Sandbox:Hannan1: Difference between revisions

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| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Medullary thyroid carcinoma<ref name="pmid29978018">{{cite journal |vauthors=Segura S, Ramos-Rivera G, Suhrland M |title=Educational Case: Endocrine Neoplasm: Medullary Thyroid Carcinoma |journal=Acad Pathol |volume=5 |issue= |pages=2374289518775722 |date=2018 |pmid=29978018 |pmc=6024338 |doi=10.1177/2374289518775722 |url=}}</ref><ref name="pmid24037980">{{cite journal |vauthors=Roy M, Chen H, Sippel RS |title=Current understanding and management of medullary thyroid cancer |journal=Oncologist |volume=18 |issue=10 |pages=1093–100 |date=2013 |pmid=24037980 |pmc=3805151 |doi=10.1634/theoncologist.2013-0053 |url=}}</ref><ref name="pmid20664475">{{cite journal |vauthors=Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K |title=The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer |journal=Pancreas |volume=39 |issue=6 |pages=775–83 |date=August 2010 |pmid=20664475 |pmc=3419007 |doi=10.1097/MPA.0b013e3181ebb4f0 |url=}}</ref>
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Medullary thyroid carcinoma<ref name="pmid29978018">{{cite journal |vauthors=Segura S, Ramos-Rivera G, Suhrland M |title=Educational Case: Endocrine Neoplasm: Medullary Thyroid Carcinoma |journal=Acad Pathol |volume=5 |issue= |pages=2374289518775722 |date=2018 |pmid=29978018 |pmc=6024338 |doi=10.1177/2374289518775722 |url=}}</ref><ref name="pmid24037980">{{cite journal |vauthors=Roy M, Chen H, Sippel RS |title=Current understanding and management of medullary thyroid cancer |journal=Oncologist |volume=18 |issue=10 |pages=1093–100 |date=2013 |pmid=24037980 |pmc=3805151 |doi=10.1634/theoncologist.2013-0053 |url=}}</ref><ref name="pmid20664475">{{cite journal |vauthors=Chen H, Sippel RS, O'Dorisio MS, Vinik AI, Lloyd RV, Pacak K |title=The North American Neuroendocrine Tumor Society consensus guideline for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer |journal=Pancreas |volume=39 |issue=6 |pages=775–83 |date=August 2010 |pmid=20664475 |pmc=3419007 |doi=10.1097/MPA.0b013e3181ebb4f0 |url=}}</ref>
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* Dysphagia
* [[Dysphagia]]
* Hoarseness
* [[Hoarseness[[
* Respiratory difficulty
* Respiratory difficulty
* Flushing
* [[Flushing]]
* Diarrhea
* [[Diarrhea]]
* Weight loss
* [[Weight]] loss
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* Palpable neck mass  
* Palpable [[neck]] mass  


* Signs of Cushing syndrome  
* Signs of [[Cushing syndrome]]
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* ↑ Calcitonin
* ↑ [[Calcitonin]]
* ↑ Serum calcium  
* ↑ [[Serum]] [[calcium]]
* ↑ or - Cortisol
* ↑ or - [[Cortisol]]
* ↑ CEA level  
* ↑ [[CEA]] level  


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* White or gray in color
* White or gray in color
* Firm to palpation
* Firm to [[palpation]]
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* Nests of uniform cells
* Nests of uniform [[cells]]
* Deposition of stromal amyloid
* Deposition of stromal [[amyloid]]
* Granular chromatin
* Granular [[chromatin]]
* C-cell hyperplasia
* C-cell [[hyperplasia]]
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* Calcitonin
* [[Calcitonin]]
* Chromogranin A
* [[Chromogranin A]]
* Carcinoembryonic antigen (CEA)
* [[Carcinoembryonic antigen]] ([[CEA]])
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* Solid thyroid nodule (US)
* Solid [[thyroid nodule]] (US)
* CT scan/MRI and PET scan for metastatis
* [[CT scan]]/[[MRI]] and [[PET scan]] for metastatis
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* Genetic testing
* Genetic testing
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* FNA cytology with immunohisto-
* [[FNA]] [[cytology]] with immunohisto-
chemistry and  
chemistry and  


calcitonin levels
[[calcitonin]] levels
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* Associated with MEN 2A and 2B
* Associated with MEN 2A and 2B
* Familial association with RET mutations
* Familial association with [[RET]] mutations
* May present as Cushing syndrome
* May present as [[Cushing syndrome]]
|-
|-
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Pheochromocytoma<ref name="pmid24636754">{{cite journal |vauthors=Martucci VL, Pacak K |title=Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment |journal=Curr Probl Cancer |volume=38 |issue=1 |pages=7–41 |date=2014 |pmid=24636754 |pmc=3992879 |doi=10.1016/j.currproblcancer.2014.01.001 |url=}}</ref><ref name="pmid20541673">{{cite journal |vauthors=Kantorovich V, Pacak K |title=Pheochromocytoma and paraganglioma |journal=Prog. Brain Res. |volume=182 |issue= |pages=343–73 |date=2010 |pmid=20541673 |pmc=4714594 |doi=10.1016/S0079-6123(10)82015-1 |url=}}</ref><ref name="pmid19605896">{{cite journal |vauthors=Miller AD, Masek-Hammerman K, Dalecki K, Mansfield KG, Westmoreland SV |title=Histologic and immunohistochemical characterization of pheochromocytoma in 6 cotton-top tamarins (Saguinus oedipus) |journal=Vet. Pathol. |volume=46 |issue=6 |pages=1221–9 |date=November 2009 |pmid=19605896 |doi=10.1354/vp.09-VP-0022-M-FL |url=}}</ref><ref name="pmid19120142">{{cite journal |vauthors=Kantorovich V, Eisenhofer G, Pacak K |title=Pheochromocytoma: an endocrine stress mimicking disorder |journal=Ann. N. Y. Acad. Sci. |volume=1148 |issue= |pages=462–8 |date=December 2008 |pmid=19120142 |pmc=2693284 |doi=10.1196/annals.1410.081 |url=}}</ref><ref name="pmid25332315">{{cite journal |vauthors=Eisenhofer G, Peitzsch M |title=Laboratory evaluation of pheochromocytoma and paraganglioma |journal=Clin. Chem. |volume=60 |issue=12 |pages=1486–99 |date=December 2014 |pmid=25332315 |doi=10.1373/clinchem.2014.224832 |url=}}</ref>
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Pheochromocytoma<ref name="pmid24636754">{{cite journal |vauthors=Martucci VL, Pacak K |title=Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment |journal=Curr Probl Cancer |volume=38 |issue=1 |pages=7–41 |date=2014 |pmid=24636754 |pmc=3992879 |doi=10.1016/j.currproblcancer.2014.01.001 |url=}}</ref><ref name="pmid20541673">{{cite journal |vauthors=Kantorovich V, Pacak K |title=Pheochromocytoma and paraganglioma |journal=Prog. Brain Res. |volume=182 |issue= |pages=343–73 |date=2010 |pmid=20541673 |pmc=4714594 |doi=10.1016/S0079-6123(10)82015-1 |url=}}</ref><ref name="pmid19605896">{{cite journal |vauthors=Miller AD, Masek-Hammerman K, Dalecki K, Mansfield KG, Westmoreland SV |title=Histologic and immunohistochemical characterization of pheochromocytoma in 6 cotton-top tamarins (Saguinus oedipus) |journal=Vet. Pathol. |volume=46 |issue=6 |pages=1221–9 |date=November 2009 |pmid=19605896 |doi=10.1354/vp.09-VP-0022-M-FL |url=}}</ref><ref name="pmid19120142">{{cite journal |vauthors=Kantorovich V, Eisenhofer G, Pacak K |title=Pheochromocytoma: an endocrine stress mimicking disorder |journal=Ann. N. Y. Acad. Sci. |volume=1148 |issue= |pages=462–8 |date=December 2008 |pmid=19120142 |pmc=2693284 |doi=10.1196/annals.1410.081 |url=}}</ref><ref name="pmid25332315">{{cite journal |vauthors=Eisenhofer G, Peitzsch M |title=Laboratory evaluation of pheochromocytoma and paraganglioma |journal=Clin. Chem. |volume=60 |issue=12 |pages=1486–99 |date=December 2014 |pmid=25332315 |doi=10.1373/clinchem.2014.224832 |url=}}</ref>
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* Headaches
* Headaches
* Palpitations
* [[Palpitations]]
* Excessive sweating
* Excessive [[sweating]]
* Anxiety
* [[Anxiety]]
* Pallor
* [[Pallor]]
* Pain in chest/abdomen
* Pain in chest/abdomen
* Weakness, fatigue
* Weakness, fatigue
* Nausea/vomiting
* [[Nausea]]/[[vomiting]]
* Dizziness
* Dizziness
* Paresthesias
* [[Paresthesias]]
* Constipation (rarely diarrhea)
* [[Constipation]] (rarely [[diarrhea]])
* Visual disturbance
* Visual disturbance
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* Hypertension
* [[Hypertension]]
* Postural hypotension
* Postural [[hypotension]]
* Tachycardia or reflex bradycardia
* [[Tachycardia]] or reflex [[bradycardia]]
* Tremulousness
* Tremulousness
* Pallor
* [[Pallor]]
* Flushing (rare)  
* [[Flushing]] (rare)  
* Weight loss  
* [[Weight]] loss  
* Fasting hyperglycaemia
* Fasting [[hyperglycaemia]]
* Decreased GI motility
* Decreased [[GI]] motility
* Pallor
* [[Pallor]]
* ↑ Respiratory rate
* ↑ Respiratory rate
* Psychosis
* [[Psychosis]]
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* ↑ Plasma and urine catecholamines
* ↑ [[Plasma]] and [[urine]] [[catecholamines]]
* ↑ Plasma and urine metanephrines
* ↑ [[Plasma]] and [[urine]] [[metanephrines]]
* ↑ Chromogranin A
* ↑ [[Chromogranin A]]
* ↑ Plasma methoxytyramine
* ↑ [[Plasma]] [[methoxytyramine]]


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* Loosely cohesive clusters
* Loosely cohesive clusters
* Scattered tumor cells with prominent anisokaryosis, abundant eosinophilic granular cytoplasm and indistinct cell borders
* Scattered [[tumor]] [[cells]] with prominent anisokaryosis, abundant eosinophilic granular [[cytoplasm]] and indistinct [[cell]] borders
* Occasional binucleate cells
* Occasional binucleate [[cells]]
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* Chromogranin A (CGA)
* [[Chromogranin A]] (CGA)
* Protein gene product (PGP) 9.5
* Protein gene product (PGP) 9.5
* Synaptophysin (SYN)  
* [[Synaptophysin]] (SYN)  
* CD56 (also known as neural cell adhesion molecule or N-CAM)  
* [[CD56]] (also known as neural cell adhesion molecule or N-CAM)  
* Glial fibrillary acidic protein (GFAP)
* [[Glial fibrillary acidic protein]] ([[GFAP]])
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* Heterogeneous appearance, often with some cystic areas. Calcifications or hemorrhage may also be present (CT)
* Heterogeneous appearance, often with some [[cystic]] areas. [[Calcification]] or [[hemorrhage]] may also be present (CT)


* T2-bright lesions, with/without cystic or necrotic components (MRI)
* T2-bright lesions, with/without [[cystic]] or [[necrotic]] components ([[MRI]])
* Cystic or solid with necrotic areas or hemorrhages (U/S)
* Cystic or solid with necrotic areas or hemorrhages (U/S)
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* Genetic testing
* Genetic testing
* Provacative glucagon test
* Provacative [[glucagon]] test
* Clonidine suppression test
* [[Clonidine]] suppression test
* Metaiodobenzyl-guanidine scintigraphy
* Metaiodobenzyl-guanidine [[scintigraphy]]
* PET scan
* [[PET]] scan
* Octereoscan
* [[Octereoscan]]
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* Plasma and urine catecholamines & metanephrines combined with clinical findings
* [[Plasma]] and [[urine]] [[catecholamines]] & [[metanephrines]] combined with [[clinical]] findings
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* May mimic panic attack
* May mimic [[panic attack]]
* May be associated with  Von Hippel-Lindau disease, MEN type 2 and Neurofibromatosis type 1.
* May be associated with  [[Von Hippel-Lindau disease]], MEN type 2 and Neurofibromatosis type 1.
* Arise from the chromaffin cells
* Arise from the [[chromaffin cells]]
|-
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| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Merkel cell carcinoma<ref name="pmid29072302">{{cite journal |vauthors=Becker JC, Stang A, DeCaprio JA, Cerroni L, Lebbé C, Veness M, Nghiem P |title=Merkel cell carcinoma |journal=Nat Rev Dis Primers |volume=3 |issue= |pages=17077 |date=October 2017 |pmid=29072302 |pmc=6054450 |doi=10.1038/nrdp.2017.77 |url=}}</ref><ref name="pmid23116583">{{cite journal |vauthors=Han SY, North JP, Canavan T, Kim N, Yu SS |title=Merkel cell carcinoma |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1351–74 |date=December 2012 |pmid=23116583 |doi=10.1016/j.hoc.2012.08.007 |url=}}</ref><ref name="pmid28916903">{{cite journal |vauthors=Amaral T, Leiter U, Garbe C |title=Merkel cell carcinoma: Epidemiology, pathogenesis, diagnosis and therapy |journal=Rev Endocr Metab Disord |volume=18 |issue=4 |pages=517–532 |date=December 2017 |pmid=28916903 |doi=10.1007/s11154-017-9433-0 |url=}}</ref>
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Merkel cell carcinoma<ref name="pmid29072302">{{cite journal |vauthors=Becker JC, Stang A, DeCaprio JA, Cerroni L, Lebbé C, Veness M, Nghiem P |title=Merkel cell carcinoma |journal=Nat Rev Dis Primers |volume=3 |issue= |pages=17077 |date=October 2017 |pmid=29072302 |pmc=6054450 |doi=10.1038/nrdp.2017.77 |url=}}</ref><ref name="pmid23116583">{{cite journal |vauthors=Han SY, North JP, Canavan T, Kim N, Yu SS |title=Merkel cell carcinoma |journal=Hematol. Oncol. Clin. North Am. |volume=26 |issue=6 |pages=1351–74 |date=December 2012 |pmid=23116583 |doi=10.1016/j.hoc.2012.08.007 |url=}}</ref><ref name="pmid28916903">{{cite journal |vauthors=Amaral T, Leiter U, Garbe C |title=Merkel cell carcinoma: Epidemiology, pathogenesis, diagnosis and therapy |journal=Rev Endocr Metab Disord |volume=18 |issue=4 |pages=517–532 |date=December 2017 |pmid=28916903 |doi=10.1007/s11154-017-9433-0 |url=}}</ref>
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* Asymptomatic red-to-violet nodules
* Asymptomatic red-to-violet [[nodules]]
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* Violaceous nodule
* Violaceous [[nodule]]
* Ulcerated Nodule
* Ulcerated [[Nodule]]
* Lympadenopathy
* [[Lympadenopathy]]
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* Seropositivity for Merkel cell polyomavirus
* [[Seropositivity]] for Merkel cell polyomavirus
* Anti-ST antibodies
* Anti-ST [[antibodies]]
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* Dermal and subcutaneous nodule
* [[Dermal]] and [[subcutaneous]] [[nodule]]
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* Monomorphic cells with 3 main types — small-cell, trabecular and intermediate
* Monomorphic [[cells]] with 3 main types — small-cell, trabecular and intermediate
* May show necrosis & pleomorphism
* May show [[necrosis]] & [[pleomorphism]]


* Epidermotropism
* Epidermotropism
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* Chromogranin-A
* [[Chromogranin A]]
* Synaptophysin
* [[Synaptophysin]]
* Cytokeratin 20 (CK20)
* [[Cytokeratin]] 20 (CK20)
* Merkel cell polyomavirus (MCPyV) large T antigen
* Merkel cell polyomavirus (MCPyV) large T antigen
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* U/S, CT scan, MRI and PET scan for metastasis  
* U/S, [[CT scan]], [[MRI]] and [[PET scan]] for metastasis  
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* N/A
* N/A
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* Biopsy with immunohisto-chemistry
* [[Biopsy]] with immunohisto-chemistry
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* Higher mortality than melanoma  
* Higher mortality than [[melanoma]]
* ↑ Risk in immun-compromised
* ↑ Risk in immun-compromised
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* Fatigue
* Fatigue
* Sleep problems
* Sleep problems
* Bone or muscle pains  
* [[Bone]] or [[muscle]] pains  
* GERD
* [[GERD]]
* ↓ concentration
* ↓ concentration
* Renal colic
* [[Renal colic]]
* Constipation
* [[Constipation]]
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* Nephrolithiasis
* [[Nephrolithiasis]]
* Nephrocalcinosis
* [[Nephrocalcinosis]]
* osteopenia
* [[osteopenia]]
* osteoporosis
* [[osteoporosis]]
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* ↑ Serum and urinary Calcium
* ↑ [[Serum]] and [[urinary]] [[Calcium]]
* ↑ or N/L PTH
* ↑ or N/L [[PTH]]
*  
*  
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* Enlarged
* Enlarged
* smooth
* [[smooth]]
* Soft  
* Soft  
* Reddish brown in color
* Reddish brown in color
Line 187: Line 187:


* May be multinodular and irregular
* May be multinodular and irregular
* Proliferation of a single cell type with solid to pseudo-glandular, follicular, and acinar structure
* Proliferation of a single [[cell]] type with solid to pseudo-glandular, follicular, and acinar structure
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* Chromogranin
* [[Chromogranin]]
* Parathyroid hormone
* [[Parathyroid hormone]]
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* Enlarged gland on U/S and <sup>99m</sup>Tc-sestamibi scintigraphy
* Enlarged gland on [[U/S]] and <sup>99m</sup>Tc-sestamibi [[scintigraphy]]
* Single photon emission computed tomography
* Single [[photon emission computed tomography]]
|Staining for  
|Staining for  
* bcl-1  
* bcl-1  
Line 200: Line 200:
* parafibromin
* parafibromin
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* Biopsy
* [[Biopsy]]
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* Associated with MEN
* Associated with MEN
Line 209: Line 209:
* Headaches
* Headaches
* Palpitations
* Palpitations
* Tinnitus
* [[Tinnitus]]
* Decreased hearing
* Decreased hearing
* Localized pain  
* Localized pain  
* GI & intracranial hemorrhage   
* [[GI]] & [[intracranial hemorrhage]]  
* Cardiomyopathy Generalized weakness
* [[Cardiomyopathy]] Generalized weakness
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* Pallor  
* Pallor  
* Hypertension  
* [[Hypertension]]
* Localized mass
* Localized mass
* Orthostasis  
* [[Orthostasis]]
* Incidentalomas
* [[Incidentalomas]]
* Cranial nerve palsies
* [[Cranial nerve]] [[palsies]]
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* ↑ Plasma and urine catecholamines
* ↑ [[Plasma]] and [[urine]] [[catecholamines]]
* ↑ Plasma and urine metanephrines
* ↑ [[Plasma]] and [[urine]] [[metanephrines]]
* ↑ Chromogranin A
* ↑ [[Chromogranin A]]
* ↑ Plasma methoxytyramine
* ↑ [[Plasma]] [[methoxytyramine]]
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* Clear margins
* Clear margins
* Encapsulated
* Encapsulated
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* Honeycomb or nests patterns separated by broad bands of fibrous tissue
* Honeycomb or nests patterns separated by broad bands of fibrous [[tissue]]
* Prominent vascularization
* Prominent [[vascularization]]
* Argyrophilic fibers
* Argyrophilic fibers
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* Chromogranin A
* [[Chromogranin A]]
* Synaptophysin
* [[Synaptophysin]]
* CD56  
* [[CD56]]
* S-100
* [[S-100]]
* Cytokeratin
* [[Cytokeratin]]
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* Mass with an un-enhanced density  
* Mass with an un-enhanced [[density]]
* Internal Hemorrhage  
* Internal [[Hemorrhage]]
* Cystic changes
* Cystic changes
* Necrosis
* [[Necrosis]]
* Internal calcifications
* Internal [[calcification]]
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* Genetic testing
* Genetic testing
* Provacative glucagon test
* Provacative [[glucagon]] test
* Clonidine suppression test
* [[Clonidine]] suppression test
* Metaiodobenzyl-guanidine scintigraphy
* Metaiodobenzyl-guanidine [[scintigraphy]]
* PET scan
* [[PET]] scan
* Octereoscan
* [[Octereoscan]]
|N/A
|N/A
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* Paragangliomas are similar to pheochromo-cytomas but arise outside adrenal medulla
* Paragangliomas are similar to pheochromo-cytomas but arise outside [[adrenal medulla]]
|-
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| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Pituitary gland tumor<ref name="pmid28264912">{{cite journal |vauthors=Asa SL, Casar-Borota O, Chanson P, Delgrange E, Earls P, Ezzat S, Grossman A, Ikeda H, Inoshita N, Karavitaki N, Korbonits M, Laws ER, Lopes MB, Maartens N, McCutcheon IE, Mete O, Nishioka H, Raverot G, Roncaroli F, Saeger W, Syro LV, Vasiljevic A, Villa C, Wierinckx A, Trouillas J |title=From pituitary adenoma to pituitary neuroendocrine tumor (PitNET): an International Pituitary Pathology Club proposal |journal=Endocr. Relat. Cancer |volume=24 |issue=4 |pages=C5–C8 |date=April 2017 |pmid=28264912 |doi=10.1530/ERC-17-0004 |url=}}</ref>s<ref name="pmid29275530">{{cite journal |vauthors=Manojlovic-Gacic E, Engström BE, Casar-Borota O |title=Histopathological classification of non-functioning pituitary neuroendocrine tumors |journal=Pituitary |volume=21 |issue=2 |pages=119–129 |date=April 2018 |pmid=29275530 |pmc=5849671 |doi=10.1007/s11102-017-0855-1 |url=}}</ref><ref name="pmid19021842">{{cite journal |vauthors=Ibrahim M, Yousef M, Bohnen N, Eisbruch A, Parmar H |title=Primary carcinoid tumor of the skull base: case report and review of the literature |journal=J Neuroimaging |volume=20 |issue=4 |pages=390–2 |date=October 2010 |pmid=19021842 |doi=10.1111/j.1552-6569.2008.00317.x |url=}}</ref><ref name="pmid28695048">{{cite journal |vauthors=Nasi D, Perano D, Ghadirpour R, Iaccarino C, Servadei F, Romano A |title=Primary pituitary neuroendocrine tumor: Case report and literature review |journal=Surg Neurol Int |volume=8 |issue= |pages=101 |date=2017 |pmid=28695048 |pmc=5473081 |doi=10.4103/sni.sni_450_16 |url=}}</ref>
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Pituitary gland tumor<ref name="pmid28264912">{{cite journal |vauthors=Asa SL, Casar-Borota O, Chanson P, Delgrange E, Earls P, Ezzat S, Grossman A, Ikeda H, Inoshita N, Karavitaki N, Korbonits M, Laws ER, Lopes MB, Maartens N, McCutcheon IE, Mete O, Nishioka H, Raverot G, Roncaroli F, Saeger W, Syro LV, Vasiljevic A, Villa C, Wierinckx A, Trouillas J |title=From pituitary adenoma to pituitary neuroendocrine tumor (PitNET): an International Pituitary Pathology Club proposal |journal=Endocr. Relat. Cancer |volume=24 |issue=4 |pages=C5–C8 |date=April 2017 |pmid=28264912 |doi=10.1530/ERC-17-0004 |url=}}</ref>s<ref name="pmid29275530">{{cite journal |vauthors=Manojlovic-Gacic E, Engström BE, Casar-Borota O |title=Histopathological classification of non-functioning pituitary neuroendocrine tumors |journal=Pituitary |volume=21 |issue=2 |pages=119–129 |date=April 2018 |pmid=29275530 |pmc=5849671 |doi=10.1007/s11102-017-0855-1 |url=}}</ref><ref name="pmid19021842">{{cite journal |vauthors=Ibrahim M, Yousef M, Bohnen N, Eisbruch A, Parmar H |title=Primary carcinoid tumor of the skull base: case report and review of the literature |journal=J Neuroimaging |volume=20 |issue=4 |pages=390–2 |date=October 2010 |pmid=19021842 |doi=10.1111/j.1552-6569.2008.00317.x |url=}}</ref><ref name="pmid28695048">{{cite journal |vauthors=Nasi D, Perano D, Ghadirpour R, Iaccarino C, Servadei F, Romano A |title=Primary pituitary neuroendocrine tumor: Case report and literature review |journal=Surg Neurol Int |volume=8 |issue= |pages=101 |date=2017 |pmid=28695048 |pmc=5473081 |doi=10.4103/sni.sni_450_16 |url=}}</ref>
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* Focal neurological deficit
* Focal neurological deficit
* Intracranial hypertension
* [[Intracranial hypertension]]
* Hypopituitarism
* [[Hypopituitarism]]
* Endocrine symptoms
* [[Endocrine]] symptoms
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* Focal neurological deficit
* Focal neurological deficit
* Intracranial hypertension
* [[Intracranial hypertension]]
* Hypopituitarism
* [[Hypopituitarism]]
* Endocrine signs
* [[Endocrine]] signs
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* ↓ Pituitary hormones
* ↓ [[Pituitary]] [[hormones]]
* ↑ in specific hormone in functional tumors
* ↑ in specific hormone in functional [[tumors]]
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* Micro or macroadenomas
* Micro or macroadenomas
* Well-defined
* Well-defined
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* uniform nuclear morphology, stippled chromatin and inconspicuous nucleoli
* uniform nuclear [[morphology]], stippled [[chromatin]] and inconspicuous nucleoli
* Dense vs sparsely granulated
* [[Dense]] vs sparsely granulated
* Crooke's hyaline change
* Crooke's hyaline change
* Null cell adenomas  
* Null cell [[adenomas]]
|
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* Synaptophysin  
* [[Synaptophysin]]
* Chromogranin A)
* [[Chromogranin A]]
* Hormone stains include prolactin, ACTH, GH, TSH, LH, FSH and alpha subunit
* [[Hormone]] stains include [[prolactin]], [[ACTH]], [[GH]], [[TSH]], [[LH]], [[FSH]] and alpha subunit
* Reticulin
* [[Reticulin]]
* CAM5.2
* CAM5.2
* SF1, PIT-1 or T-PIT
* SF1, PIT-1 or T-PIT
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* Sellar/suprasellar mass
* Sellar/suprasellar mass
* Diffuse homogeneous enhancement following contrast administration (MRI)
* Diffuse homogeneous enhancement following contrast administration ([[MRI]])
* CT
* [[CT]]
|
|
* 1C-5<sup>-</sup> hydroxytryptophan PET
* 1C-5<sup>-</sup> hydroxytryptophan [[PET]]
* Somatostatin Receptor scintigraphy
* [[Somatostatin]] [[Receptor]] [[scintigraphy]]
* Pituitary hormone levels
* [[Pituitary]] [[hormone]] levels
* Thyroid hormones and cortisol level
* ]]Thyroid hormones]] and [[cortisol]] level
|
|
* N/A
* N/A
Line 304: Line 304:
* Painless scrotal mass & swelling
* Painless scrotal mass & swelling
* Scrotal pain  
* Scrotal pain  
* Carcinoid syndrome   
* [[Carcinoid syndrome]]    
|
|
* Firm painless or painful scrotal mass & swelling
* Firm painless or painful scrotal mass & swelling
* Flushing
* Flushing
* Hydrocele
* [[Hydrocele]]
|
|
* ↑ Serum NSE
* ↑ [[Serum]] NSE
* ↑ Serum vanillylmandelic acid
* ↑ [[Serum]] vanillylmandelic acid
* ↑ Serum serotonin and serotonin metabolites
* ↑ [[Serum]] [[serotonin]] and serotonin [[metabolites]]
|
|
* Well-defined
* Well-defined
* Non-capsulated
* Non-capsulated
* Calcified cyst
* Calcified [[cyst]]
* Hemorrhage & necrosis
* [[Hemorrhage]] & [[necrosis]]
|
|
* Presence of argentaffin and argyrophilic cells
* Presence of argentaffin and argyrophilic cells
* Monomorphous polygonal cells
* Monomorphous polygonal [[cells]]
* Eosinophilic cytoplasm  
* Eosinophilic [[cytoplasm]]
* Finely dispersed chromatin  
* Finely dispersed [[chromatin]]
|
|
* Cytokeratin
* [[Cytokeratin]]
* Chromogranin
* [[Chromogranin]]
* Synaptophysin
* [[Synaptophysin]]
* Serotonin
* [[Serotonin]]
* NSE
* [[NSE]]
* Vimentin
* [[Vimentin]]
|
|
* Well-defined solid hypoechoic mass (U/S)  
* Well-defined solid hypoechoic mass (U/S)  
* Dense punctate calcifications (U/S)
* Dense punctate [[calcification]] (U/S)
|For metastasis and grading:
|For [[metastasis]] and [[grading]]:
* CT scan
* [[CT scan]]
* Octreotide scintigraphy
* [[Octreotide]] [[scintigraphy]]
* Pan GI endoscopy
* Pan [[GI]] [[endoscopy]]
|
|
* Scrotal U/S with immunohisto-chemistry
* Scrotal U/S with immunohisto-chemistry
|
|
* May be associated with testicular teratoma
* May be associated with [[testicular teratoma]]
* Normal serum levels of testicular tumor markers
* Normal [[serum]] levels of testicular [[tumor]] markers
|-
|-
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Neuroendocrine tumors of the ovaries<ref name="pmid27508272">{{cite journal |vauthors=Orsi NM, Menon M |title=Primary ovarian neuroendocrine tumor arising in association with a mature cystic teratoma: A case report |journal=Gynecol Oncol Rep |volume=17 |issue= |pages=83–5 |date=August 2016 |pmid=27508272 |pmc=4971231 |doi=10.1016/j.gore.2016.07.003 |url=}}</ref><ref name="pmid29326972">{{cite journal |vauthors=Kaiho-Sakuma M, Toyoshima M, Watanabe M, Toki A, Kameda S, Minato T, Niikura H, Yaegashi N |title=Aggressive neuroendocrine tumor of the ovary with multiple metastases treated with everolimus: A case report |journal=Gynecol Oncol Rep |volume=23 |issue= |pages=20–23 |date=February 2018 |pmid=29326972 |pmc=5760241 |doi=10.1016/j.gore.2018.01.002 |url=}}</ref><ref name="pmid11993694">{{cite journal |vauthors=Eichhorn JH, Young RH |title=Neuroendocrine tumors of the genital tract |journal=Am. J. Clin. Pathol. |volume=115 Suppl |issue= |pages=S94–112 |date=June 2001 |pmid=11993694 |doi=10.1309/64CW-WKGK-49EF-BYD1 |url=}}</ref>
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Neuroendocrine tumors of the ovaries<ref name="pmid27508272">{{cite journal |vauthors=Orsi NM, Menon M |title=Primary ovarian neuroendocrine tumor arising in association with a mature cystic teratoma: A case report |journal=Gynecol Oncol Rep |volume=17 |issue= |pages=83–5 |date=August 2016 |pmid=27508272 |pmc=4971231 |doi=10.1016/j.gore.2016.07.003 |url=}}</ref><ref name="pmid29326972">{{cite journal |vauthors=Kaiho-Sakuma M, Toyoshima M, Watanabe M, Toki A, Kameda S, Minato T, Niikura H, Yaegashi N |title=Aggressive neuroendocrine tumor of the ovary with multiple metastases treated with everolimus: A case report |journal=Gynecol Oncol Rep |volume=23 |issue= |pages=20–23 |date=February 2018 |pmid=29326972 |pmc=5760241 |doi=10.1016/j.gore.2018.01.002 |url=}}</ref><ref name="pmid11993694">{{cite journal |vauthors=Eichhorn JH, Young RH |title=Neuroendocrine tumors of the genital tract |journal=Am. J. Clin. Pathol. |volume=115 Suppl |issue= |pages=S94–112 |date=June 2001 |pmid=11993694 |doi=10.1309/64CW-WKGK-49EF-BYD1 |url=}}</ref>
|
|
* Lower abdominal pain & swelling
* Lower abdominal pain & swelling
* Carcinoid syndrome
* [[Carcinoid syndrome]]
|
|
* Abdominal and/or pelvic mass
* Abdominal and/or pelvic mass
* Ascites
* [[Ascites]]
* Flushing
* Flushing
|
|
* ↑ Urinary 5-hydroxyindole-acetic acid
* ↑ [[Urinary]] 5-hydroxyindole-acetic acid
* ↑ Chromogranin A
* ↑ [[Chromogranin A]]
* ↑ or -  CA 125
* ↑ or -  [[CA 125]]
|
|
* Well-defined
* Well-defined
* Smooth
* Smooth
* Solid with cystic areas
* Solid with [[cystic]] areas
|
|
* Insular pattern with nested areas  
* Insular pattern with nested areas  
* Cells in groups and trabeculae arrangement
* [[Cells]] in groups and trabeculae arrangement
* Mild to moderate nuclear pleomorphism
* Mild to moderate nuclear [[pleomorphism]]
|
|
* CD 56
* [[CD56]]
* Synaptophysin
* [[Synaptophysin]]
* Chromogranin
* [[Chromogranin]]
|
|
* Solid ovarian mass with significant enhancement (MRI)
* Solid [[ovarian]] mass with significant enhancement ([[MRI]])
* Ovarian solid mass (U/S)
* [[Ovarian]] solid mass (U/S)
|
|
For metastasis and grading:
For [[metastasis]] and [[grading]]:
* CT scan
* [[CT scan]]
* Octreotide scintigraphy
* [[Octreotide]] [[scintigraphy]]
* Pan GI endoscopy
* Pan [[GI endoscopy]]
|
|
* N/A
* N/A
|
|
* CDX2 may distinguish primary ovarian NET from metastatic NET from GI tract
* CDX2 may distinguish primary [[ovarian]] NET from [[metastatic]] NET from [[GI tract]]
|-
|-
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Thymic neuroendocrine cancer<ref name="pmid29201448">{{cite journal |vauthors=Bohnenberger H, Dinter H, König A, Ströbel P |title=Neuroendocrine tumors of the thymus and mediastinum |journal=J Thorac Dis |volume=9 |issue=Suppl 15 |pages=S1448–S1457 |date=November 2017 |pmid=29201448 |pmc=5690954 |doi=10.21037/jtd.2017.02.02 |url=}}</ref><ref name="pmid10884805">{{cite journal |vauthors=Moran CA, Suster S |title=Neuroendocrine carcinomas (carcinoid tumor) of the thymus. A clinicopathologic analysis of 80 cases |journal=Am. J. Clin. Pathol. |volume=114 |issue=1 |pages=100–10 |date=July 2000 |pmid=10884805 |doi=10.1309/3PDN-PMT5-EQTM-H0CD |url=}}</ref><ref name="pmid28602157">{{cite journal |vauthors=Boubacar E, Atsame-Ebang G, Rabiou S, Fatimazahra A, Mazti A, Sidibé IS, Tahiri L, Hammas N, Yassine O, Mohamed S, Laila C, Hinde EF |title=Thymic large cell neuroendocrine carcinoma - a rare and aggressive tumor: a case report |journal=J Med Case Rep |volume=11 |issue=1 |pages=155 |date=June 2017 |pmid=28602157 |pmc=5467048 |doi=10.1186/s13256-017-1331-2 |url=}}</ref><ref name="pmid30446840">{{cite journal |vauthors=Domen H, Hida Y, Sato M, Takahashi H, Ishikawa T, Shionoya Y, Hashimoto M, Nishiyama K, Aoki Y, Inoko K, Furukawa S, Ichinokawa K, Yamada H |title=Resected thymic large cell neuroendocrine carcinoma: report of a case |journal=Surg Case Rep |volume=4 |issue=1 |pages=133 |date=November 2018 |pmid=30446840 |pmc=6240022 |doi=10.1186/s40792-018-0540-2 |url=}}</ref>
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Thymic neuroendocrine cancer<ref name="pmid29201448">{{cite journal |vauthors=Bohnenberger H, Dinter H, König A, Ströbel P |title=Neuroendocrine tumors of the thymus and mediastinum |journal=J Thorac Dis |volume=9 |issue=Suppl 15 |pages=S1448–S1457 |date=November 2017 |pmid=29201448 |pmc=5690954 |doi=10.21037/jtd.2017.02.02 |url=}}</ref><ref name="pmid10884805">{{cite journal |vauthors=Moran CA, Suster S |title=Neuroendocrine carcinomas (carcinoid tumor) of the thymus. A clinicopathologic analysis of 80 cases |journal=Am. J. Clin. Pathol. |volume=114 |issue=1 |pages=100–10 |date=July 2000 |pmid=10884805 |doi=10.1309/3PDN-PMT5-EQTM-H0CD |url=}}</ref><ref name="pmid28602157">{{cite journal |vauthors=Boubacar E, Atsame-Ebang G, Rabiou S, Fatimazahra A, Mazti A, Sidibé IS, Tahiri L, Hammas N, Yassine O, Mohamed S, Laila C, Hinde EF |title=Thymic large cell neuroendocrine carcinoma - a rare and aggressive tumor: a case report |journal=J Med Case Rep |volume=11 |issue=1 |pages=155 |date=June 2017 |pmid=28602157 |pmc=5467048 |doi=10.1186/s13256-017-1331-2 |url=}}</ref><ref name="pmid30446840">{{cite journal |vauthors=Domen H, Hida Y, Sato M, Takahashi H, Ishikawa T, Shionoya Y, Hashimoto M, Nishiyama K, Aoki Y, Inoko K, Furukawa S, Ichinokawa K, Yamada H |title=Resected thymic large cell neuroendocrine carcinoma: report of a case |journal=Surg Case Rep |volume=4 |issue=1 |pages=133 |date=November 2018 |pmid=30446840 |pmc=6240022 |doi=10.1186/s40792-018-0540-2 |url=}}</ref>
|
|
* Chest pain
* Chest pain
* Cough
* [[Cough]]
* Respiratory distress  
* Respiratory distress  
* Cushing’s syndrome
* [[Cushing’s syndrome]]
* Carcinoid syndrome (rare)
* [[Carcinoid syndrome]] (rare)
|
|
* ↓ Breath sounds
* ↓ Breath sounds
* Flushing
* Flushing
* Cushing’s syndrome
* [[Cushing’s syndrome]]
* Hypercalcemia
* [[Hypercalcemia]]
|
|
* Usually normal
* Usually normal
* May see
* May see
** ↑ Serum calcium & ACTH
** ↑ [[Serum]] [[calcium]] & [[ACTH]]
** ↑ Serum serotonin and its metabolites
** ↑ [[Serum]] [[serotonin]] and its metabolites
|
|
* Solid mass
* Solid mass
* Hemorrhage & necrosis
* [[Hemorrhage]] & [[necrosis]]
* Calcification  
* [[Calcification]]
* Gross invasion  
* Gross invasion  
|
|
* Small cells with scant cytoplasm
* Small cells with scant [[cytoplasm]]
* Trabeculae
* Trabeculae
* Solid nests
* Solid nests
* Vascular invasion  
* [[Vascular]] invasion  
* Fine granular or vesicular chromatin
* Fine granular or vesicular [[chromatin]]
|
|
* Keratins (AE1/AE3, CAM5.2)
* [[Keratins]] (AE1/AE3, CAM5.2)
* Synaptophysin
* [[Synaptophysin]]
* Chromogranin
* [[Chromogranin]]
* NSE
* [[NSE]]
* CD 56
* [[CD56]]
|
|
* Mediastinal mass
* Mediastinal mass
Line 420: Line 420:
* Adjacent compression
* Adjacent compression
|
|
For metastasis and grading:
For [[metastasis]] and [[grading]]:
* PET scan
* [[PET scan]]
* Endoscopy
* [[Endoscopy]]
|
|
* H & E morphology
* H & E [[morphology]]
|
|
* May co-exist with thymoma
* May co-exist with [[thymoma]]
* Associated with MEN-1
* Associated with MEN-1
*  
*  
Line 433: Line 433:
|
|
* Abdominal pain
* Abdominal pain
* Nausea/vomiting
* [[Nausea]]/vomiting
* Bloating
* Bloating
* Bleeding
* Bleeding
* Carcinoid syndrome (rare)
* [[Carcinoid syndrome]] (rare)
|
|
*  Jaundice
* [[Jaundice]]
* Weight loss
* Weight loss
* Carcinoid syndrome (rare)
* [[Carcinoid syndrome]] (rare)
|
|
* ↑ Urinary 5-hydroxyindole-acetic acid
* ↑ Urinary 5-hydroxyindole-acetic acid
* ↑ Chromogranin A
* ↑ [[Chromogranin A]]
|
|
* N/A
* N/A
|
|
* Submucosal tumor
* Submucosal [[tumor]]
* Solid, insular (nesting), trabecular or glandular pattern
* Solid, insular (nesting), trabecular or glandular pattern
* Peripheral pallisading
* Peripheral pallisading
* Lymphovascular invasion  
* Lymphovascular invasion  
* Mucin (glandular pattern)
* [[Mucin]](glandular pattern)
|
|
* Synaptophysin
* [[Synaptophysin]]
* Chromogranin A
* [[Chromogranin A]]
* CK
* CK
* CEA
* [[CEA]]
* Leu7
* Leu7
* Fontana-Masson
* Fontana-Masson
|
|
* Desmoplastic fibrosis (CT scan)
* Desmoplastic fibrosis ([[CT scan]])
* Capsule enteroscopy (CE)
* Capsule enteroscopy (CE)
* Balloon-assisted enteroscopy
* Balloon-assisted enteroscopy
* MRI
* [[MRI]]


|
|
* Endoscopy
* [[Endoscopy]]
* PET with gallium 68–labeled octreotide
* [[PET]] with gallium 68–labeled octreotide
* Indium-labelled octreotide (<sup>111</sup>In-octreotide)
* Indium-labelled octreotide (<sup>111</sup>In-octreotide)
|
|
* Upper gastrointestinal endoscopy with biopsy
* Upper [[gastrointestinal endoscopy]] with [[biopsy]]
|
|
* Ileum is mot common location
* [[Ileum]] is mot common location
* Poorer prognosis
* Poorer [[prognosis]]
|-
|-
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Appendix neuroendocrine tumors<ref name="pmid28761642">{{cite journal |vauthors=Abdelaal A, El Ansari W, Al-Bozom I, Khawar M, Shahid F, Aleter A, Abunuwar MR, El-Menyar A |title=Frequency, characteristics and outcomes of appendicular neuroendocrine tumors: A cross-sectional study from an academic tertiary care hospital |journal=Ann Med Surg (Lond) |volume=21 |issue= |pages=20–24 |date=September 2017 |pmid=28761642 |doi=10.1016/j.amsu.2017.07.043 |url=}}</ref><ref name="pmid23365545">{{cite journal |vauthors=Holt N, Grønbæk H |title=Goblet cell carcinoids of the appendix |journal=ScientificWorldJournal |volume=2013 |issue= |pages=543696 |date=2013 |pmid=23365545 |doi=10.1155/2013/543696 |url=}}</ref><ref name="pmid26793027">{{cite journal |vauthors=Ozcelik CK, Turanli S, Bozdogan N, Dibekoglu C |title=Clinical experience in appendiceal neuroendocrine neoplasms |journal=Contemp Oncol (Pozn) |volume=19 |issue=5 |pages=410–3 |date=2015 |pmid=26793027 |pmc=4709403 |doi=10.5114/wo.2015.56008 |url=}}</ref>
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Appendix neuroendocrine tumors<ref name="pmid28761642">{{cite journal |vauthors=Abdelaal A, El Ansari W, Al-Bozom I, Khawar M, Shahid F, Aleter A, Abunuwar MR, El-Menyar A |title=Frequency, characteristics and outcomes of appendicular neuroendocrine tumors: A cross-sectional study from an academic tertiary care hospital |journal=Ann Med Surg (Lond) |volume=21 |issue= |pages=20–24 |date=September 2017 |pmid=28761642 |doi=10.1016/j.amsu.2017.07.043 |url=}}</ref><ref name="pmid23365545">{{cite journal |vauthors=Holt N, Grønbæk H |title=Goblet cell carcinoids of the appendix |journal=ScientificWorldJournal |volume=2013 |issue= |pages=543696 |date=2013 |pmid=23365545 |doi=10.1155/2013/543696 |url=}}</ref><ref name="pmid26793027">{{cite journal |vauthors=Ozcelik CK, Turanli S, Bozdogan N, Dibekoglu C |title=Clinical experience in appendiceal neuroendocrine neoplasms |journal=Contemp Oncol (Pozn) |volume=19 |issue=5 |pages=410–3 |date=2015 |pmid=26793027 |pmc=4709403 |doi=10.5114/wo.2015.56008 |url=}}</ref>
|
|
* Asymptomatic
* Asymptomatic
* Acute appendicitis  
* Acute [[appendicitis]]
** localized pain
** localized pain
** shifting pain
** shifting pain
** nausea, vomiting, anorexia
** nausea, vomiting, [[anorexia]]
** rectal bleeding
** rectal bleeding
* Carcinoid syndrome
* [[Carcinoid syndrome]]
|
|
* Asymptomatic
* Asymptomatic
* Acute appendicitis
* Acute [[appendicitis]]
** localized or generalized abdominal tenderness
** localized or generalized abdominal tenderness
** fever
** [[fever]]
** abdominal distension
** abdominal distension
 
* [[Carcinoid syndrome]]
* Carcinoid syndrome
|
|
* Normal  
* Normal  
* ↑ WBCs (appendicitis)
* ↑ [[WBCs]] ([[appendicitis]])
* ↑ Bilirubin (appendicitis)
* ↑ [[Bilirubin]] ([[appendicitis]])
* ↑ Urinary 5-hydroxyindole-acetic acid
* ↑ Urinary 5-hydroxyindole-acetic acid
* ↑ chromogranin A
* ↑ [[chromogranin A]]
|
|
* Ill-defined
* Ill-defined
* Firm
* Firm
* Nodular
* [[Nodular]]
*  
*  
|
|
* Infiltrates muscularis propria & periappendiceal fat
* Infiltrates muscularis propria & periappendiceal fat
* Solid clusters  resembling goblet cells or signet ring cells & eosinophilic cytoplasm  
* Solid clusters  resembling goblet cells or signet ring cells & eosinophilic [[cytoplasm]]
* Scattered Paneth cells  
* Scattered Paneth cells  
* Single cells
* Single [[cells]]
|
|
* Synaptophysin
* [[Synaptophysin]]
* Chromogranin A
* [[Chromogranin A]]
* CK
* CK
* CEA
* CEA
Line 516: Line 515:
* CAM 5.2
* CAM 5.2
|
|
* Thickened appendix wall (U/S and CT)
* Thickened [[appendix]] wall (U/S and [[CT]])
* Appendicitis (U/S and CT)
* [[Appendicitis]] (U/S and [[CT]])
** inflamed appendix
** inflamed [[appendix]]
** peri-appendicular fluid
** peri-appendicular fluid
|
|
* Chromogranin A enzyme linked immunoassay
* [[Chromogranin A]] [[enzyme]] linked [[immunoassay]]
* FDG-PET
* FDG-[[PET]]
* <sup>111</sup>In-labelled octreotide scintigraphy
* <sup>111</sup>In-labelled [[octreotide]] [[scintigraphy]]
|
|
* Histopathology
* [[Histopathology]]
|
|
* N/A
* N/A
Line 532: Line 531:
|
|
* Abdominal pain
* Abdominal pain
* Diarrhea
* [[Diarrhea]]
* Hematochezia
* Hematochezia
* Nausea
* [[Nausea]]
* Asymptomatic
* Asymptomatic
* Carcinoid syndrome
* [[Carcinoid syndrome]]
|
|
* Abdominal tenderness
* Abdominal [[tenderness]]
* Weight loss
* Weight loss
* Anemia related
* [[Anemia]] related
|
|
* ↑ chromogranin A
* ↑ [[chromogranin A]]
* ↑ Urinary 5-hydroxyindole-acetic acid (rare)
* ↑ [[Urinary]] 5-hydroxyindole-acetic acid (rare)
* ↓ Hb
* ↓ [[H]]b
|
|
* Flat or depressed lesion
* Flat or depressed [[lesion]]
* Plaque or polypod
* Plaque or polypod
|
|
* Islands, glandular or trabeculae
* Islands, glandular or trabeculae
* Granular cytoplasm
* Granular [[cytoplasm]]
* salt and pepper chromatin
* Salt and pepper [[chromatin]]
* Lymphovascular invasion
* Lymphovascular invasion
|
|
* Synaptophysin
* [[Synaptophysin]]
* Chromogranin
* [[Chromogranin]]
|
|
* Wall thickening
* Wall thickening
* Lumen narrowing
* Lumen narrowing
|
|
* Colonoscopy
* [[Colonoscopy]]
* Chromogranin A enzyme linked immunoassay
* [[Chromogranin A]] [[enzyme]] linked [[immunoassay]]
* PET scan
* [[PET]] scan
* SSTR (somatostatin receptors) scintigraphy
* SSTR ([[somatostatin]] [[receptors]]) [[scintigraphy]]
|
|
* Biopsy and histopathology
* [[Biopsy]] and [[histopathology]]
|
|
* Cecum is most common location
* [[Cecum]] is most common location
* ↑ prevalence in IBD patient population  
* ↑ prevalence in IBD [[patient]] [[population]]
|-
|-
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Rectum neuroendocrine tumors<ref name="pmid28280618">{{cite journal |vauthors=Uppin MS, Uppin SG, Sunil CS, Hui M, Paul TR, Bheerappa N |title=Clinicopathologic study of neuroendocrine tumors of gastroenteropancreatic tract: a single institutional experience |journal=J Gastrointest Oncol |volume=8 |issue=1 |pages=139–147 |date=February 2017 |pmid=28280618 |pmc=5334039 |doi=10.21037/jgo.2016.12.08 |url=}}</ref><ref name="pmid29181074">{{cite journal |vauthors=Gut P, Waligórska-Stachura J, Czarnywojtek A, Sawicka-Gutaj N, Bączyk M, Ziemnicka K, Woliński K, Zybek A, Fischbach J, Ruchała M |title=Hindgut neuroendocrine neoplasms - characteristics and prognosis |journal=Arch Med Sci |volume=13 |issue=6 |pages=1427–1432 |date=October 2017 |pmid=29181074 |pmc=5701690 |doi=10.5114/aoms.2017.64979 |url=}}</ref><ref name="pmid29232390">{{cite journal |vauthors=Koenig A, Krug S, Mueller D, Barth PJ, Koenig U, Scharf M, Ellenrieder V, Michl P, Moll R, Homayunfar K, Kann PH, Stroebel P, Gress TM, Rinke A |title=Clinicopathological hallmarks and biomarkers of colorectal neuroendocrine neoplasms |journal=PLoS ONE |volume=12 |issue=12 |pages=e0188876 |date=2017 |pmid=29232390 |pmc=5726657 |doi=10.1371/journal.pone.0188876 |url=}}</ref><ref name="pmid30235718">{{cite journal |vauthors=Yuan H, Yang Y, Wang W, Cheng Y |title=A case report of neuroendocrine tumor (G3) at lower rectum with liver metastasis |journal=Medicine (Baltimore) |volume=97 |issue=38 |pages=e12423 |date=September 2018 |pmid=30235718 |pmc=6160253 |doi=10.1097/MD.0000000000012423 |url=}}</ref><ref name="pmid21160637">{{cite journal |vauthors=Roy P, Chetty R |title=Goblet cell carcinoid tumors of the appendix: An overview |journal=World J Gastrointest Oncol |volume=2 |issue=6 |pages=251–8 |date=June 2010 |pmid=21160637 |pmc=2998842 |doi=10.4251/wjgo.v2.i6.251 |url=}}</ref>
| colspan="2" style="background: #DCDCDC; padding: 5px; text-align: center;" |Rectum neuroendocrine tumors<ref name="pmid28280618">{{cite journal |vauthors=Uppin MS, Uppin SG, Sunil CS, Hui M, Paul TR, Bheerappa N |title=Clinicopathologic study of neuroendocrine tumors of gastroenteropancreatic tract: a single institutional experience |journal=J Gastrointest Oncol |volume=8 |issue=1 |pages=139–147 |date=February 2017 |pmid=28280618 |pmc=5334039 |doi=10.21037/jgo.2016.12.08 |url=}}</ref><ref name="pmid29181074">{{cite journal |vauthors=Gut P, Waligórska-Stachura J, Czarnywojtek A, Sawicka-Gutaj N, Bączyk M, Ziemnicka K, Woliński K, Zybek A, Fischbach J, Ruchała M |title=Hindgut neuroendocrine neoplasms - characteristics and prognosis |journal=Arch Med Sci |volume=13 |issue=6 |pages=1427–1432 |date=October 2017 |pmid=29181074 |pmc=5701690 |doi=10.5114/aoms.2017.64979 |url=}}</ref><ref name="pmid29232390">{{cite journal |vauthors=Koenig A, Krug S, Mueller D, Barth PJ, Koenig U, Scharf M, Ellenrieder V, Michl P, Moll R, Homayunfar K, Kann PH, Stroebel P, Gress TM, Rinke A |title=Clinicopathological hallmarks and biomarkers of colorectal neuroendocrine neoplasms |journal=PLoS ONE |volume=12 |issue=12 |pages=e0188876 |date=2017 |pmid=29232390 |pmc=5726657 |doi=10.1371/journal.pone.0188876 |url=}}</ref><ref name="pmid30235718">{{cite journal |vauthors=Yuan H, Yang Y, Wang W, Cheng Y |title=A case report of neuroendocrine tumor (G3) at lower rectum with liver metastasis |journal=Medicine (Baltimore) |volume=97 |issue=38 |pages=e12423 |date=September 2018 |pmid=30235718 |pmc=6160253 |doi=10.1097/MD.0000000000012423 |url=}}</ref><ref name="pmid21160637">{{cite journal |vauthors=Roy P, Chetty R |title=Goblet cell carcinoid tumors of the appendix: An overview |journal=World J Gastrointest Oncol |volume=2 |issue=6 |pages=251–8 |date=June 2010 |pmid=21160637 |pmc=2998842 |doi=10.4251/wjgo.v2.i6.251 |url=}}</ref>
Line 574: Line 573:
* Asymptomatic
* Asymptomatic
* Rectal pain
* Rectal pain
* Pruritus
* [[Pruritus]]
* Hematochezia
* [[Hematochezia]]
* Constipation
* [[Constipation]]
* Carcinoid syndrome (rare)  
* [[Carcinoid syndrome]] (rare)  
|
|
* Abdominal tenderness
* Abdominal [[tenderness]]
* Weight loss
* Weight loss
* Carcinoid signs (rare)
* Carcinoid signs (rare)
|
|
* ↑ chromogranin A
* ↑ [[Chromogranin A]]
* ↑ Urinary 5-hydroxyindole-acetic acid (rare)
* ↑ [[Urinary]] 5-hydroxyindole-acetic acid (rare)
* ↓ Hb
* ↓ [[Hb]]
|
|
* Small and multiple
* Small and multiple
* Nodules or polypoid
* [[Nodules]] or polypoid
|
|
* Islands, glandular or trabeculae
* Islands, glandular or trabeculae
* Granular cytoplasm
* Granular [[cytoplasm]]
* salt and pepper chromatin
* Salt and pepper [[chromatin]]
* Mucin secretion (rare)
* [[Mucin]] secretion (rare)
|
|
* Synaptophysin
* [[Synaptophysin]]
* Chromogranin
* [[Chromogranin]]
* Neuron-specific enolase
* Neuron-specific enolase
* CD56
* [[CD56]]
|
|
* Rectal mass or nodule
* Rectal mass or [[nodule]]
* Ulcerative mass (high grade)
* Ulcerative mass (high grade)
* Hemorrhage
* [[Hemorrhage]]
* Liver mass (metastatic)
* [[Liver]] mass ([[metastatic]])
|
|
* Colonoscopy
* [[Colonoscopy]]
* Chromogranin A enzyme linked immunoassay
* [[Chromogranin A]] [[enzyme]] linked [[immunoassay]]
* PET scan
* [[PET]] scan
* SSTR (somatostatin receptors) scintigraphy
* SSTR ([[somatostatin]] [[receptors]]) [[scintigraphy]]
|
|
* Biopsy and histopathology
* [[Biopsy]] and [[histopathology]]
|
|
* May exist with adenocarcinoma  
* May exist with [[adenocarcinoma]]
* Exhibits malignant potential  
* Exhibits [[malignant]] potential  
|-
|-
| rowspan="4" style="background: #DCDCDC; padding: 5px; text-align: center;" |Gastric neuroendocrine tumors
| rowspan="4" style="background: #DCDCDC; padding: 5px; text-align: center;" |Gastric neuroendocrine tumors
Line 619: Line 618:
;
;
|
|
* Dyspepsia
* [[Dyspepsia]]
* Abdominal discomfort and/or pain
* Abdominal discomfort and/or pain
* Anemia related
* [[Anemia]] related
* Neurological symptoms  
* Neurological symptoms  
|
|
* Weight loss
* Weight loss
* Pallor  
* [[Pallor]]
* Neurological signs  
* Neurological signs  
|
|
* ↑ Gastrin level
* ↑ [[Gastrin]] level
* ↑ chromogranin A
* ↑ [[chromogranin A]]
* ↓ Hb
* ↓ [[Hb]]
* ↓ Serum iron
* ↓ [[Serum iron]]
* ↓ Serum B12 levels
* ↓ [[Serum B12]] levels


*
*
|
|
* Flattened gastric folds
* Flattened gastric folds
* Multiple polyps or nodules
* Multiple polyps or [[nodules]]
|
|
* Dense core granules
* Dense core [[granules]]
* Glandular or trabecular  
* Glandular or trabecular  
* Salt and pepper chromatin
* Salt and pepper [[chromatin]]
|
|
* Keratins
* Keratins
* Chromogranin
* [[Chromogranin]]
* NSE
* [[NSE]]
* Synaptophysin
* [[Synaptophysin]]
* VMAT 2  
* VMAT 2  


*
*
|
|
* Well demarcated hypoechoiec lesions
* Well demarcated hypoechoiec [[lesions]]
* Mucosal / submucosal
* [[Mucosal]] / submucosal
* Regular borders
* Regular borders
|
|
* Gastroscopy
* [[Gastroscopy]]
* pH on gastric aspirate
* [[pH]] on gastric aspirate
* Axial CT
* Axial [[CT]]
* Somatostatin receptor imaging
* [[Somatostatin]] [[receptor]] [[imaging]]
|
|
* N/A
* N/A
|
|
* Chronic atrophic gastritis → gastric achlorhydria and hypergastrinaemia → proliferation of ECL cells → type 1 G-NETs
* [[Chronic atrophic gastritis]] → gastric [[achlorhydria]] and [[hypergastrinaemia]] → proliferation of ECL cells → type 1 G-NETs
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: left;" |Type II GNET<ref name="pmid28355596">{{cite journal |vauthors=Sundin A, Arnold R, Baudin E, Cwikla JB, Eriksson B, Fanti S, Fazio N, Giammarile F, Hicks RJ, Kjaer A, Krenning E, Kwekkeboom D, Lombard-Bohas C, O'Connor JM, O'Toole D, Rockall A, Wiedenmann B, Valle JW, Vullierme MP |title=ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: Radiological, Nuclear Medicine & Hybrid Imaging |journal=Neuroendocrinology |volume=105 |issue=3 |pages=212–244 |date=2017 |pmid=28355596 |doi=10.1159/000471879 |url=}}</ref><ref name="pmid29207862" /><ref name="pmid21443889">{{cite journal |vauthors=Pritchard DM |title=Zollinger-Ellison syndrome: still a diagnostic challenge in the 21st century? |journal=Gastroenterology |volume=140 |issue=5 |pages=1380–3 |date=May 2011 |pmid=21443889 |doi=10.1053/j.gastro.2011.03.026 |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: left;" |Type II GNET<ref name="pmid28355596">{{cite journal |vauthors=Sundin A, Arnold R, Baudin E, Cwikla JB, Eriksson B, Fanti S, Fazio N, Giammarile F, Hicks RJ, Kjaer A, Krenning E, Kwekkeboom D, Lombard-Bohas C, O'Connor JM, O'Toole D, Rockall A, Wiedenmann B, Valle JW, Vullierme MP |title=ENETS Consensus Guidelines for the Standards of Care in Neuroendocrine Tumors: Radiological, Nuclear Medicine & Hybrid Imaging |journal=Neuroendocrinology |volume=105 |issue=3 |pages=212–244 |date=2017 |pmid=28355596 |doi=10.1159/000471879 |url=}}</ref><ref name="pmid29207862" /><ref name="pmid21443889">{{cite journal |vauthors=Pritchard DM |title=Zollinger-Ellison syndrome: still a diagnostic challenge in the 21st century? |journal=Gastroenterology |volume=140 |issue=5 |pages=1380–3 |date=May 2011 |pmid=21443889 |doi=10.1053/j.gastro.2011.03.026 |url=}}</ref>
|
|
* Abdominal pain and/or discomfort
* Abdominal pain and/or discomfort
* Severe watery diarrhea  
* Severe watery [[diarrhea]]
* GI bleed
* [[GI]] bleed
* Anemia related
* [[Anemia]] related
* GERD
* [[GERD]]
|
|
* Abdominal tenderness
* Abdominal [[tenderness]]
* Hypercalcemia
* [[Hypercalcemia]]
* Anemia related
* [[Anemia]] related
|
|
* ↑ Gastrin or gastrin precursors
* ↑ [[Gastrin]] or [[gastrin]] precursors
* ↑ Serum calcium
* ↑ [[Serum calcium]]
* ↑ Chromogranin A
* ↑ [[Chromogranin A]]
|
|
* N/A
* N/A
|
|
* Dense core granules
* Dense core [[granules]]
* Glandular or trabecular  
* Glandular or trabecular  
* Salt and pepper chromatin
* Salt and pepper [[chromatin]]
|
|
* Keratins
* Keratins
* Chromogranin
* [[Chromogranin]]
* NSE
* NSE
* Synaptophysin
* [[Synaptophysin]]
|
|
* Hypertrophied gastric mucosal folds
* Hypertrophied gastric mucosal folds
* Well demarcated hypoechoiec lesions
* Well demarcated hypoechoiec [[lesions]]
|
|
* Secretin test
* [[Secretin]] test
* pH on gastric aspirate
* [[pH]] on gastric aspirate
* <sup>111</sup>Indium Octreotide scintigraphy
* <sup>111</sup>Indium [[Octreotide]] [[scintigraphy]]
* <sup>68</sup>Gallium-DOTATOC/DOTATATE PET/CT
* <sup>68</sup>Gallium-DOTATOC/DOTATATE [[PET]]/[[CT]]
|
|
* N/A
* N/A
|
|
* Caused by gastrinoma  
* Caused by [[gastrinoma]]
* Most patient have MEN-1 syndrome → evaluate
* Most patient have MEN-1 syndrome → evaluate
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: left;" |Type III GNET<ref name="pmid292078622">{{cite journal |vauthors=Chin JL, O'Toole D |title=Diagnosis and Management of Upper Gastrointestinal Neuroendocrine Tumors |journal=Clin Endosc |volume=50 |issue=6 |pages=520–529 |date=November 2017 |pmid=29207862 |pmc=5719910 |doi=10.5946/ce.2017.181 |url=}}</ref><ref name="pmid292578542">{{cite journal |vauthors=Dias AR, Azevedo BC, Alban LBV, Yagi OK, Ramos MFKP, Jacob CE, Barchi LC, Cecconello I, Ribeiro U, Zilberstein B |title=GASTRIC NEUROENDOCRINE TUMOR: REVIEW AND UPDATE |journal=Arq Bras Cir Dig |volume=30 |issue=2 |pages=150–154 |date=2017 |pmid=29257854 |pmc=5543797 |doi=10.1590/0102-6720201700020016 |url=}}</ref><ref name="pmid24628514">{{cite journal |vauthors=Basuroy R, Srirajaskanthan R, Prachalias A, Quaglia A, Ramage JK |title=Review article: the investigation and management of gastric neuroendocrine tumours |journal=Aliment. Pharmacol. Ther. |volume=39 |issue=10 |pages=1071–84 |date=May 2014 |pmid=24628514 |doi=10.1111/apt.12698 |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: left;" |Type III GNET<ref name="pmid292078622">{{cite journal |vauthors=Chin JL, O'Toole D |title=Diagnosis and Management of Upper Gastrointestinal Neuroendocrine Tumors |journal=Clin Endosc |volume=50 |issue=6 |pages=520–529 |date=November 2017 |pmid=29207862 |pmc=5719910 |doi=10.5946/ce.2017.181 |url=}}</ref><ref name="pmid292578542">{{cite journal |vauthors=Dias AR, Azevedo BC, Alban LBV, Yagi OK, Ramos MFKP, Jacob CE, Barchi LC, Cecconello I, Ribeiro U, Zilberstein B |title=GASTRIC NEUROENDOCRINE TUMOR: REVIEW AND UPDATE |journal=Arq Bras Cir Dig |volume=30 |issue=2 |pages=150–154 |date=2017 |pmid=29257854 |pmc=5543797 |doi=10.1590/0102-6720201700020016 |url=}}</ref><ref name="pmid24628514">{{cite journal |vauthors=Basuroy R, Srirajaskanthan R, Prachalias A, Quaglia A, Ramage JK |title=Review article: the investigation and management of gastric neuroendocrine tumours |journal=Aliment. Pharmacol. Ther. |volume=39 |issue=10 |pages=1071–84 |date=May 2014 |pmid=24628514 |doi=10.1111/apt.12698 |url=}}</ref>
|
|
* Dyspepsia
* [[Dyspepsia]]
* Abdominal discomfort and/or pain
* Abdominal discomfort and/or pain
* May present with carciniod syndrome
* May present with [[carciniod syndrome]]
|
|
* Weight loss
* Weight loss
* Anaemia related
* [[Anaemia]] related
* Flushing
* Flushing
|
|
* ↓ Hb
* ↓ [[Hb]]
* ↑ Urinary 5-hydroxyindole-acetic acid (rare)
* ↑ [[Urinary]] 5-hydroxyindole-acetic acid (rare)
|
|
* Large lesion with poor margins
* Large lesion with poor margins
Line 722: Line 721:
* High grade
* High grade
* Invasive  
* Invasive  
* Dense core granules
* Dense core [[granules]]
* Glandular or trabecular  
* Glandular or trabecular  
|
|
* Keratins
* Keratins
* NSE
* NSE
* Synaptophysin
* [[Synaptophysin]]
|
|
* Absence of surface villiform architecture
* Absence of surface villiform architecture
* Single large lesion
* Single large [[lesion]]
* Ulcerated appearance
* Ulcerated appearance
|
|
* Gastroscopy
* [[Gastroscopy]]
* CT
* [[CT]]
* <sup>111</sup>Indium octreotide scintigraphy
* <sup>111</sup>Indium [[octreotide]] [[scintigraphy]]
|
|
* Biopsy and histopathological studies
* [[Biopsy]] and histopathological studies
|
|
* Early involvement of regional lymph nodes
* Early involvement of regional [[lymph nodes]]
* May present with metastatic disease at diagnosed
* May present with metastatic disease at diagnosis
|-
|-
| style="background: #DCDCDC; padding: 5px; text-align: left;" |Type IV GNET<ref name="pmid24415864">{{cite journal |vauthors=Li TT, Qiu F, Qian ZR, Wan J, Qi XK, Wu BY |title=Classification, clinicopathologic features and treatment of gastric neuroendocrine tumors |journal=World J. Gastroenterol. |volume=20 |issue=1 |pages=118–25 |date=January 2014 |pmid=24415864 |doi=10.3748/wjg.v20.i1.118 |url=}}</ref><ref name="pmid12673427">{{cite journal |vauthors=Hosoya Y, Nagai H, Koinuma K, Yasuda Y, Kaneko Y, Saito K |title=A case of aggressive neuroendocrine carcinoma of the stomach |journal=Gastric Cancer |volume=6 |issue=1 |pages=55–9 |date=2003 |pmid=12673427 |doi=10.1007/s101200300007 |url=}}</ref><ref name="pmid15477709">{{cite journal |vauthors=Rindi G, Klöppel G |title=Endocrine tumors of the gut and pancreas tumor biology and classification |journal=Neuroendocrinology |volume=80 Suppl 1 |issue= |pages=12–5 |date=2004 |pmid=15477709 |doi=10.1159/000080733 |url=}}</ref>
| style="background: #DCDCDC; padding: 5px; text-align: left;" |Type IV GNET<ref name="pmid24415864">{{cite journal |vauthors=Li TT, Qiu F, Qian ZR, Wan J, Qi XK, Wu BY |title=Classification, clinicopathologic features and treatment of gastric neuroendocrine tumors |journal=World J. Gastroenterol. |volume=20 |issue=1 |pages=118–25 |date=January 2014 |pmid=24415864 |doi=10.3748/wjg.v20.i1.118 |url=}}</ref><ref name="pmid12673427">{{cite journal |vauthors=Hosoya Y, Nagai H, Koinuma K, Yasuda Y, Kaneko Y, Saito K |title=A case of aggressive neuroendocrine carcinoma of the stomach |journal=Gastric Cancer |volume=6 |issue=1 |pages=55–9 |date=2003 |pmid=12673427 |doi=10.1007/s101200300007 |url=}}</ref><ref name="pmid15477709">{{cite journal |vauthors=Rindi G, Klöppel G |title=Endocrine tumors of the gut and pancreas tumor biology and classification |journal=Neuroendocrinology |volume=80 Suppl 1 |issue= |pages=12–5 |date=2004 |pmid=15477709 |doi=10.1159/000080733 |url=}}</ref>
;
;
|
|
* Dyspepsia
* [[Dyspepsia]]
* Abdominal discomfort and/or pain
* Abdominal discomfort and/or pain
|
|
* Weight loss
* Weight loss
* Anaemia related
* [[Anaemia]] related
|
|
* ↑ Gastrin or gastrin precursors
* ↑ [[Gastrin]] or [[gastrin]] precursors
* ↑ Serum calcium
* ↑ [[Serum calcium]]
|
|
* Invasion of adjacent structures
* Invasion of adjacent structures
* Large solid lesion
* Large solid [[lesion]]
|
|
* Solid structure
* Solid structure
* Necrosis
* [[Necrosis]]
* Atypia
* [[Atypia]]
* Invasive
* Invasive
|
|
* Synaptophysin
* [[Synaptophysin]]
* NSE
* NSE
* PGP9.5 positive
* PGP9.5
|
|
* N/A
* N/A
Line 770: Line 769:
*
*
|
|
* Gastroscopy
* [[Gastroscopy]]
* CT
* [[CT]]
* <sup>111</sup>Indium octreotide scintigraphy
* <sup>111</sup>Indium [[octreotide]] [[scintigraphy]]
|
|
* Biopsy and histopathological studies
* [[Biopsy]] and histopathological studies
|Structural abnormality → no HCL secretion → achlorhydria →
|Structural abnormality → no [[HCL]] secretion → [[achlorhydria]]
hypergastrinemia → hyperplasia
[[hypergastrinemia]] [[hyperplasia]]
|-
|-
| rowspan="5" style="background: #DCDCDC; padding: 5px; text-align: center;" |Duodenal neuroendocrine tumors
| rowspan="5" style="background: #DCDCDC; padding: 5px; text-align: center;" |Duodenal neuroendocrine tumors

Revision as of 15:11, 30 January 2019

Neuroendocrine tumor Clinical manifestations Dianosis Gold

standard

Other features
Symptoms Signs Blood & Urine Gross Histology Immunohistochemistry Imaging Others
Medullary thyroid carcinoma[1][2][3]
  • Genetic testing

chemistry and

calcitonin levels

  • Associated with MEN 2A and 2B
  • Familial association with RET mutations
  • May present as Cushing syndrome
Pheochromocytoma[4][5][6][7][8]
  • N/A
  • Loosely cohesive clusters
  • Scattered tumor cells with prominent anisokaryosis, abundant eosinophilic granular cytoplasm and indistinct cell borders
  • Occasional binucleate cells
  • T2-bright lesions, with/without cystic or necrotic components (MRI)
  • Cystic or solid with necrotic areas or hemorrhages (U/S)
Merkel cell carcinoma[9][10][11]
  • Epidermotropism
  • N/A
  • Biopsy with immunohisto-chemistry
  • Higher mortality than melanoma
  • ↑ Risk in immun-compromised
Parathyroid adenoma[12][13][14]
  • Enlarged
  • smooth
  • Soft
  • Reddish brown in color
  • Discrete with thin fibrous capsule
  • May be multinodular and irregular
  • Proliferation of a single cell type with solid to pseudo-glandular, follicular, and acinar structure
Staining for
  • bcl-1
  • Ki67
  • p27
  • parafibromin
  • Associated with MEN
  • Associated with HPRT
Paraganglioma[15][16][17][18]
  • Clear margins
  • Encapsulated
  • Honeycomb or nests patterns separated by broad bands of fibrous tissue
  • Prominent vascularization
  • Argyrophilic fibers
N/A
  • Paragangliomas are similar to pheochromo-cytomas but arise outside adrenal medulla
Pituitary gland tumor[19]s[20][21][22]
  • Micro or macroadenomas
  • Well-defined
  • Sellar/suprasellar mass
  • Diffuse homogeneous enhancement following contrast administration (MRI)
  • CT
  • N/A
  • To learn more about pituitary neuroendocrine tumors, click here.
  • To learn more about pituitary hormones, click here.
Neuroendocrine tumors of the testicles[23][24][25]
  • Firm painless or painful scrotal mass & swelling
  • Flushing
  • Hydrocele
  • Presence of argentaffin and argyrophilic cells
  • Monomorphous polygonal cells
  • Eosinophilic cytoplasm
  • Finely dispersed chromatin
  • Well-defined solid hypoechoic mass (U/S)
  • Dense punctate calcification (U/S)
For metastasis and grading:
  • Scrotal U/S with immunohisto-chemistry
Neuroendocrine tumors of the ovaries[26][27][28]
  • Abdominal and/or pelvic mass
  • Ascites
  • Flushing
  • Well-defined
  • Smooth
  • Solid with cystic areas
  • Insular pattern with nested areas
  • Cells in groups and trabeculae arrangement
  • Mild to moderate nuclear pleomorphism

For metastasis and grading:

  • N/A
Thymic neuroendocrine cancer[29][30][31][32]
  • Mediastinal mass
  • Necrotic areas
  • Unclear margins
  • Adjacent compression

For metastasis and grading:

  • May co-exist with thymoma
  • Associated with MEN-1
Jejuno-Ileal neuroendocrine tumors[33][34][35][36]
  • N/A
  • Submucosal tumor
  • Solid, insular (nesting), trabecular or glandular pattern
  • Peripheral pallisading
  • Lymphovascular invasion
  • Mucin(glandular pattern)
  • Desmoplastic fibrosis (CT scan)
  • Capsule enteroscopy (CE)
  • Balloon-assisted enteroscopy
  • MRI
  • Endoscopy
  • PET with gallium 68–labeled octreotide
  • Indium-labelled octreotide (111In-octreotide)
Appendix neuroendocrine tumors[37][38][39]
  • Infiltrates muscularis propria & periappendiceal fat
  • Solid clusters resembling goblet cells or signet ring cells & eosinophilic cytoplasm
  • Scattered Paneth cells
  • Single cells
  • N/A
Colon neuroendocrine tumors[40][41][42][43]
  • Flat or depressed lesion
  • Plaque or polypod
  • Islands, glandular or trabeculae
  • Granular cytoplasm
  • Salt and pepper chromatin
  • Lymphovascular invasion
  • Wall thickening
  • Lumen narrowing
Rectum neuroendocrine tumors[44][40][45][46][47]
  • Abdominal tenderness
  • Weight loss
  • Carcinoid signs (rare)
  • Small and multiple
  • Nodules or polypoid
Gastric neuroendocrine tumors Type I GNET[48][49][50]
  • Dyspepsia
  • Abdominal discomfort and/or pain
  • Anemia related
  • Neurological symptoms
  • Weight loss
  • Pallor
  • Neurological signs
  • Flattened gastric folds
  • Multiple polyps or nodules
  • Well demarcated hypoechoiec lesions
  • Mucosal / submucosal
  • Regular borders
  • N/A
Type II GNET[48][49][51]
  • N/A
  • Hypertrophied gastric mucosal folds
  • Well demarcated hypoechoiec lesions
  • N/A
  • Caused by gastrinoma
  • Most patient have MEN-1 syndrome → evaluate
Type III GNET[52][53][54]
  • Weight loss
  • Anaemia related
  • Flushing
  • Hb
  • Urinary 5-hydroxyindole-acetic acid (rare)
  • Large lesion with poor margins
  • Ulcerated appearance
  • High grade
  • Invasive
  • Dense core granules
  • Glandular or trabecular
  • Absence of surface villiform architecture
  • Single large lesion
  • Ulcerated appearance
  • Biopsy and histopathological studies
  • Early involvement of regional lymph nodes
  • May present with metastatic disease at diagnosis
Type IV GNET[55][56][41]
  • Invasion of adjacent structures
  • Large solid lesion
  • N/A
  • Biopsy and histopathological studies
Structural abnormality → no HCL secretion → achlorhydria

hypergastrinemiahyperplasia

Duodenal neuroendocrine tumors Gastrinoma[57][58][59][60]
  • Atypical PUD
  • Abdominal pain and/or cramps
  • Esophagitis
  • GERD
  • Upper GI blee
  • Secretory diarrhea
  • Abdominal tenderness
  • Hypercalcemia
  • ↑ Gastrin or gastrin precursors
  • ↑ Serum calcium
  • ↑ Chromogranin A
  • N/A
  • Trabeculae
  • Solid nests
  • Presence of argentaffin and argyrophilic cells
  • Chromogranin A
  • Neuron-specific enolase
  • Synaptophysin
  • Gastrin
  • Diffuse erosive esophagitis, gastric and duodenal ulcer (Endoscopic gastroduo-denoscopy)
  • Enhancing lobulated lesion (CT)
  • Gastrin levels
  • Pancreatic polypeptide levels
  • Insulin levels
  • Glucagon levels
  • VIP levels
  • PET with gallium 68–labeled octreotide
  • Provocative secretin test & biopsy
  • Associated with MEN-1
Somatostatinomas[61][62][63][64]
  • Abdominal pain
  • Jaundice
  • Gastrointestinal bleeding
  • Diarrhea
  • Weight loss
  • Hypoglycemia related
  • Jaundice
  • Diabetes mellitus
  • Gallstones
  • Hypochlorhydria
  • Hypoglycemia related
  • ↑ Somatostatin
  • Anemia
  • Hyper or hypoglycemia
  • N/A
  • Acinar structures
  • Trabeculae
  • Psammoma body
  • Abundant fine granular cytoplasm
  • Salt and pepper chromatin
  • Chromogranin A
  • Neuron-specific enolase
  • Synaptophysin
  • Leu-7
  • Isodense
  • Hypervascular lesion
  • Dual phase CT
  • MRI
  • Endoscopic ultrasonography
  • 68Ga-DOTATATE
  • Octreoscan
  • Gastrin levels
  • Pancreatic polypeptide levels
  • Insulin levels
  • Glucagon levels
  • N/A
  • Associated with NF-1
Gangliocytic paraganglionomas[65][66][67][68]
  • GI bleed
  • Abdominal pain
  • Anemia related
  • Carcinoid syndrome (rare)
  • Anemia related
  • May mimic other neuroendocrine tumors such as somatostatinoma
  • ↓ Hb
  • ↑ Chromogranin A
  • May have ↑ urinary 5-hydroxyindole-acetic acid or serum somatostatin
  • Solid tumor
  • Ulceration
  • Sessile or polypod
  • Covered by the smooth
  • Spindle cells, ganglion-like cells and epithelioid cells
  • Nests and trabeculae
  • Chromogranine
  • AE1–3
  • Cytokeratins
  • S100
  • Neuron-specific enolase
  • Synaptophysin
  • CD56
  • Ampullary smooth mass (CT scan and endoscopic ultrasonography)
  • Endoscopic ultrasonography-guided FNA
  • Serum serotonin assay
  • Biopsy with immunohisto-chemistry
  • Associated with NF-1
Nonfunctioning NET[69]
  • Asymptomatic
  • GI bleed
  • Abdominal pain
  • Anemia related
  • Anemia related
  • ↓ Hb
  • Solid tumor
  • Trabecular or microlobular structure
  • Mucosal-submucosal tumor
  • Chromogranin A
  • Synaptophysin
  • Hormones such as gastrin, somatostatin, serotonin
  • CT scan
  • Endoscopic ultrasonography
  • Endoscopic ultrasonography-guided FNA
  • Biopsy with immunohisto-chemistry
  • May produce hormones but do not secrete
neuroendocrine carcinomas[70][71][72][73]
  • Upper abdominal pain
  • Abdominal Discomfort
  • Obstruction
  • Gastrointestinal bleeding
  • Constitutional
  • Anemia related
  • ↑ Chromogranin A
  • Progastrin-releasing peptide (pro-GRP)
  • Cytokeratin fragments (CKfr, CK8, 18, 19)
  • Solid tumor
  • Ulceration
  • Poorly differentiated
  • Necrosis
  • Lymphovascular invasion
  • Marked pleomorphism
  • Chromogranin A
  • Neuron-specific enolase
  • Synaptophysin
  • Protein-gene-product 9.5 (PGP 9.5)
  • CD56 (N-CAM)
  • Polypoid intraluminal mass (CT scan)
  • MRI
  • FDG-PET
  • Somatostatin receptor imaging (SRI)
  • Biopsy with immunohisto-chemistry
  • Typically non-functioning
  • May exist with adenocarcinoma
Pancreatic neuroendocrine tumours Gastrinoma[57][58][59][60]
  • Atypical PUD
  • Abdominal pain and/or cramps
  • Esophagitis
  • GERD
  • Upper GI blee
  • Secretory diarrhea
  • Abdominal tenderness
  • Hypercalcemia
  • ↑ Gastrin or gastrin precursors
  • ↑ Serum calcium
  • ↑ Chromogranin A
  • N/A
  • Trabeculae
  • Solid nests
  • Presence of argentaffin and argyrophilic cells
  • Chromogranin A
  • Neuron-specific enolase
  • Synaptophysin
  • Pancreastatin
  • Gastrin
  • Diffuse erosive esophagitis, gastric and duodenal ulcer (Endoscopic gastroduo-denoscopy)
  • Enhancing lobulated lesion (CT)
  • Gastrin levels
  • Insulin levels
  • Glucagon levels
  • VIP levels
  • PET with gallium 68–labeled octreotide
  • Indium-labelled octreotide (111In-octreotide)
  • Provocative secretin test & biopsy
  • Associated with MEN-1
Insulinoma[74][75][76][77]
  • Visual disturbances
  • Weakness
  • Seizures
  • Palpitations
  • Sweating
  • Hyperphagia/obesity
  • Tachycardia
  • Altered mental state
  • Amnesia
  • Confusion
  • Coma
  • Tremors
  • ↓ Blood sugar level
  • ↑ or inappropriately normal insulin level
  • ↑ Plasma proinsulin
  • Firm
  • Well-circumscribed nodule
  • Encapsulated mass
  • Solid or gyriform patterns
  • May be associated with nesidioblastosis
  • Amyloid
  • Chromogranin A
  • Neuron-specific enolase
  • Synaptophysin
  • CD56
  • Insulin
  • Hypervascular lesion (CT)
  • Calcification (malignant)
  • Homogeneously hypoechoic (endoscopic U/S)
  • MRI
  • Transabdominal U/S
  • C peptidelevel
  • Urinary Sulfonylurea
  • Pancreatic angiography
  • Transhepatic portal venous sampling (THPVS)
  • Intraoperative U/S
  • Intra-arterial calcium stimulation (IAC)
  • Associated with MEN-1
Glucagonoma[78][79][80][81]
  • Rash
  • Weight loss
  • Anemia
  • Diarrhea
  • Abdominal pain
  • Nausea or anorexia
  • DVT or PE related
  • Neurological symptoms
  • Weakness or fatigue
  • Necrotizing migratory erythema
  • Glossitis
  • Stomatitis
  • Cheilitis
  • Anemia related
  • DVT or PE related
  • Onychodystrophy
  • Peripheral edema
  • Depression
  • ↑ Glucagon levels
  • Diabetes or impaired fasting glucose
  • Hypoaminoacidemia
  • ↓ Serum zinc
  • ↓ Hb
  • N/A
  • Dense core granules
  • Nests pattern
  • Salt and pepper chromatin
  • Amphophilic cytoplasm
  • PGP 9.5
  • CAM 5.2
  • anti-glucagon
  • Chromogranin A
  • Neuron-specific enolase
  • Synaptophysin
  • Helical multiphasic contrast-enhanced CT scan
  • MRI
  • Somatostatin receptor scintigraphy (SRS)
  • FPET imaging technique with DOTA peptides
  • Serum parathyroid hormone, gastrin, insulin, pancreatic polypeptide, serotonin, VIP, prolactin and ACTH levels
  • Skin biopsy
  • N/A
  • Associated with MEN-1
Somatostatinoma[61][62][63]
  • Abdominal pain
  • Jaundice
  • Gastrointestinal bleeding
  • Diarrhea
  • Weight loss
  • Hypoglycemia related
  • Jaundice
  • Diabetes mellitus
  • Gallstones
  • Hypochlorhydria
  • Hypoglycemia related
  • ↑ Somatostatin
  • Anemia
  • Hyper or hypoglycemia
  • N/A
  • Acinar structures
  • Trabeculae
  • Psammoma body
  • Abundant fine granular cytoplasm
  • Salt and pepper chromatin
  • Chromogranin A
  • Neuron-specific enolase
  • Synaptophysin
  • Leu-7
  • Isodense
  • Hypervascular lesion
  • Dual phase CT
  • MRI
  • Endoscopic ultrasonography
  • 68Ga-DOTATATE
  • Octreoscan
  • Gastrin levels
  • Pancreatic polypeptide levels
  • Insulin levels
  • Glucagon levels
  • N/A
  • Associated with MEN-1
VIPoma[82][83][84][85]
  • Secretory diarrhea with odorless and tea colored stools
  • Lethargy
  • Nausea & vomiting
  • Muscle weakness & cramps
  • Tetany
  • Dehydration
  • Flushing
  • Cardiac arrhythmias, myopathy, tetany and hypovolemic shock (crisis)
  • ↑ VIP levels
  • ↑ Serum pancreatic polypeptide level
  • Hypokalemia
  • Hypomagnesemia
  • Hypochlorhydria
  • Hyperglycemia
  • Hypercalcemia
  • Encapsulated
  • Necrosis
  • Nested cells
  • Trabeculae
  • Secretory granules
  • Granular cytoplasm
  • Chromogrannin A
  • VIP
  • Pancreatic polypeptide
  • Encapsulated and necrotic mass (CT scan & MRI)
  • Somatostatin receptor scintigraphy
  • Endoscopic U/S
  • FPET imaging with DOTA peptides
  • VIP radioimmunoassay
  • Celiac, superior mesentericand renal angiography
  • Levels of other PNETs
  • VIP radioimmunoassay with clinical manifestations
  • Associated with MEN-1
ACTHoma[86][87][88][89]
  • Weight gain
  • Acne
  • Ophthalmologic discomfort
  • Extremity weakness
  • Hypertension
  • Moon facies
  • Easy bruising
  • Buffalo-hump
  • Abdominal striae
  • ↑ Serum ACTH
  • ↑ Serum and urinary cortisol
  • Diabetes mellitus
  • Hypokalemic alkalosis
  • Firm
  • Well circumscribed
  • Encapsulated
  • Nested cells
  • Trabeculae
  • Secretory granules
  • Lymphovascular invasion
  • Synaptophysin
  • Chromogranin A
  • ACTH
  • Hypoechoic, homogenous, solid mass (endoscopic U/S)
  • Dexamethasone suppression test
  • Somatostatin receptor scintigraphy (SRS)
  • FPET imaging
  • N/A
  • Osteoporosis
  • Menstrual irregularities
Lung neuroendocrine tumors Typical carcinoid tumours[90][91][92][93]
  • Asymptomatic
  • Constitutional
  • Cough
  • Hemoptysis
  • Recurrent pneumonia
  • Seizures
  • ↓ Breath sounds
  • Fever
  • Weight loss
  • Carcinoid syndrome related
  • Cushing's syndrome related
  • ↑ Urinary 5-hydroxyindole-acetic acid
  • ↑ Chromogranin A
  • Central
  • Polypoid
  • Usually well defined
  • Smooth
  • No necrosis
  • Organized structure
  • <2 mitoses/2 mm2
  • No necroses
  • Nesting and/or trabecular pattern
  • Synaptophysin
  • Chromogranin A
  • CD56/NCAM
  • TTF1
  • Estrogen receptor
  • N/L
  • Coin lesion
  • Enlarged lymph nodes
  • Mass (rare)
  • Bronchoscopy (Gold standard for tumor visualization)
  • Endoscopic biopsy
  • N/A
  • 5‐year survival rate >90%
  • 12% metastasis at presentation
Atypical carcinoid tumours[90][91][92][93]
  • Asymptomatic
  • Constitutional
  • Cough
  • Hemoptysis
  • Recurrent pneumonia
  • Seizures
  • ↓ Breath sounds
  • Fever
  • Weight loss
  • Carcinoid syndrome related
  • Cushing's syndrome related
  • ↑ Urinary 5-hydroxyindole-acetic acid
  • ↑ Chromogranin A
  • Peripheral
  • Polypoid
  • Irregular margins
  • Smooth
  • Necrosis
  • More pleomorphism
  • 2–10 mitoses/2 mm2
  • Necroses
  • Nesting and/or trabecular pattern
  • Synaptophysin
  • Chromogranin A
  • CD56/NCAM
  • TTF1
  • Estrogen receptor
  • N/L
  • Coin lesion
  • Enlarged lymph nodes
  • Mass (rare)
  • Bronchoscopy (Gold standard for tumor visualization)
  • Endoscopic biopsy
  • N/A
  • 5‐year survival rate 40-75%
  • 50% metastasis at presentation
Large cell lung neuroendocrine carcinomas[94][95][96][97]
  • Asymptomatic
  • Cough
  • Hemoptysis
  • Recurrent infections
  • Seizures
  • ↓ Breath sounds
  • Fever
  • Carcinoid syndrome related (rare)
  • Cushing's syndrome related (rare)
  • ↑ Urinary 5-hydroxyindole-acetic acid (rare)
  • ↑ Serum ACTH (rare)
  • ↑ Chromogranin A
  • Less differentiated
  • Grey-white
  • Invasive
  • Hemorrhage/necrosis
  • Organoid or trabecular pattern
  • Rosette-like structures
  • Cells are ×3 the diameter of resting lymphocytes
  • Necrosis
  • Chromogranin
  • Neuron-specific enolase
  • Synaptophysin
  • Somatostatin
  • CD56
  • Peripheral lesion
  • Expansively growing
  • Irregular margins
  • Mediastinal lymph node enlargement
  • Bronchoscopy (Gold standard for tumor visualization)
  • SSTR (somatostatin receptors ) scintigraphy
  • Biopsy with immunohisto-chemistry
Associated
  • Smoking
  • Humoral hypercalcemia of malignancy
  • SIADH
  • Hypoglycemia
  • Acromegaly
  • Carcinoid syndrome
  • Neurological syndromes
  • Gynecomastia
  • Hyperthyroidism
Small cell lung neuroendocrine carcinomas[98][99][100][101]
  • Asymptomatic
  • Cough
  • Hemoptysis
  • Recurrent infections
  • Seizures
  • ↓ Breath sounds
  • Fever
  • Carcinoid syndrome related (rare)
  • Cushing's syndrome related (rare)
  • ↑ Urinary 5-hydroxyindole-acetic acid (rare)
  • ↑ Serum ACTH (rare)
  • ↑ Chromogranin A
  • Less differentiated
  • Grey-white
  • Invasive
  • Hemorrhage/necrosis
  • Diffuse sheets
  • No prominent neucleoli
  • Cells < size of three small resting lymphocytes
  • The mitotic rate averages over 60 mitoses/2 mm2
  • Extensive necrosis
  • Chromogranin
  • Neuron-specific enolase
  • Synaptophysin
  • Somatostatin
  • CD56
  • Homogeneous or noncontiguous parenchymal mass, or peripheral nodule
  • Adjacent compression
  • Atelectasis
  • Pleural effusion .
  • Intratumoral calcification
  • Lymphadenopathy
  • PET scan
  • Bronchoscopy
  • Scintigraphy
  • Biopsy with immunohisto-chemistry
Associated
  • Smoking
  • Humoral hypercalcemia of malignancy
  • SIADH
  • Hypoglycemia
  • Acromegaly
  • Neurological syndromes
  • Gynecomastia
  • Hyperthyroidism

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