Primitive neuroectodermal tumor: Difference between revisions

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*[[Histopathological|Histopathologically]], primitive neuroectodermal tumors should be [[Differentiate|differentiated]] from other [[tumors]] causing small, round, blue cell [[tumors]] involving [[bone]] and [[soft tissue]], including [[lymphoma]], small cell [[osteosarcoma]], undifferentiated [[neuroblastoma]], desmoplastic small round cell tumors, mesenchymal [[chondrosarcoma]], [[rhabdomyosarcoma]], and poorly differentiated [[synovial]] [[sarcoma]].<ref name="pmid1848471">{{cite journal |vauthors=Ambros IM, Ambros PF, Strehl S, Kovar H, Gadner H, Salzer-Kuntschik M |title=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 |journal=Cancer |volume=67 |issue=7 |pages=1886–93 |date=April 1991 |pmid=1848471 |doi=10.1002/1097-0142(19910401)67:7<1886::aid-cncr2820670712>3.0.co;2-u |url=}}</ref>
*[[Histopathological|Histopathologically]], primitive neuroectodermal tumors should be [[Differentiate|differentiated]] from other [[tumors]] causing small, round, blue cell [[tumors]] involving [[bone]] and [[soft tissue]], including [[lymphoma]], small cell [[osteosarcoma]], undifferentiated [[neuroblastoma]], desmoplastic small round cell tumors, mesenchymal [[chondrosarcoma]], [[rhabdomyosarcoma]], and poorly differentiated [[synovial]] [[sarcoma]].<ref name="pmid1848471">{{cite journal |vauthors=Ambros IM, Ambros PF, Strehl S, Kovar H, Gadner H, Salzer-Kuntschik M |title=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 |journal=Cancer |volume=67 |issue=7 |pages=1886–93 |date=April 1991 |pmid=1848471 |doi=10.1002/1097-0142(19910401)67:7<1886::aid-cncr2820670712>3.0.co;2-u |url=}}</ref>
==Epidemiology and Demographics==
==Epidemiology and Demographics==
*The annual [[incidence]] of PNETs from birth to 20 years of age is 0.29 per 100,000.<ref name="ViseeSoltner2005">{{cite journal|last1=Visee|first1=S|last2=Soltner|first2=C|last3=Rialland|first3=X|last4=Machet|first4=M C|last5=Loussouarn|first5=D|last6=Milinkevitch|first6=S|last7=Pasco-Papon|first7=A|last8=Mercier|first8=P|last9=Rousselet|first9=M C|title=Supratentorial primitive neuroectodermal tumours of the brain: multidirectional differentiation does not influence prognosis. A clinicopathological report of 18 patients|journal=Histopathology|volume=46|issue=4|year=2005|pages=403–412|issn=0309-0167|doi=10.1111/j.1365-2559.2005.02101.x}}</ref>
*The incidence of PNETs is from birth to 20 years of age approximately 0.29 per 100,000.<ref name="ViseeSoltner2005">{{cite journal|last1=Visee|first1=S|last2=Soltner|first2=C|last3=Rialland|first3=X|last4=Machet|first4=M C|last5=Loussouarn|first5=D|last6=Milinkevitch|first6=S|last7=Pasco-Papon|first7=A|last8=Mercier|first8=P|last9=Rousselet|first9=M C|title=Supratentorial primitive neuroectodermal tumours of the brain: multidirectional differentiation does not influence prognosis. A clinicopathological report of 18 patients|journal=Histopathology|volume=46|issue=4|year=2005|pages=403–412|issn=0309-0167|doi=10.1111/j.1365-2559.2005.02101.x}}</ref>
*The [[prevalence]] of primitive neuroectodermal tumors remains unknown.
*The [[prevalence]] of primitive neuroectodermal tumors remains unknown.
*PNETs are more common among children.
*PNETs are more common among children.
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*PNETs are more prevalent in Hispanic and white races.  
*PNETs are more prevalent in Hispanic and white races.  
==Risk Factors==
==Risk Factors==
*Prenatal exposure to [[alcohol]] seems to be a [[risk factor]] for developing PNET.<ref name=":2">{{Cite journal|last=G R Bunin, J D Buckley, C P Boesel, L B Rorke and A T Meadows|first=|date=1994|title=Risk factors for astrocytic glioma and primitive neuroectodermal tumor of the brain in young children: a report from the Children's Cancer Group.|url=http://cebp.aacrjournals.org/content/3/3/197.full-text.pdf|journal=Cancer Epidemiol Biomarkers Prev.|volume=|pages=|via=}}</ref>
*The most potent risk factor in the development of PNET is prenatal exposure to [[alcohol]] prenatal.<ref name=":2">{{Cite journal|last=G R Bunin, J D Buckley, C P Boesel, L B Rorke and A T Meadows|first=|date=1994|title=Risk factors for astrocytic glioma and primitive neuroectodermal tumor of the brain in young children: a report from the Children's Cancer Group.|url=http://cebp.aacrjournals.org/content/3/3/197.full-text.pdf|journal=Cancer Epidemiol Biomarkers Prev.|volume=|pages=|via=}}</ref>
*Children who had lived in farms for at least 1 year showed an increased risk for PNET.
*Children who had lived in farms for at least 1 year showed an increased risk for PNET.
*Certain [[syndromes]] seem to play the role of a [[risk factor]] for PNETs including the following:  
*Certain [[syndromes]] seem to play the role of a [[risk factor]] for PNETs including the following:  
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*If left untreated, [[patients]] with primitive neuroectodermal tumors may 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>
*If left untreated, [[patients]] with primitive neuroectodermal tumors may 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-year [[survival rate]] of [[patients]] with PNET less than 35% in adults and 64% in children.
*[[Prognosis]] is generally poor, and the 5-year [[survival rate]] of [[patients]] with PNET less than 35% in adults and 64% in [[children]].
*[[Prognosis]] is better in adult patients.
*[[Prognosis]] is better in adult [[Patient|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 [[Mili litr|cc]], and [[axial]] location.


== 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><ref name="pmid20959975">{{cite journal |vauthors=Li X, Zhang W, Song T, Sun C, Shen Y |title=Primitive neuroectodermal tumor arising in the abdominopelvic region: CT features and pathology characteristics |journal=Abdom Imaging |volume=36 |issue=5 |pages=590–5 |date=October 2011 |pmid=20959975 |doi=10.1007/s00261-010-9655-z |url=}}</ref>
* 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><ref name="pmid20959975">{{cite journal |vauthors=Li X, Zhang W, Song T, Sun C, Shen Y |title=Primitive neuroectodermal tumor arising in the abdominopelvic region: CT features and pathology characteristics |journal=Abdom Imaging |volume=36 |issue=5 |pages=590–5 |date=October 2011 |pmid=20959975 |doi=10.1007/s00261-010-9655-z |url=}}</ref>
*Clinical presentation of primitive neuroectodermal tumors is often non-specific and depend on the site of the [[tumor]].
*[[Clinical]] presentation of primitive neuroectodermal tumors is often non-specific and depend on the site of the [[tumor]].
*Patients with PNETs may present with only constitutional [[symptoms]] such as [[fever]], severe [[pain]], and [[paresthesia]].
*Patients with PNETs may present with only constitutional [[symptoms]] such as [[fever]], severe [[pain]], and [[paresthesia]].
*Other symptoms of primitive neuroectodermal tumor may include the following:   
*Other [[Symptom|symptoms]] of primitive neuroectodermal tumor may include the following:   
**[[Morning headache]]  
**[[Morning headache]]
**[[Restlessness]]
**[[Restlessness]]
**Recurrent [[vomiting]]
**Recurrent [[vomiting]]
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=== 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]].<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>
*[[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.<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>
*[[Laboratory]] findings consistent with the [[diagnosis]] of primitive neuroectodermal tumor 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>
*[[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>


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== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*There is no consensus in the treatment of PNET.
*There is no treatment for PNET.
*Chemotherapy is controversial in the treatment of PNET.
*[[Chemotherapy]] is controversial in the treatment of PNET.
*For the management of peripheral form of PNET, systemic [[chemotherapy]] has been associated with a better [[prognosis]] and is generally recommended.<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>
*For the management of peripheral form of PNET, [[systemic]] [[chemotherapy]] has been associated with a better [[prognosis]] and is generally recommended.<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>


=== Surgery ===
=== Surgery ===
*Based on the site of the [[tumor]], maximum resection must be performed.
*The feasibility of surgery depends on the site of the [[tumor]], at diagnosis. Maximum resection must be performed.
 
=== Primary Prevention ===
=== Primary Prevention ===
*There are no [[Primary prevention|primary preventive]] measures available for primitive neuroectodermal tumors.
*There are no [[Primary prevention|primary preventive]] measures available for primitive neuroectodermal tumors.

Latest revision as of 17:02, 14 November 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 was more commonly described in 1973 by Hart and Earle. In fact, PNETs are members of the Ewing tumor family. Primitive neuroectodermal tumor are classified into 3 subtypes. Histopathologically, PNETs should be differentiated from other tumors causing small round blue cell tumors involving bone and soft tissue. PNETs are more common among children. Clinical presentation of primitive neuroectodermal tumors is often non-specific and depend on the site of the tumor. Physical examination may be remarkable for papilledema, strabismus, nystagmus, imbalance, motor weakness, facial sensory loss, third, fourth, and sixth cranial nerve palsies, hemiplegia, hepatosplenomegaly, and adenopathy. On CT, findings associated with the diagnosis of PNETs, may include a large irregular mass with heterogeneous contrast enhancement. On MRI, findings of the PNETs 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. For the management of peripheral form of PNET, systemic chemotherapy has been associated with a better prognosis and is generally recommended.

Historical Perspective

Classification

Pathophysiology

Courtesy of image Wikipedia

Differentiating Primitive Neuroectodermal Tumor from Other Diseases

Epidemiology and Demographics

  • The incidence of PNETs is from birth to 20 years of age approximately 0.29 per 100,000.[13]
  • The prevalence of primitive neuroectodermal tumors remains unknown.
  • PNETs are more common among children.
  • PNETs are more prevalent in men than women.[14]
  • PNETs are more prevalent in Hispanic and white races.

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

Axial T2 image of PNET, Case courtesy of Dr Prashant Mudgal, Radiopaedia.org, rID: 32696

MRI

  • MRI is the imaging modality of choice for primitive neuroectodermal tumors.[22]
  • 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 treatment for PNET.
  • Chemotherapy is controversial in the treatment of PNET.
  • For the management of peripheral form of PNET, systemic chemotherapy has been associated with a better prognosis and is generally recommended.[11]

Surgery

  • The feasibility of surgery depends on the site of the tumor, at diagnosis. 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. 11.0 11.1 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.
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  19. 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.
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