Difference between revisions of "Primitive neuroectodermal tumor"

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*On gross pathology, white, hemorrhagic and necrotic mass are characteristic of PNET.<ref name="pmid26793768">{{cite journal| author=Novo J, Bitterman P, Guirguis A| title=Central-type primitive neuroectodermal tumor of the uterus: Case report of remission of stage IV disease using adjuvant cisplatin/etoposide/bevacizumab chemotherapy and review of the literature. | journal=Gynecol Oncol Rep | year= 2015 | volume= 14 | issue=  | pages= 26-30 | pmid=26793768 | doi=10.1016/j.gore.2015.09.002 | pmc=4688884 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26793768  }} </ref>
 
*On gross pathology, white, hemorrhagic and necrotic mass are characteristic of PNET.<ref name="pmid26793768">{{cite journal| author=Novo J, Bitterman P, Guirguis A| title=Central-type primitive neuroectodermal tumor of the uterus: Case report of remission of stage IV disease using adjuvant cisplatin/etoposide/bevacizumab chemotherapy and review of the literature. | journal=Gynecol Oncol Rep | year= 2015 | volume= 14 | issue=  | pages= 26-30 | pmid=26793768 | doi=10.1016/j.gore.2015.09.002 | pmc=4688884 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26793768  }} </ref>
 
*On microscopy histopathological analysis, small round blue cells, fine chromatin, eosinophilic cytoplasm,homer-Wright rosettes, and high mitotic figures.<ref name="pmid7803540">{{cite journal| author=Jürgens HF| title=Ewing's sarcoma and peripheral primitive neuroectodermal tumor. | journal=Curr Opin Oncol | year= 1994 | volume= 6 | issue= 4 | pages= 391-6 | pmid=7803540 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7803540  }} </ref><ref name="pmid10623711">{{cite journal| author=de Alava E, Gerald WL| title=Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family. | journal=J Clin Oncol | year= 2000 | volume= 18 | issue= 1 | pages= 204-13 | pmid=10623711 | doi=10.1200/JCO.2000.18.1.204 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10623711  }} </ref>
 
*On microscopy histopathological analysis, small round blue cells, fine chromatin, eosinophilic cytoplasm,homer-Wright rosettes, and high mitotic figures.<ref name="pmid7803540">{{cite journal| author=Jürgens HF| title=Ewing's sarcoma and peripheral primitive neuroectodermal tumor. | journal=Curr Opin Oncol | year= 1994 | volume= 6 | issue= 4 | pages= 391-6 | pmid=7803540 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7803540  }} </ref><ref name="pmid10623711">{{cite journal| author=de Alava E, Gerald WL| title=Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family. | journal=J Clin Oncol | year= 2000 | volume= 18 | issue= 1 | pages= 204-13 | pmid=10623711 | doi=10.1200/JCO.2000.18.1.204 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10623711  }} </ref>
 +
{| align="right"
 +
|[[File:PNET Histopathology HE 200x.jpg|thumb|right|200px|Courtesy of image Wikipedia]]
 +
|}
 
*On microscopic histopathological analysis,  characteristic findings of the primitive neuroectodermal tumor, include small blue cell tumor with abundant mitotic figures, Homer-Wright rosettes, in which tumor cells surround neutrophils, fibrosis, and short and round or spindle-shaped nuclei.
 
*On microscopic histopathological analysis,  characteristic findings of the primitive neuroectodermal tumor, include small blue cell tumor with abundant mitotic figures, Homer-Wright rosettes, in which tumor cells surround neutrophils, fibrosis, and short and round or spindle-shaped nuclei.
 
*[[Immunohistochemical]] analysis can also be positive for [[CD99]], [[CD56]], [[Neuron-specific enolase]] (NSE), S-100 protein, [[synaptophysin]], and [[chromogranin A]].<ref name="AlonsoYi2017">{{cite journal|last1=Alonso|first1=Marta M.|last2=Yi|first2=Xiaoping|last3=Liu|first3=Wenguang|last4=Zhang|first4=Youming|last5=Xiao|first5=Desheng|last6=Yin|first6=Hongling|last7=Long|first7=Xueying|last8=Li|first8=Li|last9=Zai|first9=Hongyan|last10=Chen|first10=Minfeng|last11=Li|first11=Wenzheng|last12=Sun|first12=Lunquan|title=Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases|journal=PLOS ONE|volume=12|issue=3|year=2017|pages=e0173536|issn=1932-6203|doi=10.1371/journal.pone.0173536}}</ref>
 
*[[Immunohistochemical]] analysis can also be positive for [[CD99]], [[CD56]], [[Neuron-specific enolase]] (NSE), S-100 protein, [[synaptophysin]], and [[chromogranin A]].<ref name="AlonsoYi2017">{{cite journal|last1=Alonso|first1=Marta M.|last2=Yi|first2=Xiaoping|last3=Liu|first3=Wenguang|last4=Zhang|first4=Youming|last5=Xiao|first5=Desheng|last6=Yin|first6=Hongling|last7=Long|first7=Xueying|last8=Li|first8=Li|last9=Zai|first9=Hongyan|last10=Chen|first10=Minfeng|last11=Li|first11=Wenzheng|last12=Sun|first12=Lunquan|title=Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases|journal=PLOS ONE|volume=12|issue=3|year=2017|pages=e0173536|issn=1932-6203|doi=10.1371/journal.pone.0173536}}</ref>
[[File:PNET Histopathology HE 200x.jpg|thumb|H&E staining of PNET. Courtesy of image: [https://en.wikipedia.org/wiki/Primitive_neuroectodermal_tumor Wikipedia]]]
 
 
==Differentiating Primitive Neuroectodermal Tumor from Other Diseases==
 
==Differentiating Primitive Neuroectodermal Tumor from Other Diseases==
 
*Primitive neuroectodermal tumor must be [[Differentiate|differentiated]] from other [[diseases]] that cause [[seizures]] or an [[Increased intracranial pressure|increase in intracranial pressure]], such as [[astrocytoma]], [[ependymoma]], [[oligodendroglioma]], intracranial [[teratoma]], [[meningitis]], [[encephalitis]], and other [[brain]] [[tumors]].
 
*Primitive neuroectodermal tumor must be [[Differentiate|differentiated]] from other [[diseases]] that cause [[seizures]] or an [[Increased intracranial pressure|increase in intracranial pressure]], such as [[astrocytoma]], [[ependymoma]], [[oligodendroglioma]], intracranial [[teratoma]], [[meningitis]], [[encephalitis]], and other [[brain]] [[tumors]].
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== Natural History, Complications and Prognosis==
 
== Natural History, Complications and Prognosis==
  
*If left untreated, patients with primitive neuroectodermal tumors may progress to develop [[metastases]].
+
*If left untreated, patients with primitive neuroectodermal tumors may progress to develop [[metastases]].<ref>{{Cite journal|last=Smoll NR.|first=|date=2012|title=Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs).|url=|journal=Cancer|volume=|pages=|via=}}</ref>
 
*Common [[complications]] of the primitive neuroectodermal tumor, include [[increased intracranial pressure]], [[cranial nerve palsy]], and [[seizures]].
 
*Common [[complications]] of the primitive neuroectodermal tumor, include [[increased intracranial pressure]], [[cranial nerve palsy]], and [[seizures]].
*Prognosis is generally poor, and the 5-survival rate of patients with PNET less than 35% in adults and 64% in children<ref>{{Cite journal|last=Smoll NR.|first=|date=2012|title=Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs).|url=|journal=Cancer|volume=|pages=|via=}}</ref>.
+
*Prognosis is generally poor, and the 5-survival rate of patients with PNET less than 35% in adults and 64% in children.
 
*[[Prognosis]] is more favorable for adult patients.
 
*[[Prognosis]] is more favorable for adult patients.
 
*Features associated with favorable [[prognosis]] include early [[diagnosis]], combination treatment approach including [[tumor]] resection, [[chemotherapy]] and [[radiotherapy]], intratumoral [[calcification]], [[Ki-67 (Biology)|Ki-67]] <30%, elevated [[LDH]], [[tumor]] volume >100 cc, and [[axial]] location.
 
*Features associated with favorable [[prognosis]] include early [[diagnosis]], combination treatment approach including [[tumor]] resection, [[chemotherapy]] and [[radiotherapy]], intratumoral [[calcification]], [[Ki-67 (Biology)|Ki-67]] <30%, elevated [[LDH]], [[tumor]] volume >100 cc, and [[axial]] location.
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== Diagnosis ==
 
== Diagnosis ==
 
=== History and Symptoms ===
 
=== History and Symptoms ===
 
+
* The majority of [[patients]] with primitive neuroectodermal tumors remain [[asymptomatic]] for years.<ref name="pmid2776115">{{cite journal |vauthors=Rud NP, Reiman HM, Pritchard DJ, Frassica FJ, Smithson WA |title=Extraosseous Ewing's sarcoma. A study of 42 cases |journal=Cancer |volume=64 |issue=7 |pages=1548–53 |date=October 1989 |pmid=2776115 |doi=10.1002/1097-0142(19891001)64:7<1548::aid-cncr2820640733>3.0.co;2-w |url=}}</ref>
* The majority of [[patients]] with primitive neuroectodermal tumors remain [[asymptomatic]] for years.
+
*Clinical presentation of primitive neuroectodermal tumors is often non-specific and depend on the site of the [[tumor]].
 
+
*[[Symptoms]] of primitive neuroectodermal tumor may include [[morning headache]], [[restlessness]], recurrent [[vomiting]], [[diplopia]], frequent falls, positional [[dizziness]], [[forgetfulness]], progressive [[visual]] disturbances, constitutional [[symptoms]] such as [[fever]], severe [[pain]], and [[paresthesia]].
*Clinical presentation of primitive neuroectodermal tumors is often non-specific and depend on the site of the tumor.
 
*Symptoms of primitive neuroectodermal tumor may include [[morning headache]], [[restlessness]], recurrent [[vomiting]], [[diplopia]], frequent falls, positional [[dizziness]], [[forgetfulness]], progressive [[visual]] disturbances, constitutional [[symptoms]] such as [[fever]], severe [[pain]], and [[paresthesia]].
 
 
 
 
=== Physical Examination ===
 
=== Physical Examination ===
*Physical examination may be remarkable for [[papilledema]], [[strabismus]], [[nystagmus]], [[ataxia|imbalance]], motor [[weakness]], facial [[sensory loss]], third, fourth, and sixth [[cranial nerve palsies]], [[hemiplegia]], [[hepatosplenomegaly]], and [[lymphadenopathy|Adenopathy]]
+
*Physical examination may be remarkable for [[papilledema]], [[strabismus]], [[nystagmus]], [[ataxia|imbalance]], motor [[weakness]], facial [[sensory loss]], third, fourth, and sixth [[cranial nerve palsies]], [[hemiplegia]], [[hepatosplenomegaly]], and [[lymphadenopathy|Adenopathy]].<ref name="pmid2776115">{{cite journal |vauthors=Rud NP, Reiman HM, Pritchard DJ, Frassica FJ, Smithson WA |title=Extraosseous Ewing's sarcoma. A study of 42 cases |journal=Cancer |volume=64 |issue=7 |pages=1548–53 |date=October 1989 |pmid=2776115 |doi=10.1002/1097-0142(19891001)64:7<1548::aid-cncr2820640733>3.0.co;2-w |url=}}</ref>
 
=== Laboratory Findings ===
 
=== Laboratory Findings ===
*Laboratory findings associated with the diagnosis of primitive neuroectodermal tumor may include elevated [[erythrocyte sedimentation rate]], positive [[C-reactive protein]], [[anemia]], [[leukocytosis]], [[thrombocytosis]], [[hypoalbuminemia]], increased [[Lactate dehydrogenase|LDH]] levels.
+
*Laboratory findings associated with the diagnosis of primitive neuroectodermal tumor may include elevated [[erythrocyte sedimentation rate]], positive [[C-reactive protein]], [[anemia]], [[leukocytosis]], [[thrombocytosis]], [[hypoalbuminemia]], increased [[Lactate dehydrogenase|LDH]] levels.<ref name="pmid3992134">{{cite journal |vauthors=Bacci G, Capanna R, Orlandi M, Mancini I, Bettelli G, Dallari D, Campanacci M |title=Prognostic significance of serum lactic acid dehydrogenase in Ewing's tumor of bone |journal=Ric Clin Lab |volume=15 |issue=1 |pages=89–96 |date=1985 |pmid=3992134 |doi= |url=}}</ref>
 +
*[[Neuroblastoma]] may be associated with an elevated level of [[urinary]] [[catecholamines]].<ref name="pmid16732582">{{cite journal |vauthors=Strenger V, Kerbl R, Dornbusch HJ, Ladenstein R, Ambros PF, Ambros IM, Urban C |title=Diagnostic and prognostic impact of urinary catecholamines in neuroblastoma patients |journal=Pediatr Blood Cancer |volume=48 |issue=5 |pages=504–9 |date=May 2007 |pmid=16732582 |doi=10.1002/pbc.20888 |url=}}</ref>
 
=== Electrocardiogram ===
 
=== Electrocardiogram ===
 
*There are no [[ECG]] findings associated with primitive neuroectodermal tumors.
 
*There are no [[ECG]] findings associated with primitive neuroectodermal tumors.
Line 74: Line 74:
 
=== Other Diagnostic Studies ===
 
=== Other Diagnostic Studies ===
 
*There are no other diagnostic studies associated with primitive neuroectodermal tumors.
 
*There are no other diagnostic studies associated with primitive neuroectodermal tumors.
 +
 
== Treatment ==
 
== Treatment ==
 
=== Medical Therapy ===
 
=== Medical Therapy ===

Revision as of 21:46, 27 October 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Gertrude Djouka, M.D.[2], Maria Fernanda Villarreal, M.D. [3]

Synonyms and Keywords: Primitive neuroectodermal tumors; PNET; CNS PNET; Askin tumor; Peripheral neuroepithelioma; Ependymoblastoma

Overview

Primitive neuroectodermal tumor (also known as "PNET") is a rare type of malignant tumor originating from neuroectoderm. Neuroectoderm is normally involved in the development of the nervous system. Apart from central nervous system (CNS), PNETs can involve other tissues originating from the neuroectoderm such as muscles and bones. PNET was first discovered by James Ewing, an American pathologist, in 1921. However, the term PNETs is more commonly was described in 1973 by Hart and Earle. In fact, PNETs are members of the Ewing tumor family. These tumors have small round cells, are believed to originate from postganglionic parasympathetic primordial cells and have mutations of the EWS gene. Due to their origin, PNETs can be found at any site within the parasympathetic system. Apart from Ewing's Sarcoma (ES) and PNET, this family of tumors includes other tumors such as Askin's tumor (a malignant small-cell tumor in the chest) and paravertebral small-cell tumors. PNETs are divided into peripheral and central based on their presentation site. Central PNETs are more commonly seen among children and young adults and account for approximately 1% of PNETs. Peripheral PNETs mostly occur in bones and surrounding tissues. PNETs are more commonly seen among children and young adults. The median age at diagnosis is 25 years of age. PNETs are highly malignant and their prognosis is generally poor, however, the prognosis is more favorable for adult patients with PNET. The 5-survival rate of patients with PNET is less than 35%. The disease affects both men and women, however, there is a slight tendency toward affecting males in the cases of peripheral PNET.

Historical Perspective

Classification

Pathophysiology

  • The pathogenesis of peripheral primitive neuroectodermal tumor is characterized by the chromosomal translocation t(11;22)(q24q12).[6][7]
  • This translocation fuses the EWS gene on chromosome 22 with the FLI1 gene on chromosome 11.
  • The EWS-FLI1 gene has been associated with the development of PNET involving the synthesis of adrenal pathway.
  • On gross pathology, white, hemorrhagic and necrotic mass are characteristic of PNET.[8]
  • On microscopy histopathological analysis, small round blue cells, fine chromatin, eosinophilic cytoplasm,homer-Wright rosettes, and high mitotic figures.[9][10]
Courtesy of image Wikipedia
  • On microscopic histopathological analysis, characteristic findings of the primitive neuroectodermal tumor, include small blue cell tumor with abundant mitotic figures, Homer-Wright rosettes, in which tumor cells surround neutrophils, fibrosis, and short and round or spindle-shaped nuclei.
  • Immunohistochemical analysis can also be positive for CD99, CD56, Neuron-specific enolase (NSE), S-100 protein, synaptophysin, and chromogranin A.[11]

Differentiating Primitive Neuroectodermal Tumor from Other Diseases

Epidemiology and Demographics

  • The annual incidence of PNETs from birth to 20 years of age is 0.29 per 100,000.[13]
  • The prevalence of primitive neuroectodermal tumors remains unknown.
  • PNETs are more common among children.
  • PNETs have a slight tendency toward affecting men compared to women. [14]
  • PNETs usually affect Hispanic and white individuals.

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

  • There are no ECG findings associated with primitive neuroectodermal tumors.

X-ray

  • There are no x-ray findings associated with primitive neuroectodermal tumors.

Echocardiography or Ultrasound

CT

MRI

  • MRI is the imaging modality of choice for primitive neuroectodermal tumors.[21]
  • On MRI, findings of the primitive neuroectodermal tumor may include highly variable and can be hypo-intense to isointense, but usually, hypo-intense on T1-weighted images and high signal solid components on T2-weighted images.

Other Imaging Findings

  • There are no other imaging findings associated with primitive neuroectodermal tumors.

Other Diagnostic Studies

  • There are no other diagnostic studies associated with primitive neuroectodermal tumors.

Treatment

Medical Therapy

  • There is no consensus in the treatment of PNET.
  • Chemotherapy is controversial in the treatment of PNET.
  • Temozolomide can be added to conventional treatment of excision and radiotherapy.
  • 7 to 8 weeks of radiotherapy at a dose of 50-55 Gy is recommended [22].

Surgery

  • Based on the site of the tumor, maximum resection must be performed.

Primary Prevention

Secondary Prevention

References

  1. Yagnik, Vipul D; Dawka, Sushil (2019). "

    Extraskeletal Ewing's sarcoma/peripheral primitive neuroectodermal tumor of the small bowel presenting with gastrointestinal perforation

    ". Clinical and Experimental Gastroenterology. Volume 12: 279–285. doi:10.2147/CEG.S203697. ISSN 1178-7023.
  2. Rorke LB. (1983). "The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors". J Neuropathol Exp Neuro.
  3. Batsakis, John G.; MacKay, Bruce; El-Naggar, Adel K. (2016). "Ewing's Sarcoma and Peripheral Primitive Neuroectodermal Tumor: An Interim Report". Annals of Otology, Rhinology & Laryngology. 105 (10): 838–843. doi:10.1177/000348949610501014. ISSN 0003-4894.
  4. Castro, E. C.; Parwani, A. V. (2012). "Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: Two Unusual Presentations of a Rare Tumor". Case Reports in Medicine. 2012: 1–7. doi:10.1155/2012/190581. ISSN 1687-9627.
  5. Triarico S, Attinà G, Maurizi P, Mastrangelo S, Nanni L, Briganti V, Meacci E, Margaritora S, Balducci M, Ruggiero A (July 2018). "Multimodal treatment of pediatric patients with Askin's tumors: our experience". World J Surg Oncol. 16 (1): 140. doi:10.1186/s12957-018-1434-2. PMC 6044084. PMID 30005673.
  6. Zucman J, Delattre O, Desmaze C, Plougastel B, Joubert I, Melot T; et al. (1992). "Cloning and characterization of the Ewing's sarcoma and peripheral neuroepithelioma t(11;22) translocation breakpoints". Genes Chromosomes Cancer. 5 (4): 271–7. PMID 1283315.
  7. Delattre O, Zucman J, Plougastel B, Desmaze C, Melot T, Peter M; et al. (1992). "Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours". Nature. 359 (6391): 162–5. doi:10.1038/359162a0. PMID 1522903.
  8. Novo J, Bitterman P, Guirguis A (2015). "Central-type primitive neuroectodermal tumor of the uterus: Case report of remission of stage IV disease using adjuvant cisplatin/etoposide/bevacizumab chemotherapy and review of the literature". Gynecol Oncol Rep. 14: 26–30. doi:10.1016/j.gore.2015.09.002. PMC 4688884. PMID 26793768.
  9. Jürgens HF (1994). "Ewing's sarcoma and peripheral primitive neuroectodermal tumor". Curr Opin Oncol. 6 (4): 391–6. PMID 7803540.
  10. de Alava E, Gerald WL (2000). "Molecular biology of the Ewing's sarcoma/primitive neuroectodermal tumor family". J Clin Oncol. 18 (1): 204–13. doi:10.1200/JCO.2000.18.1.204. PMID 10623711.
  11. Alonso, Marta M.; Yi, Xiaoping; Liu, Wenguang; Zhang, Youming; Xiao, Desheng; Yin, Hongling; Long, Xueying; Li, Li; Zai, Hongyan; Chen, Minfeng; Li, Wenzheng; Sun, Lunquan (2017). "Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases". PLOS ONE. 12 (3): e0173536. doi:10.1371/journal.pone.0173536. ISSN 1932-6203.
  12. Ambros IM, Ambros PF, Strehl S, Kovar H, Gadner H, Salzer-Kuntschik M (April 1991). "MIC2 is a specific marker for Ewing's sarcoma and peripheral primitive neuroectodermal tumors. Evidence for a common histogenesis of Ewing's sarcoma and peripheral primitive neuroectodermal tumors from MIC2 expression and specific chromosome aberration". Cancer. 67 (7): 1886–93. doi:10.1002/1097-0142(19910401)67:7<1886::aid-cncr2820670712>3.0.co;2-u. PMID 1848471.
  13. Visee, S; Soltner, C; Rialland, X; Machet, M C; Loussouarn, D; Milinkevitch, S; Pasco-Papon, A; Mercier, P; Rousselet, M C (2005). "Supratentorial primitive neuroectodermal tumours of the brain: multidirectional differentiation does not influence prognosis. A clinicopathological report of 18 patients". Histopathology. 46 (4): 403–412. doi:10.1111/j.1365-2559.2005.02101.x. ISSN 0309-0167.
  14. Ohba S, Yoshida K, Hirose Y, Ikeda E, Kawase T. (2008). "A supratentorial primitive neuroectodermal tumor in an adult: a case report and review of the literature". J Neurooncol.
  15. G R Bunin, J D Buckley, C P Boesel, L B Rorke and A T Meadows (1994). "Risk factors for astrocytic glioma and primitive neuroectodermal tumor of the brain in young children: a report from the Children's Cancer Group" (PDF). Cancer Epidemiol Biomarkers Prev.
  16. Smoll NR. (2012). "Relative survival of childhood and adult medulloblastomas and primitive neuroectodermal tumors (PNETs)". Cancer.
  17. 17.0 17.1 Rud NP, Reiman HM, Pritchard DJ, Frassica FJ, Smithson WA (October 1989). "Extraosseous Ewing's sarcoma. A study of 42 cases". Cancer. 64 (7): 1548–53. doi:10.1002/1097-0142(19891001)64:7<1548::aid-cncr2820640733>3.0.co;2-w. PMID 2776115.
  18. Bacci G, Capanna R, Orlandi M, Mancini I, Bettelli G, Dallari D, Campanacci M (1985). "Prognostic significance of serum lactic acid dehydrogenase in Ewing's tumor of bone". Ric Clin Lab. 15 (1): 89–96. PMID 3992134.
  19. Strenger V, Kerbl R, Dornbusch HJ, Ladenstein R, Ambros PF, Ambros IM, Urban C (May 2007). "Diagnostic and prognostic impact of urinary catecholamines in neuroblastoma patients". Pediatr Blood Cancer. 48 (5): 504–9. doi:10.1002/pbc.20888. PMID 16732582.
  20. Xiao H, Bao F, Tan H, Wang B, Liu W, Gao J, Gao X (2016). "CT and clinical findings of peripheral primitive neuroectodermal tumour in children". Br J Radiol. 89 (1060): 20140450. doi:10.1259/bjr.20140450. PMC 4846188. PMID 26847997.
  21. Shi H, Kong X, Xu H, Xu L, Liu D (2004). "MRI features of intracranial primitive neuroectodermal tumors in adults: comparing with histopathological findings". J. Huazhong Univ. Sci. Technol. Med. Sci. 24 (1): 99–102. PMID 15165129.
  22. Batsakis JG, Mackay B, el-Naggar AK (1996). "Ewing's sarcoma and peripheral primitive neuroectodermal tumor: an interim report". Ann Otol Rhinol Laryngol.

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