
| align="left" style="background:#F5F5F5;" |
*Age: 26-76 years
*Rare in children
*Gender: No predilection
| align="left" style="background:#F5F5F5;" |
*Growing [[palpable]] painless [[mass]] on jaw or in [[oral cavity]]
*[[Facial swelling]]
*[[Lymphadenopathy]] (if transformed to [[malignant]])
*[[Ulceration|Pain and ulceration]] (in later stage)
| align="center" style="background:#F5F5F5;" | ±
| align="center" style="background:#F5F5F5;" | ±
| align="left" style="background:#F5F5F5;" |
* [[Nodular]]
* Fluctuant [[swelling]]
| align="left" style="background:#F5F5F5;" |
*Normal
*Redness
*Skin [[ulceration]]
*May have [[lymphadenopathy]]
| align="left" style="background:#F5F5F5;" |
* Normal
| align="left" style="background:#F5F5F5;" |
*Straw colored fluid on [[aspiration]]
| align="left" style="background:#F5F5F5;" |
* [[Ultrasound]]:
**Used to [[biopsy]] the [[lesion]]
**May show cystic an solid components
* [[Computed tomography|CT:]]
**useful for [[lesions]] with [[calcification]] and venous phleboliths
* [[Magnetic resonance imaging|MRI:]]
**Test of choice
**Differentiate [[benign]] from [[malignant]]
**Defines [[tumor]] extent
**Shows perineural spread
| align="left" style="background:#F5F5F5;" |
* [[Biopsy|Incisional biopsy]] and [[Histopathological|histopathological examination]]
| align="center" style="background:#F5F5F5;" |-
|-
! align="center" style="background:#DCDCDC;" |[[Mucoepidermoid carcinoma]]
<ref name="pmid21243374">{{cite journal |vauthors=Chenevert J, Barnes LE, Chiosea SI |title=Mucoepidermoid carcinoma: a five-decade journey |journal=Virchows Arch. |volume=458 |issue=2 |pages=133–40 |date=February 2011 |pmid=21243374 |doi=10.1007/s00428-011-1040-y |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Malignant]]
| align="left" style="background:#F5F5F5;" |
*Age: Mean age of 59
*Female predilection
| align="left" style="background:#F5F5F5;" |
*Painlesss [[mass]]
*[[Swelling]] in [[oral cavity]]
*[[Lymphadenopathy]]
| align="center" style="background:#F5F5F5;" | ±
| align="center" style="background:#F5F5F5;" | ±
| align="left" style="background:#F5F5F5;" |
* [[Cystic]] and [[Mass|solid mass]]
| align="left" style="background:#F5F5F5;" |
* May have [[lymphadenopathy]]
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
* [[Gross examination|Gross findings]]:
**Firm
**Tan-white to yellow
**Bosselated
**Cystic
* [[Microscopic]] findings:
**Encapsulated 
**[[squamous]] and [[Glandular|glandular]] components
| align="left" style="background:#F5F5F5;" |
* Cystic and solid component with variable appearance on [[Computed tomography|CT]] and [[MRI]]
| align="left" style="background:#F5F5F5;" |
* Incisional [[biopsy]] and [[Histopathological|histopathological examination]]
| align="left" style="background:#F5F5F5;" |
* Association with [[CMV]]
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Category
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Benign
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |History
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Pain
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Dysphagia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mass exam
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Others
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab findings
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
|-
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Neoplasm
! rowspan="4" align="center" style="background:#DCDCDC;" |[[Salivary gland neoplasm]]
! align="center" style="background:#DCDCDC;" |[[Adenoid cystic cancer|Adenoid cystic carcinoma]] <ref name="pmid17825603">{{cite journal |vauthors=Jones AV, Craig GT, Speight PM, Franklin CD |title=The range and demographics of salivary gland tumours diagnosed in a UK population |journal=Oral Oncol. |volume=44 |issue=4 |pages=407–17 |date=April 2008 |pmid=17825603 |doi=10.1016/j.oraloncology.2007.05.010 |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Malignant]]
| align="left" style="background:#F5F5F5;" |
* Age: 40s-60s
* Gender: Female predominance
| align="left" style="background:#F5F5F5;" |
* Slow growing rare tumor with low [[Recurrence plot|recurrence]]
| align="center" style="background:#F5F5F5;" | ±
| align="center" style="background:#F5F5F5;" | ±
| align="left" style="background:#F5F5F5;" |
* Solid [[mass]]
| align="left" style="background:#F5F5F5;" |
* Normal to [[Ulcerated lesion|ulcerated lesions]]
* May have [[lymphadenopathy]]
| align="center" style="background:#F5F5F5;" |−
| align="left" style="background:#F5F5F5;" |
* [[Gross]] findings:
** Tubular
** Cribriform
** Solid pattern of growth
* [[Microscopic]] findings:
** Components of large cells with [[Pleomorphic|pleomorphic nuclei]]
** Increased [[mitotic]] activity
** Focal [[Necrosis|necrosis]]
| align="left" style="background:#F5F5F5;" |
* [[Imaging]] reveal dimensions of the [[tumor]], local spread, and [[Metastasis|distant metastasis]]
| align="left" style="background:#F5F5F5;" |
* [[Biopsy]] and [[Histopathological|histopathological examination]]
| align="center" style="background:#F5F5F5;" |−
|-
! align="center" style="background:#DCDCDC;" |[[Adenocarcinoma]]
<ref name="pmid16487803">{{cite journal |vauthors=Beltran D, Faquin WC, Gallagher G, August M |title=Selective immunohistochemical comparison of polymorphous low-grade adenocarcinoma and adenoid cystic carcinoma |journal=J. Oral Maxillofac. Surg. |volume=64 |issue=3 |pages=415–23 |date=March 2006 |pmid=16487803 |doi=10.1016/j.joms.2005.11.027 |url=}}</ref>
| align="center" style="background:#F5F5F5;" |
* [[Malignant]]
| align="left" style="background:#F5F5F5;" |
* Age: young age predilection
| align="left" style="background:#F5F5F5;" |
* Its a [[tumor]] of minor [[salivary glands]]
* May present as small [[ulceration]] or [[nodules]] in [[oral cavity]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
* Small [[nodules]] in [[oral cavity]]
* With or without [[lymphadenopathy]]
| align="left" style="background:#F5F5F5;" |
* Normal to [[Ulcerated lesion|ulcerated lesions]]
* May have [[lymphadenopathy]]
| align="left" style="background:#F5F5F5;" |
* May be normal
* Or may show [[anemia]] and [[blood cell]] disorders with distant bone [[invasion]]
| align="left" style="background:#F5F5F5;" |
* On [[histology]] it is confused with Adeocyctic [[carcinoma]]
* Components of [[gland]] and [[cyst]] formations
* More perineural [[invasion]]
| align="left" style="background:#F5F5F5;" |
* [[CT]] and [[MRI]] 
* [[MRI]] being more accurate for adjacent [[tissue]] involvement and [[lymphadenopathy]]
| align="left" style="background:#F5F5F5;" |
* [[Biopsy]] and [[histopathological]] examination
| align="center" style="background:#F5F5F5;" |−
|-
|-
! align="center" style="background:#DCDCDC;" |[[Salivary gland cancer|Salivary duct cancer]]<ref name="pmid22434951">{{cite journal |vauthors=Mlika M, Kourda N, Zidi Y, Aloui R, Zneidi N, Rammeh S, Zermani R, Jilani SB |title=Salivary duct carcinoma of the parotid gland |journal=J Oral Maxillofac Pathol |volume=16 |issue=1 |pages=134–6 |date=January 2012 |pmid=22434951 |pmc=3303509 |doi=10.4103/0973-029X.92992 |url=}}</ref><ref name="pmid29103750">{{cite journal |vauthors=Schmitt NC, Kang H, Sharma A |title=Salivary duct carcinoma: An aggressive salivary gland malignancy with opportunities for targeted therapy |journal=Oral Oncol. |volume=74 |issue= |pages=40–48 |date=November 2017 |pmid=29103750 |pmc=5685667 |doi=10.1016/j.oraloncology.2017.09.008 |url=}}</ref><ref name="pmid23821208">{{cite journal |vauthors=Simpson RH |title=Salivary duct carcinoma: new developments--morphological variants including pure in situ high grade lesions; proposed molecular classification |journal=Head Neck Pathol |volume=7 Suppl 1 |issue= |pages=S48–58 |date=July 2013 |pmid=23821208 |pmc=3712088 |doi=10.1007/s12105-013-0456-x |url=}}</ref>
|'''Location'''
| align="left" style="background:#F5F5F5;" |
|
* [[Malignant]]
*Intracranial schwannoma:
(Highly aggressive)
:*[[Acoustic neuroma]] (most common)
| align="left" style="background:#F5F5F5;" |
:*Trigeminal schwannoma
* [[Incidence]]: 1-3%
:*Facial nerve schwannoma
* Gender: Male predilection
:*Jugular foramen schwannoma
* Mean age: 55-61 years old
:*Hypoglossal schwannomas
| align="left" style="background:#F5F5F5;" |
*Spinal schwannoma
* Rapidly growing [[mass]] with jaw involvement
*Intercostal nerve schwannoma
| align="center" style="background:#F5F5F5;" | ±
*Intramuscular schwannoma
| align="center" style="background:#F5F5F5;" | ±
*Posterior mediastinum schwannoma
| align="left" style="background:#F5F5F5;" |
*Retroperitoneum schwannoma
*Painless
*Intracerebral schwannoma
*Hard
*Non-compressible [[mass]]
| align="left" style="background:#F5F5F5;" |
* Ulceration of [[mucosa]] and [[Ulceration|skin]]
* May have [[lymphadenopathy]]
* [[facial paralysis]] in case of [[facial nerve]] involvement
| align="left" style="background:#F5F5F5;" |
* Pathomorphologically [[tumor]] of [[Salivary gland|salivary ducts]] resembles tumor of [[breast]] ducts
| align="left" style="background:#F5F5F5;" |
* [[Gross examination|Gross]] findings:
**Firm [[mass]]
**[[Cystic|Cystic component]] of variable size and dimension
*[[Microscopic|Microscopic finding]]:
**Resembling ductal carcinoma of [[breast]]
**Intraductal components invading surrounding tissue in several forms:
***cribriform
***[[papillary]]
***Solid with comedo-like central [[necrosis]]
| align="left" style="background:#F5F5F5;" |
* Non-specific features on [[Computed tomography|CT]] and [[MRI]]
* Shows [[neural]] and [[jaw]] involvement
| align="left" style="background:#F5F5F5;" |
* [[Biopsy]] and [[histopathological]] examination
| align="center" style="background:#F5F5F5;" |−
|-
! align="center" style="background:#DCDCDC;" |[[Squamous cell carcinoma]]<ref name="pmid25328317">{{cite journal |vauthors=Manvikar V, Ramulu S, Ravishanker ST, Chakravarthy C |title=Squamous cell carcinoma of submandibular salivary gland: A rare case report |journal=J Oral Maxillofac Pathol |volume=18 |issue=2 |pages=299–302 |date=May 2014 |pmid=25328317 |pmc=4196305 |doi=10.4103/0973-029X.140909 |url=}}</ref><ref name="pmid16475198">{{cite journal |vauthors=Ying YL, Johnson JT, Myers EN |title=Squamous cell carcinoma of the parotid gland |journal=Head Neck |volume=28 |issue=7 |pages=626–32 |date=July 2006 |pmid=16475198 |doi=10.1002/hed.20360 |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Malignant]]
| align="left" style="background:#F5F5F5;" |
* Incidence: rare
* Age: Old age , 61-68 years
* Male predilection
| align="left" style="background:#F5F5F5;" |
* Present as painful growing [[mass]] on [[jaw]]
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
*Teneder
*Firm
*[[Swelling|Solitary swelling]] on jaw
| align="left" style="background:#F5F5F5;" |
* [[Submandibular gland]] predilection
* Thinning and discoloration of [[skin]]
| align="left" style="background:#F5F5F5;" |
* Past [[radiation]] exposure is a strong [[risk factor]]
| align="left" style="background:#F5F5F5;" |
* [[Gross]] findings: Thinning of [[skin]]
* [[Microscopically]] findings: Nest and solid sheets of [[Tumor cell|tumor cells]] arranged in [[glandular]] pattern
* [[Immunohistochemical staining]] can be used to mark the [[squamous]] and [[keratin]] component
| align="left" style="background:#F5F5F5;" |
* [[Tumor]] dimension can be delineated using both [[CT]] and [[MRI]]
| align="left" style="background:#F5F5F5;" |
* [[Biopsy]] and [[histopathological]] examination
| align="center" style="background:#F5F5F5;" |−
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Category
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Benign
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |History
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Pain
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Dysphagia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mass exam
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Others
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab findings
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
|-
! rowspan="4" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Neoplasm
! colspan="2" align="center" style="background:#DCDCDC;" |[[Hypopharyngeal cancer]]<ref name="pmid12560383">{{cite journal |vauthors=Helliwell TR |title=acp Best Practice No 169. Evidence based pathology: squamous carcinoma of the hypopharynx |journal=J. Clin. Pathol. |volume=56 |issue=2 |pages=81–5 |date=February 2003 |pmid=12560383 |pmc=1769882 |doi= |url=}}</ref><ref>{{cite journal|journal=International Journal of Recent Scientific Research|issn=09763031|doi=10.24327/IJRSR}}</ref><ref name="Maaslandvan den Brandt2014">{{cite journal|last1=Maasland|first1=Denise HE|last2=van den Brandt|first2=Piet A|last3=Kremer|first3=Bernd|last4=Goldbohm|first4=R Alexandra|last5=Schouten|first5=Leo J|title=Alcohol consumption, cigarette smoking and the risk of subtypes of head-neck cancer: results from the Netherlands Cohort Study|journal=BMC Cancer|volume=14|issue=1|year=2014|issn=1471-2407|doi=10.1186/1471-2407-14-187}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Malignant]]
| align="left" style="background:#F5F5F5;" |
* More common in males
* [[Age]]: 55-65 years old
* [[Incidence]]: < 1/100,000 in U.S.
* More common in Japan, India, Iran
| align="left" style="background:#F5F5F5;" |
* [[Tobacco]] use
* [[Abuse|Abuse alcohol]] consumption
* [[HPV infection]]
* Lump in the [[neck]]
* [[Odynophagia]]
* [[Hoarseness]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | +
| align="left" style="background:#F5F5F5;" |
* Non tender [[Cervical|cervical node]]
| align="left" style="background:#F5F5F5;" |
* [[Lymphadenopathy]]
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
* [[Spindle cells]]
* [[Nuclear|Nuclear atypia]]
* Basaloid [[cells]]
* Abundant [[chromatin]]
| align="left" style="background:#F5F5F5;" |
* [[Neck]] [[CT]] scan:
** [[Soft tissue]] mass
** Irregular thickening of [[mucosa]]
** [[Necrotic|Necrotic region]]
* [[MRI]]:
** [[Tumors]] are hypointense on T1 and hyperintense on T2
| align="left" style="background:#F5F5F5;" |
* [[Biopsy]]
| align="center" style="background:#F5F5F5;" | −
|-
! colspan="2" align="center" style="background:#DCDCDC;" |[[Parathyroid cancer]]<ref name="pmid22327883">{{cite journal |vauthors=Wei CH, Harari A |title=Parathyroid carcinoma: update and guidelines for management |journal=Curr Treat Options Oncol |volume=13 |issue=1 |pages=11–23 |date=March 2012 |pmid=22327883 |doi=10.1007/s11864-011-0171-3 |url=}}</ref><ref name="pmid17713315">{{cite journal |vauthors=Sahasranam P, Tran MT, Mohamed H, Friedman TC |title=Multiglandular parathyroid carcinoma: a case report and brief review |journal=South. Med. J. |volume=100 |issue=8 |pages=841–4 |date=August 2007 |pmid=17713315 |doi=10.1097/SMJ.0b013e318073ca37 |url=}}</ref><ref name="pmid4886854">{{cite journal |vauthors=Holmes EC, Morton DL, Ketcham AS |title=Parathyroid carcinoma: a collective review |journal=Ann. Surg. |volume=169 |issue=4 |pages=631–40 |date=April 1969 |pmid=4886854 |pmc=1387475 |doi= |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Malignant]]
| align="left" style="background:#F5F5F5;" |
* [[Incidence]]: Rare
* Mean age : 44-54 years old
* Gender: Female predilection
| align="left" style="background:#F5F5F5;" |
*Presents with the [[hyperparathyroidism]]
*[[Bone]] pains
*[[Abdominal pain]]
*[[Nausea and vomiting]]
*[[Fatigue]]
*[[Confusion]]
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | +
| align="left" style="background:#F5F5F5;" |
* Lower [[Neck masses|neck mass]]
| align="left" style="background:#F5F5F5;" |
*[[Tachycardia]]
*[[Weight loss]]
*[[Sweating]]
*Neck [[swelling]]
| align="left" style="background:#F5F5F5;" |
*Low [[TSH]]
*Increased [[T4]] and [[T3]]
*[[Hypercalcemia]]
| align="left" style="background:#F5F5F5;" |
* [[Microscopic]] findings:
** Trabecular [[Growth|growth pattern]]
** High [[mitosis]]
** Surrounding thick fibrotic bands
** Capsular involvement
** Vascular [[invasion]] is common
| align="left" style="background:#F5F5F5;" |
*[[Computed tomography|CT]] and [[MRI]] shows more frequent lower lobe involvement, vascular involvement , [[lymph node]] [[metastasis]], and perineural involvement
*[[Bone scan]] may show decreasing [[bone density]]
| align="left" style="background:#F5F5F5;" |
* [[Biopsy]] and [[histopathological]] examination
| align="center" style="background:#F5F5F5;" |
* Hereditary syndromes
* [[Multiple endocrine neoplasia type 2|MEN-2B syndrome]]
* [[Neurofibromatosis type 1]]
* [[VHL syndrome|VHL]] disease
|-
! colspan="2" align="center" style="background:#DCDCDC;" |[[Carotid body tumor|Carotid body tumors]]<ref name="pmid174004872">{{cite journal |vauthors=Sajid MS, Hamilton G, Baker DM |title=A multicenter review of carotid body tumour management |journal=Eur J Vasc Endovasc Surg |volume=34 |issue=2 |pages=127–30 |date=August 2007 |pmid=17400487 |doi=10.1016/j.ejvs.2007.01.015 |url=}}</ref><ref name="pmid158837112">{{cite journal |vauthors=Boedeker CC, Ridder GJ, Schipper J |title=Paragangliomas of the head and neck: diagnosis and treatment |journal=Fam. Cancer |volume=4 |issue=1 |pages=55–9 |date=2005 |pmid=15883711 |doi=10.1007/s10689-004-2154-z |url=}}</ref><ref name="pmid15063383">{{cite journal |vauthors=Pellitteri PK, Rinaldo A, Myssiorek D, Gary Jackson C, Bradley PJ, Devaney KO, Shaha AR, Netterville JL, Manni JJ, Ferlito A |title=Paragangliomas of the head and neck |journal=Oral Oncol. |volume=40 |issue=6 |pages=563–75 |date=July 2004 |pmid=15063383 |doi=10.1016/j.oraloncology.2003.09.004 |url=}}</ref><ref name="pmid28478173">{{cite journal |vauthors=Darouassi Y, Alaoui M, Mliha Touati M, Al Maghraoui O, En-Nouali A, Bouaity B, Ammar H |title=Carotid Body Tumors: A Case Series and Review of the Literature |journal=Ann Vasc Surg |volume=43 |issue= |pages=265–271 |date=August 2017 |pmid=28478173 |doi=10.1016/j.avsg.2017.03.167 |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Benign]]
| align="left" style="background:#F5F5F5;" |
* Age: 26-55 years
* Male predominance
| align="left" style="background:#F5F5F5;" |
*A slow growing [[Neck masses|neck mass]]
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
*Mobile
*Non-tender [[Neck masses|neck mass]] (horizontally more than vertically)
*[[Pulsatile Flow|Pulsatile]]
*[[Bruit]] may be present
| align="left" style="background:#F5F5F5;" |
*Change in voice
*[[Dizziness]]
*[[Tinnitus]]
*[[Headache]]
| align="left" style="background:#F5F5F5;" |
* Rasised [[catecholamine]] levels
| align="left" style="background:#F5F5F5;" |
* Microscopically they are extra-adrenal [[paragangliomas]]
| align="left" style="background:#F5F5F5;" |
*[[Doppler ultrasound]], [[Computed tomography|CT,]] [[MRI]] and [[angiography]] is used to visualize the [[tumor]]
*[[Metaiodobenzylguanidine|Metaiodobenzylguanidine (MIBG)]] testing
| align="left" style="background:#F5F5F5;" |
* [[Histopathology]] analysis and [[catecholamine]] levels
| align="center" style="background:#F5F5F5;" |−
|-
! colspan="2" align="center" style="background:#DCDCDC;" |[[Paraganglioma]]<ref name="pmid15328326">{{cite journal |vauthors=Neumann HP, Pawlu C, Peczkowska M, Bausch B, McWhinney SR, Muresan M, Buchta M, Franke G, Klisch J, Bley TA, Hoegerle S, Boedeker CC, Opocher G, Schipper J, Januszewicz A, Eng C |title=Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations |journal=JAMA |volume=292 |issue=8 |pages=943–51 |date=August 2004 |pmid=15328326 |doi=10.1001/jama.292.8.943 |url=}}</ref><ref name="pmid11701678">{{cite journal |vauthors=Erickson D, Kudva YC, Ebersold MJ, Thompson GB, Grant CS, van Heerden JA, Young WF |title=Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients |journal=J. Clin. Endocrinol. Metab. |volume=86 |issue=11 |pages=5210–6 |date=November 2001 |pmid=11701678 |doi=10.1210/jcem.86.11.8034 |url=}}</ref><ref name="pmid8678971">{{cite journal |vauthors=O'Riordain DS, Young WF, Grant CS, Carney JA, van Heerden JA |title=Clinical spectrum and outcome of functional extraadrenal paraganglioma |journal=World J Surg |volume=20 |issue=7 |pages=916–21; discussion 922 |date=September 1996 |pmid=8678971 |doi= |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Benign]] (Majority)
* [[Malignant]] (rare)
| align="left" style="background:#F5F5F5;" |
* Age 50-70 years
* More in females
| align="left" style="background:#F5F5F5;" |
* May be an accidental finding depending on their secretory nature or present with following symptoms:
** [[Palpitation]]
**[[Tremor]]
**Pulse-like vibratory sense
**[[Headache]]
**Change in voice
**Vertigo
*[[Catecholamine]] secreting [[paragangliomas]] presents with :
**[[Hypertension]]
**[[Headache]]
**[[Sweating]]
**[[Tachycardia]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
* No visible [[mass]]
* Located deep in the the [[neck]] along the [[glossopharyngeal nerve]]
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
* [[Biochemical testing]] may show [[catecholamine]] metabolites in [[serum]] or [[urine]] samples
| align="left" style="background:#F5F5F5;" |
* Highly [[vascular tumors]] that involves [[nerves]] around [[vessels]]
* [[Gross examination|Gross findings]]:
**Fleshy [[tumor]]
**Pink to red-brown to gray in color
**Associated with [[hemorrhage]] or [[fibrosis]]
* [[Microscopic|Microscopic findings]]:
** Round or polygonal cells
** Nests or trabecular structures inside the capsule
| align="left" style="background:#F5F5F5;" |
*[[Ultrasound]]
*[[Computed tomography]]
*[[Magnetic resonance imaging]]
*[[Angiography]]
*[[metaiodobenzylguanidine]] (MIBG)
*18F-fluoro-2-deoxyglucose Positron emission tomography (FDG-PET)
| align="left" style="background:#F5F5F5;" |
* [[Imaging]] and [[Catecholamine|serum catecholamine analysis]]
| align="center" style="background:#F5F5F5;" |−
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Category
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Benign
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |History
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Pain
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Dysphagia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mass exam
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Others
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab findings
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
|-
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Neoplasm
! colspan="2" align="center" style="background:#DCDCDC;" |[[Schwannoma]]<ref name="pmid24450866">{{cite journal |vauthors=Hilton DA, Hanemann CO |title=Schwannomas and their pathogenesis |journal=Brain Pathol. |volume=24 |issue=3 |pages=205–20 |date=April 2014 |pmid=24450866 |doi=10.1111/bpa.12125 |url=}}</ref><ref name="pmid28237565">{{cite journal |vauthors=Albert P, Patel J, Badawy K, Weissinger W, Brenner M, Bourhill I, Parnell J |title=Peripheral Nerve Schwannoma: A Review of Varying Clinical Presentations and Imaging Findings |journal=J Foot Ankle Surg |volume=56 |issue=3 |pages=632–637 |date=2017 |pmid=28237565 |doi=10.1053/j.jfas.2016.12.003 |url=}}</ref><ref name="pmid27020268">{{cite journal |vauthors=Wong BLK, Bathala S, Grant D |title=Laryngeal schwannoma: a systematic review |journal=Eur Arch Otorhinolaryngol |volume=274 |issue=1 |pages=25–34 |date=January 2017 |pmid=27020268 |doi=10.1007/s00405-016-4013-6 |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Benign]]
| align="left" style="background:#F5F5F5;" |
* Rare [[tumor]]
* Incidence: 1-10%
| align="left" style="background:#F5F5F5;" |
* Slow growing [[mass]]
* Localized neural deficit depending on the site of [[peripheral nerve]] involved
* [[Vagus nerve]] or [[Sympathetic chain|superior cervical sympathetic chain]] involvement (most common locations)
| align="center" style="background:#F5F5F5;" | +
| align="center" style="background:#F5F5F5;" | ±
| align="left" style="background:#F5F5F5;" |
* Multiple
* Slow growing  [[nodules]] on the skin
| align="left" style="background:#F5F5F5;" |
* [[Vagal]] involvement:
** [[Hoarseness]]
** [[Dysphagia]]
* [[Sympathetic nerve]] involvement may present as [[Horner's syndrome]]:
**[[Dilated pupil]]
** Decrease [[sweating]]
** Dropping eyelid
* [[Vestibular nerve]] involvement & [[Hearing impairment]] (most common)
| align="left" style="background:#F5F5F5;" |
* May be normal
| align="left" style="background:#F5F5F5;" |
* Encapsulated neural tissue growth
| align="left" style="background:#F5F5F5;" |
* Resembling [[Carotid body tumor]] on [[Computed tomography|CT]]
* [[Magnetic resonance imaging|MRI]] and [[Angiography|MRI angiography]] confirm the diagnosis
| align="left" style="background:#F5F5F5;" |
* [[Imaging]]
| align="left" style="background:#F5F5F5;" |
* [[neurofibromatosis type II]]
|-
! colspan="2" align="center" style="background:#DCDCDC;" |[[Lymphoma]] <ref name="pmid7139563">{{cite journal |vauthors=Anderson T, Chabner BA, Young RC, Berard CW, Garvin AJ, Simon RM, DeVita VT |title=Malignant lymphoma. 1. The histology and staging of 473 patients at the National Cancer Institute |journal=Cancer |volume=50 |issue=12 |pages=2699–707 |date=December 1982 |pmid=7139563 |doi= |url=}}</ref><ref name="pmid71395632">{{cite journal |vauthors=Anderson T, Chabner BA, Young RC, Berard CW, Garvin AJ, Simon RM, DeVita VT |title=Malignant lymphoma. 1. The histology and staging of 473 patients at the National Cancer Institute |journal=Cancer |volume=50 |issue=12 |pages=2699–707 |date=December 1982 |pmid=7139563 |doi= |url=}}</ref><ref name="pmid15185336">{{cite journal |vauthors=Negri E, Little D, Boiocchi M, La Vecchia C, Franceschi S |title=B-cell non-Hodgkin's lymphoma and hepatitis C virus infection: a systematic review |journal=Int. J. Cancer |volume=111 |issue=1 |pages=1–8 |date=August 2004 |pmid=15185336 |doi=10.1002/ijc.20205 |url=}}</ref><ref name="pmid2406917">{{cite journal |vauthors=Moormeier JA, Williams SF, Golomb HM |title=The staging of non-Hodgkin's lymphomas |journal=Semin. Oncol. |volume=17 |issue=1 |pages=43–50 |date=February 1990 |pmid=2406917 |doi= |url=}}</ref><ref name="pmid151853362">{{cite journal |vauthors=Negri E, Little D, Boiocchi M, La Vecchia C, Franceschi S |title=B-cell non-Hodgkin's lymphoma and hepatitis C virus infection: a systematic review |journal=Int. J. Cancer |volume=111 |issue=1 |pages=1–8 |date=August 2004 |pmid=15185336 |doi=10.1002/ijc.20205 |url=}}</ref><ref name="pmid71395633">{{cite journal |vauthors=Anderson T, Chabner BA, Young RC, Berard CW, Garvin AJ, Simon RM, DeVita VT |title=Malignant lymphoma. 1. The histology and staging of 473 patients at the National Cancer Institute |journal=Cancer |volume=50 |issue=12 |pages=2699–707 |date=December 1982 |pmid=7139563 |doi= |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Benign]] or [[malignant]]
| align="left" style="background:#F5F5F5;" |
* Age: Predilection for older age
* Mean age: 55
| align="left" style="background:#F5F5F5;" |
* Insidious onset slow growing [[Lymph node|lymph nodes]]
* Non-specific systemic [[B symptoms]]:
** [[Fever]]
** [[Night sweats]]
** [[Weight loss]])
** [[Rash]]
* Waxing and waning [[lymphadenopathy]]
* [[Abdominal fullness]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | ±
| align="left" style="background:#F5F5F5;" |
* Multiple chain [[lymphadenopathy]]
* [[Hepatosplenomegaly]]
* [[Ascites]]
* [[Crackles]] heard on [[Auscultation|chest auscultation]]
| align="left" style="background:#F5F5F5;" |
* [[Rash]] and [[pruritus]]
| align="left" style="background:#F5F5F5;" |
* [[Pancytopenia]]
* [[Hypercalcemia]]
* [[Hyperuricemia]] (increased cell turnover)
* [[Immunoglobulin|Monoclonal immunoglobulin]] (M-spike)
* Raised [[LDH]] levels
| align="left" style="background:#F5F5F5;" |
* On complete node analysis four patterns are described:
** Nodular/follicular
** Diffuse pattern
** Transition from a nodular to a diffuse pattern in adjacent nodes
** Transition from a lower to a higher grade of involvement within a single node
| align="left" style="background:#F5F5F5;" |
* Imaging to stage the disease
* [[Positron emission tomography]] with computed tomography is preferred over [[MRI]]
| align="left" style="background:#F5F5F5;" |
* [[Lymph node]] biopsy coupled with [[cytometry]]
| align="left" style="background:#F5F5F5;" |
* [[Infections]] due to [[cytopenias]]
* With acquired form of [[C1 inhibitor deficiency]] patients may develop [[angioedema]]
|-
! colspan="2" align="center" style="background:#DCDCDC;" |[[Liposarcoma]] <ref name="pmid171979142">{{cite journal |vauthors=Evans HL |title=Atypical lipomatous tumor, its variants, and its combined forms: a study of 61 cases, with a minimum follow-up of 10 years |journal=Am. J. Surg. Pathol. |volume=31 |issue=1 |pages=1–14 |date=January 2007 |pmid=17197914 |doi=10.1097/01.pas.0000213406.95440.7a |url=}}</ref><ref name="pmid21253554">{{cite journal |vauthors=Conyers R, Young S, Thomas DM |title=Liposarcoma: molecular genetics and therapeutics |journal=Sarcoma |volume=2011 |issue= |pages=483154 |date=2011 |pmid=21253554 |pmc=3021868 |doi=10.1155/2011/483154 |url=}}</ref><ref name="pmid19194281">{{cite journal |vauthors=Alaggio R, Coffin CM, Weiss SW, Bridge JA, Issakov J, Oliveira AM, Folpe AL |title=Liposarcomas in young patients: a study of 82 cases occurring in patients younger than 22 years of age |journal=Am. J. Surg. Pathol. |volume=33 |issue=5 |pages=645–58 |date=May 2009 |pmid=19194281 |doi=10.1097/PAS.0b013e3181963c9c |url=}}</ref><ref name="pmid176106862">{{cite journal |vauthors=Serpell JW, Chen RY |title=Review of large deep lipomatous tumours |journal=ANZ J Surg |volume=77 |issue=7 |pages=524–9 |date=July 2007 |pmid=17610686 |doi=10.1111/j.1445-2197.2007.04042.x |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Malignant]]
| align="left" style="background:#F5F5F5;" |
* Rare [[tumor]]
* Age: Relatively in older age
* Gender: No gender predilection
| align="left" style="background:#F5F5F5;" |
* Mobile [[Mass|mass]]
* Few symptoms until they grow enough to compress the surrounding structures
* Symptoms of [[neural]] deficit, pain, [[tingling]], or [[skin]] changes
| align="center" style="background:#F5F5F5;" | ±
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
* Mobile soft [[mass]]
* Intact overlying [[skin]]
* Blue discoloration due to intra-lesion [[hemorrhage]]
| align="left" style="background:#F5F5F5;" |
* Intact [[skin]] and normal color
| align="left" style="background:#F5F5F5;" |
* Normal
| align="left" style="background:#F5F5F5;" |
* [[Gross examination]]:
**Bulk of yellow colored [[fat tissue]]
* [[Microscopic|Microscopic features]]:
** [[Adipose tissue]] containing lipoblasts
** Atypical [[nucleus]] pushed to side by intracytoplasmic vacuoles
* Tissue [[biopsy]] may show [[histological]] sub-groups:
** Well-differentiated
** Myxoid/round cell
** Pleomorphic liposarcomas
| align="left" style="background:#F5F5F5;" |
* [[Imaging]] not usually required for diagnosis
* May show deeper [[invasion]]
* [[Ultrasound]] shows homogeneous hyperechoic [[mass]]
| align="left" style="background:#F5F5F5;" |
* [[Biopsy]] and [[Histopathology|histopathology analysis]]
| align="center" style="background:#F5F5F5;" | −
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Category
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Benign
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |History
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Pain
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Dysphagia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mass exam
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Others
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab findings
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
|-
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Neoplasm
! colspan="2" align="center" style="background:#DCDCDC;" |[[Lipoma]] <ref name="pmid24800932">{{cite journal |vauthors=de Bree E, Karatzanis A, Hunt JL, Strojan P, Rinaldo A, Takes RP, Ferlito A, de Bree R |title=Lipomatous tumours of the head and neck: a spectrum of biological behaviour |journal=Eur Arch Otorhinolaryngol |volume=272 |issue=5 |pages=1061–77 |date=May 2015 |pmid=24800932 |doi=10.1007/s00405-014-3065-8 |url=}}</ref><ref name="pmid6670522">{{cite journal |vauthors=Rydholm A, Berg NO |title=Size, site and clinical incidence of lipoma. Factors in the differential diagnosis of lipoma and sarcoma |journal=Acta Orthop Scand |volume=54 |issue=6 |pages=929–34 |date=December 1983 |pmid=6670522 |doi= |url=}}</ref><ref name="pmid7282321">{{cite journal |vauthors=Myhre-Jensen O |title=A consecutive 7-year series of 1331 benign soft tissue tumours. Clinicopathologic data. Comparison with sarcomas |journal=Acta Orthop Scand |volume=52 |issue=3 |pages=287–93 |date=June 1981 |pmid=7282321 |doi= |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Benign]]
| align="left" style="background:#F5F5F5;" |
* [[Genetic predisposition]]
* Unspecific gender or age association
| align="left" style="background:#F5F5F5;" |
* One or multiple soft, painless skin [[nodules]]
* May causes pain or compressive [[symptoms]]
| align="center" style="background:#F5F5F5;" | ±
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
* Mobile soft [[nodule]]
* Intact overlying [[skin]]
| align="left" style="background:#F5F5F5;" |
* Normal
| align="left" style="background:#F5F5F5;" |
* Normal
| align="left" style="background:#F5F5F5;" |
* Diagnoses is usually [[clinical]]
* Tissue [[biopsy]] may show:
** Bundle of well-demarcated lipocytes
** Single [[nuclei]] aligned to the side
** Intra-cytoplasimic fat granules
| align="left" style="background:#F5F5F5;" |
* Diagnosis is usually clinical
* [[ultrasound]] is used to differentiate [[lipoma]] from other [[benign]] lesions such as:
** [[Epidermoid cyst]]
** A [[ganglion]]
| align="left" style="background:#F5F5F5;" |
* [[Clinical]] evaluation
| align="left" style="background:#F5F5F5;" |
* Multiple [[lipomas]]
* Associated with [[familial multiple lipomatosis]]
|-
! colspan="2" align="center" style="background:#DCDCDC;" |Glomus vagale, [[Glomus jugulare tumor|glomus jugulare]] tumors<ref name="pmid8164483">{{cite journal |vauthors=Urquhart AC, Johnson JT, Myers EN, Schechter GL |title=Glomus vagale: paraganglioma of the vagus nerve |journal=Laryngoscope |volume=104 |issue=4 |pages=440–5 |date=April 1994 |pmid=8164483 |doi=10.1288/00005537-199404000-00008 |url=}}</ref><ref name="pmid6308990">{{cite journal |vauthors=Valavanis A, Schubiger O, Oguz M |title=High-resolution CT investigation of nonchromaffin paragangliomas of the temporal bone |journal=AJNR Am J Neuroradiol |volume=4 |issue=3 |pages=516–9 |date=1983 |pmid=6308990 |doi= |url=}}</ref><ref name="pmid81644832">{{cite journal |vauthors=Urquhart AC, Johnson JT, Myers EN, Schechter GL |title=Glomus vagale: paraganglioma of the vagus nerve |journal=Laryngoscope |volume=104 |issue=4 |pages=440–5 |date=April 1994 |pmid=8164483 |doi=10.1288/00005537-199404000-00008 |url=}}</ref><ref name="pmid1988766">{{cite journal |vauthors=Stein PP, Black HR |title=A simplified diagnostic approach to pheochromocytoma. A review of the literature and report of one institution's experience |journal=Medicine (Baltimore) |volume=70 |issue=1 |pages=46–66 |date=January 1991 |pmid=1988766 |doi= |url=}}</ref><ref name="pmid17400487">{{cite journal |vauthors=Sajid MS, Hamilton G, Baker DM |title=A multicenter review of carotid body tumour management |journal=Eur J Vasc Endovasc Surg |volume=34 |issue=2 |pages=127–30 |date=August 2007 |pmid=17400487 |doi=10.1016/j.ejvs.2007.01.015 |url=}}</ref><ref name="pmid15883711">{{cite journal |vauthors=Boedeker CC, Ridder GJ, Schipper J |title=Paragangliomas of the head and neck: diagnosis and treatment |journal=Fam. Cancer |volume=4 |issue=1 |pages=55–9 |date=2005 |pmid=15883711 |doi=10.1007/s10689-004-2154-z |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Benign]]
| align="left" style="background:#F5F5F5;" |
* Rare tumor
| align="left" style="background:#F5F5F5;" |
* Painless slowly enlarging [[Mass|mass]] in the [[neck]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | ±
| align="left" style="background:#F5F5F5;" |
* Non-compressible
* Firm
* Non-tender [[swelling]]
* No [[thrill]] or [[bruit]]
* Normal overlying skin
| align="left" style="background:#F5F5F5;" |
* Secretory tumors
* May have compressive signs such as:
** [[Dysphagia]]
** [[Hoarseness]]
** [[Cranial nerves]] deficits
** [[Horner's syndrome]]
| align="left" style="background:#F5F5F5;" |
* Normal
| align="left" style="background:#F5F5F5;" |
* [[Glomus tumor|Glomus tumors]] arise from Non [[Chromaffin cells]]
* [[histopathology]] reveals "salt and pepper" [[chromatin]]
* On [[immunohistochemistry]] [[Tumor cell|tumor cells]] show [[chromogranin]] and [[S-100]] positivity
| align="left" style="background:#F5F5F5;" |
* [[MRI]] ([[Imaging]] of choice): Typical appearance of the [[tumor]] along [[vagus nerve]]
* [[Ultrasonography]] (early stage of diagnosis): Isoechoic to hypoechoic well defined [[tumor]]
* [[CT-scans|CT]]: Vascularity of the [[tumor]]
* Biochemical testing: Shows secretary nature of the [[tumor]]
| align="left" style="background:#F5F5F5;" |
* [[Imaging]] and [[Metaiodobenzylguanidine]] (MIBG) testing
| align="center" style="background:#F5F5F5;" | −
|-
! colspan="2" align="center" style="background:#DCDCDC;" |[[Head and neck cancer|Metastatic head and neck cancer]]<ref name="pmid2211107">{{cite journal |vauthors=Gluckman JL, Robbins KT, Fried MP |title=Cervical metastatic squamous carcinoma of unknown or occult primary source |journal=Head Neck |volume=12 |issue=5 |pages=440–3 |date=1990 |pmid=2211107 |doi= |url=}}</ref><ref name="pmid19841343">{{cite journal |vauthors=Waltonen JD, Ozer E, Hall NC, Schuller DE, Agrawal A |title=Metastatic carcinoma of the neck of unknown primary origin: evolution and efficacy of the modern workup |journal=Arch. Otolaryngol. Head Neck Surg. |volume=135 |issue=10 |pages=1024–9 |date=October 2009 |pmid=19841343 |doi=10.1001/archoto.2009.145 |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Malignant]]
| align="left" style="background:#F5F5F5;" |
* Depends on the nature of [[metastatic]] [[tumor]]
| align="left" style="background:#F5F5F5;" |
* Asymptomatic
* Painless [[lymphadenopathy]]
* Supra clavicular fullness in case of [[stomach cancer]] [[metastasis]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | ±
| align="left" style="background:#F5F5F5;" |
* Non-[[Tenderness|tender]] [[mass]] in the [[neck]]
* Non-tender [[lymphadenopathy]]
| align="left" style="background:#F5F5F5;" |
* Majority of metastatic [[head and neck cancer]]
* Metastatise from [[Gastrointestinal tract|GIT]] and lungs
| align="left" style="background:#F5F5F5;" |
* Vary depending on the underlying [[cancer]]
| align="left" style="background:#F5F5F5;" |
* [[Histology]] of primary cancer
| align="left" style="background:#F5F5F5;" |
* [[Computed tomography|CT]] and [[Magnetic resonance imaging|MRI]] to see extent of [[tumor]]
| align="left" style="background:#F5F5F5;" |
* [[Biopsy]] and [[histopathology]] of the primary site of [[tumor]]
| align="center" style="background:#F5F5F5;" | −
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Category
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Benign
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |History
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Pain
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Dysphagia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mass exam
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Others
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab findings
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
|-
! rowspan="3" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Other
! colspan="2" align="center" style="background:#DCDCDC;" |[[Laryngeal cancer]]<ref name="pmid6639441">{{cite journal |vauthors=Feldman PS, Kaplan MJ, Johns ME, Cantrell RW |title=Fine-needle aspiration in squamous cell carcinoma of the head and neck |journal=Arch Otolaryngol |volume=109 |issue=11 |pages=735–42 |date=November 1983 |pmid=6639441 |doi= |url=}}</ref><ref name="pmid26237923">{{cite journal |vauthors=Grénman R, Koivunen P, Minn H |title=[Laryngeal cancer in Finland] |language=Finnish |journal=Duodecim |volume=131 |issue=4 |pages=331–7 |date=2015 |pmid=26237923 |doi= |url=}}</ref>
| align="left" style="background:#F5F5F5;" |[[Benign]]/[[Malignant]]
| align="left" style="background:#F5F5F5;" |
* Older males
* Younger patients with [[Human papillomavirus|HPV]] infection or smoking history
| align="left" style="background:#F5F5F5;" |
* [[Neck masses|Neck mass]]
* [[Hoarseness]]
* Throat pain
* [[Snoring]]
* [[Obstructive sleep apnea]]
| align="center" style="background:#F5F5F5;" | ±
| align="center" style="background:#F5F5F5;" | ±
| align="left" style="background:#F5F5F5;" |
* Examination of [[neck]] and [[oral cavity]] may show :
** [[mass]] 
** [[lymphadenopathy]]
*Examination of [[laryngeal cancer]] is done using flexible [[laryngoscopy]] under [[anesthesia]].
| align="left" style="background:#F5F5F5;" |
* [[Smoking]] is the most common risk factor
* [[Smoking]] with [[alcohol]] increases the risk
* Oropharyngeal cancers presenting with [[neck masses]] are associated with
[[Human papillomavirus|human papillomavirus (HPV)]]  infection
| align="left" style="background:#F5F5F5;" |
* [[Human papillomavirus|HPV testing]] may show [[HPV infection]]
| align="left" style="background:#F5F5F5;" |
* [[FNA]] of [[Neck masses|neck mass]]
* Followed by [[biopsy]] of [[laryngeal cancer]]
* Show type cancer cells
| align="left" style="background:#F5F5F5;" |
* [[Computed tomography|CT]], [[Magnetic resonance imaging|MRI]] and [[Positron emission tomography|PET]] are used to see local infiltration by [[cancer]]
* Also to see distant [[metastases]].
* Panendoscopy is done to see extent of the [[tumor]].
| align="left" style="background:#F5F5F5;" |
* [[Laryngoscopy]] and [[biopsy]]
| align="center" style="background:#F5F5F5;" | −
|-
! colspan="2" align="center" style="background:#DCDCDC;" |[[Arteriovenous fistula]]
<ref name="pmid26972281">{{cite journal |vauthors=Guneyli S, Cinar C, Bozkaya H, Korkmaz M, Oran I |title=Endovascular management of congenital arteriovenous fistulae in the neck |journal=Diagn Interv Imaging |volume=97 |issue=9 |pages=871–5 |date=September 2016 |pmid=26972281 |doi=10.1016/j.diii.2015.08.006 |url=}}</ref><ref name="pmid8264877">{{cite journal |vauthors=Gobin YP, Garcia de la Fuente JA, Herbreteau D, Houdart E, Merland JJ |title=Endovascular treatment of external carotid-jugular fistulae in the parotid region |journal=Neurosurgery |volume=33 |issue=5 |pages=812–6 |date=November 1993 |pmid=8264877 |doi= |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Benign]]/[[Malignant]]
| align="left" style="background:#F5F5F5;" |
* Depends on the risk factors
| align="left" style="background:#F5F5F5;" |
* Expanding [[Neck masses|neck mass]]
* [[Headaches]]
* [[Dizziness]]
* [[Neurological|Neurological sequels]]
| align="center" style="background:#F5F5F5;" | −
| align="center" style="background:#F5F5F5;" | −
| align="left" style="background:#F5F5F5;" |
* Pulsating [[Neck masses|neck mass]]
* [[Bruit]]
| align="left" style="background:#F5F5F5;" |
* May be associated with [[vasculopathies]]
* [[metastatic]] invasion of vessels and neck surgery
| align="left" style="background:#F5F5F5;" |
| align="left" style="background:#F5F5F5;" |
* Varies depending on the etiology
| align="left" style="background:#F5F5F5;" |
* [[MR angiography]]
| align="left" style="background:#F5F5F5;" |
* [[MR angiography]]
| align="center" style="background:#F5F5F5;" | −
|-
! colspan="2" align="center" style="background:#DCDCDC;" |[[Thyroid mass causes|Thyroid nodule]]/ [[Goiter]]
<ref name="pmid7606997">{{cite journal |vauthors=Madjar S, Weissberg D |title=Retrosternal goiter |journal=Chest |volume=108 |issue=1 |pages=78–82 |date=July 1995 |pmid=7606997 |doi= |url=}}</ref><ref name="pmid11893102">{{cite journal |vauthors=Hedayati N, McHenry CR |title=The clinical presentation and operative management of nodular and diffuse substernal thyroid disease |journal=Am Surg |volume=68 |issue=3 |pages=245–51; discussion 251–2 |date=March 2002 |pmid=11893102 |doi= |url=}}</ref><ref name="pmid23145396">{{cite journal |vauthors=Hughes K, Eastman C |title=Goitre - causes, investigation and management |journal=Aust Fam Physician |volume=41 |issue=8 |pages=572–6 |date=August 2012 |pmid=23145396 |doi= |url=}}</ref><ref name="pmid10972051">{{cite journal |vauthors=Hermus AR, Huysmans DA |title=[Diagnosis and therapy of patients with euthyroid goiter] |language=Dutch; Flemish |journal=Ned Tijdschr Geneeskd |volume=144 |issue=34 |pages=1623–7 |date=August 2000 |pmid=10972051 |doi= |url=}}</ref>
| align="left" style="background:#F5F5F5;" |
* [[Benign]]/ [[Malignant]]
| align="left" style="background:#F5F5F5;" |
* Female predominance
* Young age (benign causes)
* Old age ([[malignant]] etiology)
| align="left" style="background:#F5F5F5;" |
* Growing painless [[Neck masses|neck mass]] in front of neck
* [[Weight loss]]
* Palpitation
* [[Hoarseness]]
* [[Irritability]]
| align="center" style="background:#F5F5F5;" | ±
| align="center" style="background:#F5F5F5;" | ±
| align="left" style="background:#F5F5F5;" |
* Painless
* Non-tender
* Asymmetrical [[Neck masses|neck mass]] in front of neck
* With smooth overlying skin
* Nodular surface
* Depending on the type:
** May be mobile
** Adherent to the underlying structure
** [[Lymphadenopathy]] in case of [[malignant]] features
| align="left" style="background:#F5F5F5;" |
* [[Goiter]] is most commonly associated with [[iodine deficiency]]
| align="left" style="background:#F5F5F5;" |
* Normal to low [[TSH|TSH levels]] in case of malignancy
* High TSH levels in case of [[goiter]]
| align="left" style="background:#F5F5F5;" |
* [[FNA]] is done in case of [[goiter]]
* [[Biopsy|Core biopsy]] is performed if [[malignancy]] is suspected
| align="left" style="background:#F5F5F5;" |
* [[Ultrasonography|USG]]:
** Shows nodular or non- nodular lesions in [[Thyroid]]
** [[Ultrasonography|US]] is better than [[Computed tomography|CT]].
* [[Thyroid]] [[radionuclide imaging]]:
** Shows [[radioiodine]] uptake
** Cold in case of [[malignancy]]
** Cold or hot in case of [[goiter]].
| align="left" style="background:#F5F5F5;" |
* [[Biopsy]] and [[histopathology]] of nodules
| align="center" style="background:#F5F5F5;" | −
|-
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Category
! colspan="2" style="background: #4479BA; color: #FFFFFF; text-align: center;" |Diseases
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Benign
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Demography
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |History
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Pain
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Dysphagia
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Mass exam
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Others
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Lab findings
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Histopathology
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Imaging
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Gold standard diagnosis
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Associated findings
|}
|}
==[[Schwannoma pathophysiology|Pathophysiology]]==
==[[Schwannoma causes|Causes]]==
==[[Schwannoma differential diagnosis|Differentiating Schwannoma from other Diseases]]==
==[[Schwannoma epidemiology and demographics|Epidemiology and Demographics]]==
==== Age ====
Peak presentation is in the 5th-6th decades. When they occur in patients with neurofibromatosis type 2 (NF2), schwannomas usually present by the 3rd decade 4.
=== Gender ===
There is no sex predilection.
==[[Schwannoma risk factors|Risk Factors]]==
==[[Schwannoma screening|Screening]]==
==[[Schwannoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]==
{{SI}}
==== Clinical presentation ====
Presentation depends on the location of the tumor (see below) but generally, symptoms are due to local mass effect or dysfunction of the nerve they arise from. 
Symptoms of schwannoma depend on the location of the tumor.
*Intracranial schwannoma:
:*[[Acoustic neuroma]] (most common)
::*[[Sensorineural]] [[hearing]] loss
::*[[Vertigo]]
::*[[Tinnitus]]
::*[[Facial weakness]]
::*Facial [[numbness]] and [[tingling]]
::*[[Headaches]]
::*[[Dizziness]]
::*Difficulty swallowing and [[hoarseness]]
::*Taste changes
::*[[Confusion]]
:*Trigeminal schwannoma
::*[[Trigeminal nerve]] dysfunction
:*Facial nerve schwannoma
::*[[Facial nerve]] dysfunction
:*Jugular foramen schwannoma
::*[[Hearing loss]]
::*[[Tinnitus]]
::*[[Dysphagia]]
::*[[Ataxia]]
::*[[Hoarseness]]
:*Hypoglossal schwannomas
::*[[Hypoglossal nerve]] dysfunction
*Spinal schwannoma
::*[[Back pain]]
::*[[Urinary incontinence]]
::*[[Urinary retention]]
::*[[Clumsiness]]
::*[[Weakness]]
::*[[Paresthesias]]
*Intercostal nerve schwannoma
:*Usually asymptomatic
*Intramuscular schwannoma
:*Painless [[mass]]
=== Physical Examination ===
Common physical examination findings of schwannoma include [[papilledema]], impaired hearing, [[hypoesthesia]], and extremities [[paresis]].
===HEENT===
*[[Papilledema]] on fundoscopic examination
*[[Hypoesthesia]]
*[[Aphasia]]
*Impaired hearing
*Facial paralysis
*[[Nystagmus]]
===Neuromuscular===
*[[Hyporeflexia]]
*Extremities [[paresis]]
*[[Ataxia]]
*Extremities [[hypoesthesia]]
==== Pathology ====
==== Macroscopic appearance ====
Schwannomas are benign encapsulated neoplasms of Schwann cells (WHO grade I). They arise eccentrically from their parent nerve, with the nerve fibers splayed along their surface (as distinct to neurofibromas which arise within the nerve).
==== Microscopic appearance ====
Conventional schwannomas are composed of spindle cells which demonstrate two growth patterns: Antoni type A and Antoni type B 7,8.
Antoni type A pattern: elongated cells are densely packed and arranged in fascicles. Palisades are sometimes seen; when prominent these form Verocay bodies.
Antoni type B pattern cells are less compact and are prone to cystic degeneration.
===== Variants =====
Schwannoma variants include 6,8:
* ancient schwannoma
* cellular schwannoma
** predominantly composed of Antoni A tissue
** no Verocay bodies
** most commonly found in a paravertebral location, or trigeminal nerves (CN V)
* melanotic schwannoma: dense melanin pigment
* plexiform schwannoma
** usually arise from skin or subcutaneous tissues
** usually diagnosed at birth or childhood
** usually sporadic, but rarely associated with NF2
** should not be confused with plexiform neurofibromas
*** associated with NF1
*** may undergo malignant change
===== Locations =====
* intracranial schwannomas
** cranial nerves: although almost any cranial nerve may be involved, except olfactory nerves and optic nerves which lack sheaths composed of Schwann cells, by far the most commonly involved nerve is the vestibulocochlear nerve (CN VIII)
** non-cranial nerve or intracerebral (very rare)
* spinal schwannoma
** arising from spinal nerve roots  
* trunk
** intercostal nerves – see intercostal nerve schwannoma
** posterior mediastinum
** retroperitoneum
** gastrointestinal schwannoma
* limbs
** especially flexor surfaces (specifically ulnar and peroneal nerves)
==== Radiographic features ====
General imaging features of schwannomas include:
* well circumscribed masses which displace adjacent structures without direct invasion
* cystic and fatty degeneration are common 4
* the larger a schwannoma, the more likely it is to show heterogeneity because of cystic degeneration or hemorrhage 3
* hemorrhage occurs in 5% of cases 3
* calcification is rare
===== CT =====
CT is not as sensitive or specific for the diagnosis of schwannoma as MRI but is often the first investigation obtained. It is particularly useful in assessing bony changes adjacent to the tumor.
Imaging features include:
* low to intermediate attenuation
* intense contrast enhancement
** small tumors typically demonstrate homogeneous enhancement
** larger tumors may show heterogeneous enhancement
* adjacent bone remodelling with smooth corticated edges
===== MRI =====
Schwannomas have fairly predictable signal characteristics 7:
* '''T1''': isointense or hypointense
* '''T1 C+ (Gd)''': intense enhancement
* '''T2:''' heterogeneously hyperintense (Antoni A: relatively low, Antoni B: high)
** cystic degenerative areas may be present, especially in larger tumors
* '''T2*:''' larger tumors often have areas of hemosiderin
A number of signs can also be useful:
* split-fat sign: thin peripheral rim of fat best seen on planes along long axis of the lesion in non-fat-suppressed sequences
* target sign
** peripheral high T2 signal
** central low signal
** rarely seen intracranially 7
* fascicular sign: multiple small ring-like structures
==== Treatment and prognosis ====
Schwannomas are slow-growing lesions. Surgery is the treatment of choice. As schwannomas do not infiltrate the parent nerve, they can usually be separated from it. Recurrence is unusual, even after complete resection. They almost never undergo malignant change.
==Overview==
'''Schwannomatosis''' is one form of a genetic disorder called [[Neurofibromatosis]] (NF) that has only recently been recognized. It is a rare disorder, affecting only around 1:40,000 individuals.
== Symptoms ==
* [[Schwannomas_and_Neurofibromas|Schwannomas]] occur instead of [[Neurofibroma|Neurofibromas]].
* Multiple Schwannomas occur.
* The Schwannomas develop on cranial, spinal and peripheral nerves.
* Chronic pain, and sometimes numbness, tingling and weakness.
* About 1/3 of patients have segmental Schwannomatosis, which means that the Schwannomas are limited to a single part of the body, such as an arm, a leg or the spine.
* There are several cases where people with Schwannomatosis have developed a vestibular Schwannoma ([[acoustic neuroma]]).
* Patients with Schwannomatosis do not have learning disabilities related to the disease.
* Symptoms are generally brought on by hormonal changes such as puberty and pregnancy.
== Cause ==
* Like the NF1 and NF2 genes, the candidate Schwannomatosis gene, named INI1, is a tumor suppressor that regulates cell cycle, growth and differentiation. INI1 is also known as SMARCB1, hSNF5, or BAF47, and lies on human Chromosome 22. INI1 is also mutated in other tumors including malignant brain & kidney tumors in infants.
* Schwannomatosis is known to be a genetic disorder, however it can skip generations.
== Treatment ==
* If feasible, the Schwannomas can be surgically removed. Any tumor-associated pain usually subsides after tumor removal.  Damaged nerves and scar tissue can be a result of surgery and pain can be an ongoing problem.
* Sometimes, a tumor will reappear at the same site after surgery.
* If surgery is unfeasible, then pain management will have to be used.  Schwannomatosis can sometimes cause severe, untreatable pain over time.
* Other than surgery and/or pain management, there are no other medical treatments available. There are no drugs available to treat Schwannomatosis. 
* Gamma knife surgery can be performed on head tumors to help slow growth of a tumor, although there is no guarantee that it will work.
* Recently, many advances are being made in the treatment of schwannomas. Of interest is '''CyberKnife''', manufactured by '''Accuray'''. Success rates, although limited in data, appear to be in the low to mid ninety percent range.
[[Category:Neurological disorders]]
[[Category:Types of cancer]]
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==Diagnosis==
[[Schwannoma staging|Staging]] | [[Schwannoma history and symptoms|History and Symptoms]] | [[Schwannoma physical examination|Physical Examination]] | [[Schwannoma laboratory findings|Laboratory Findings]] | [[Schwannoma x ray|X Ray]] | [[Schwannoma CT|CT]] | [[Schwannoma MRI|MRI]] | [[Schwannoma ultrasound|Ultrasound]] | [[Schwannoma other imaging findings|Other Imaging Findings]] | [[Schwannoma other diagnostic studies|Other Diagnostic Studies]] | [[Schwannoma biopsy|Biopsy]]
==Treatment==
A schwannoma (sh-won-oma) is a tumour of the tissue that covers nerves, called the nerve sheath. These tumours develop from a type of cell called a Schwann cell, which gives them their name.
Schwannomas are often not cancerous (benign).
The most common type of schwannoma is a vestibular schwannoma. It affects the nerve responsible for balance (also called the vestibular nerve).
It can cause inner ear deafness because the hearing and balance nerve run together, and as the tumour grows it damages the hearing nerve (cochlear nerve). Because of this doctors used to call them acoustic neuromas.
* Find out more about vestibular schwannoma
When these tumours are cancerous they are called malignant schwannomas. They are also called malignant peripheral nerve sheath tumours or neurofibrosarcomas.
They can start anywhere in the body.
The most common areas are:
* the major nerve of the leg (the sciatic nerve)
* the nerves at the top of the arm (the brachial plexus)
* the lower back (the network of nerves called the sacral plexus).
* Find out more about malignant schwannomas
*
== Treatment of Schwannomas ==
Treatment depends on where the tumour is in your body and whether it is benign or malignant (cancerous). Your specialist may monitor you carefully at first rather than suggest treatment. They will see your regularly and monitor any symptoms you have. This is because the treatment can cause a lot of side effects.
The main treatment for schwannoma is surgery to remove the tumour. The type of surgery you have depends on where in the body the tumour is.
You might also have radiotherapy and chemotherapy if you have a malignant tumour.  

Revision as of 15:45, 15 October 2019


Overview

Schwannomas are benign tumors of Schwann cell origin and are the most common tumor of peripheral nerves, including cranial nerves.

Classification

Schwannoma may be classified according to pathology into 4 subtypes: conventional schwannoma, cellular schwannoma, plexiform schwannoma, and melanotic schwannoma.[1][2][3]

Classification
Pathology
  • Conventional schwannoma
  • Cellular schwannoma
  • Plexiform schwannoma
  • Melanotic schwannoma
Location
  • Intracranial schwannoma:
  • Acoustic neuroma (most common)
  • Trigeminal schwannoma
  • Facial nerve schwannoma
  • Jugular foramen schwannoma
  • Hypoglossal schwannomas
  • Spinal schwannoma
  • Intercostal nerve schwannoma
  • Intramuscular schwannoma
  • Posterior mediastinum schwannoma
  • Retroperitoneum schwannoma
  • Intracerebral schwannoma

Pathophysiology

Causes

Differentiating Schwannoma from other Diseases

Epidemiology and Demographics

Age

Peak presentation is in the 5th-6th decades. When they occur in patients with neurofibromatosis type 2 (NF2), schwannomas usually present by the 3rd decade 4.

Gender

There is no sex predilection.

Risk Factors

Screening

Natural History, Complications and Prognosis

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Clinical presentation

Presentation depends on the location of the tumor (see below) but generally, symptoms are due to local mass effect or dysfunction of the nerve they arise from.

Symptoms of schwannoma depend on the location of the tumor.

  • Intracranial schwannoma:
  • Trigeminal schwannoma
  • Facial nerve schwannoma
  • Jugular foramen schwannoma
  • Hypoglossal schwannomas
  • Spinal schwannoma
  • Intercostal nerve schwannoma
  • Usually asymptomatic
  • Intramuscular schwannoma

Physical Examination

Common physical examination findings of schwannoma include papilledema, impaired hearing, hypoesthesia, and extremities paresis.

HEENT

Neuromuscular

Pathology

Macroscopic appearance

Schwannomas are benign encapsulated neoplasms of Schwann cells (WHO grade I). They arise eccentrically from their parent nerve, with the nerve fibers splayed along their surface (as distinct to neurofibromas which arise within the nerve).

Microscopic appearance

Conventional schwannomas are composed of spindle cells which demonstrate two growth patterns: Antoni type A and Antoni type B 7,8.

Antoni type A pattern: elongated cells are densely packed and arranged in fascicles. Palisades are sometimes seen; when prominent these form Verocay bodies.

Antoni type B pattern cells are less compact and are prone to cystic degeneration.

Variants

Schwannoma variants include 6,8:

  • ancient schwannoma
  • cellular schwannoma
    • predominantly composed of Antoni A tissue
    • no Verocay bodies
    • most commonly found in a paravertebral location, or trigeminal nerves (CN V)
  • melanotic schwannoma: dense melanin pigment
  • plexiform schwannoma
    • usually arise from skin or subcutaneous tissues
    • usually diagnosed at birth or childhood
    • usually sporadic, but rarely associated with NF2
    • should not be confused with plexiform neurofibromas
      • associated with NF1
      • may undergo malignant change
Locations
  • intracranial schwannomas
    • cranial nerves: although almost any cranial nerve may be involved, except olfactory nerves and optic nerves which lack sheaths composed of Schwann cells, by far the most commonly involved nerve is the vestibulocochlear nerve (CN VIII)
    • non-cranial nerve or intracerebral (very rare)
  • spinal schwannoma
    • arising from spinal nerve roots  
  • trunk
    • intercostal nerves – see intercostal nerve schwannoma
    • posterior mediastinum
    • retroperitoneum
    • gastrointestinal schwannoma
  • limbs
    • especially flexor surfaces (specifically ulnar and peroneal nerves)

Radiographic features

General imaging features of schwannomas include:

  • well circumscribed masses which displace adjacent structures without direct invasion
  • cystic and fatty degeneration are common 4
  • the larger a schwannoma, the more likely it is to show heterogeneity because of cystic degeneration or hemorrhage 3
  • hemorrhage occurs in 5% of cases 3
  • calcification is rare
CT

CT is not as sensitive or specific for the diagnosis of schwannoma as MRI but is often the first investigation obtained. It is particularly useful in assessing bony changes adjacent to the tumor.

Imaging features include:

  • low to intermediate attenuation
  • intense contrast enhancement
    • small tumors typically demonstrate homogeneous enhancement
    • larger tumors may show heterogeneous enhancement
  • adjacent bone remodelling with smooth corticated edges
MRI

Schwannomas have fairly predictable signal characteristics 7:

  • T1: isointense or hypointense
  • T1 C+ (Gd): intense enhancement
  • T2: heterogeneously hyperintense (Antoni A: relatively low, Antoni B: high)
    • cystic degenerative areas may be present, especially in larger tumors
  • T2*: larger tumors often have areas of hemosiderin

A number of signs can also be useful:

  • split-fat sign: thin peripheral rim of fat best seen on planes along long axis of the lesion in non-fat-suppressed sequences
  • target sign
    • peripheral high T2 signal
    • central low signal
    • rarely seen intracranially 7
  • fascicular sign: multiple small ring-like structures

Treatment and prognosis

Schwannomas are slow-growing lesions. Surgery is the treatment of choice. As schwannomas do not infiltrate the parent nerve, they can usually be separated from it. Recurrence is unusual, even after complete resection. They almost never undergo malignant change.

Overview

Schwannomatosis is one form of a genetic disorder called Neurofibromatosis (NF) that has only recently been recognized. It is a rare disorder, affecting only around 1:40,000 individuals.

Symptoms

  • Schwannomas occur instead of Neurofibromas.
  • Multiple Schwannomas occur.
  • The Schwannomas develop on cranial, spinal and peripheral nerves.
  • Chronic pain, and sometimes numbness, tingling and weakness.
  • About 1/3 of patients have segmental Schwannomatosis, which means that the Schwannomas are limited to a single part of the body, such as an arm, a leg or the spine.
  • There are several cases where people with Schwannomatosis have developed a vestibular Schwannoma (acoustic neuroma).
  • Patients with Schwannomatosis do not have learning disabilities related to the disease.
  • Symptoms are generally brought on by hormonal changes such as puberty and pregnancy.

Cause

  • Like the NF1 and NF2 genes, the candidate Schwannomatosis gene, named INI1, is a tumor suppressor that regulates cell cycle, growth and differentiation. INI1 is also known as SMARCB1, hSNF5, or BAF47, and lies on human Chromosome 22. INI1 is also mutated in other tumors including malignant brain & kidney tumors in infants.
  • Schwannomatosis is known to be a genetic disorder, however it can skip generations.

Treatment

  • If feasible, the Schwannomas can be surgically removed. Any tumor-associated pain usually subsides after tumor removal. Damaged nerves and scar tissue can be a result of surgery and pain can be an ongoing problem.
  • Sometimes, a tumor will reappear at the same site after surgery.
  • If surgery is unfeasible, then pain management will have to be used. Schwannomatosis can sometimes cause severe, untreatable pain over time.
  • Other than surgery and/or pain management, there are no other medical treatments available. There are no drugs available to treat Schwannomatosis.
  • Gamma knife surgery can be performed on head tumors to help slow growth of a tumor, although there is no guarantee that it will work.
  • Recently, many advances are being made in the treatment of schwannomas. Of interest is CyberKnife, manufactured by Accuray. Success rates, although limited in data, appear to be in the low to mid ninety percent range.

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Diagnosis

Staging | History and Symptoms | Physical Examination | Laboratory Findings | X Ray | CT | MRI | Ultrasound | Other Imaging Findings | Other Diagnostic Studies | Biopsy

Treatment

A schwannoma (sh-won-oma) is a tumour of the tissue that covers nerves, called the nerve sheath. These tumours develop from a type of cell called a Schwann cell, which gives them their name.

Schwannomas are often not cancerous (benign).

The most common type of schwannoma is a vestibular schwannoma. It affects the nerve responsible for balance (also called the vestibular nerve).

It can cause inner ear deafness because the hearing and balance nerve run together, and as the tumour grows it damages the hearing nerve (cochlear nerve). Because of this doctors used to call them acoustic neuromas.

  • Find out more about vestibular schwannoma

When these tumours are cancerous they are called malignant schwannomas. They are also called malignant peripheral nerve sheath tumours or neurofibrosarcomas.

They can start anywhere in the body.

The most common areas are:

  • the major nerve of the leg (the sciatic nerve)
  • the nerves at the top of the arm (the brachial plexus)
  • the lower back (the network of nerves called the sacral plexus).
  • Find out more about malignant schwannomas

Treatment of Schwannomas

Treatment depends on where the tumour is in your body and whether it is benign or malignant (cancerous). Your specialist may monitor you carefully at first rather than suggest treatment. They will see your regularly and monitor any symptoms you have. This is because the treatment can cause a lot of side effects.

The main treatment for schwannoma is surgery to remove the tumour. The type of surgery you have depends on where in the body the tumour is.

You might also have radiotherapy and chemotherapy if you have a malignant tumour.  

  1. Touzri RA, Errais K, Zermani R, Benjilani S, Ouertani A (2009). "Schwannoma of the eyelid: apropos of two cases". Indian J Ophthalmol. 57 (4): 318–20. doi:10.4103/0301-4738.53063. PMC 2712707. PMID 19574706.
  2. Sharma S, Rai G (2016). "Schwannoma (Neurilemmoma) on the Base of the Tongue: A Rare Clinical Case". Am J Case Rep. 17: 203–6. doi:10.12659/ajcr.897063. PMC 4815988. PMID 27018477.
  3. Cohen M, Wang MB (2009). "Schwannoma of the tongue: two case reports and review of the literature". Eur Arch Otorhinolaryngol. 266 (11): 1823–9. doi:10.1007/s00405-008-0907-2. PMC 2758150. PMID 19130068.