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Peripheral neuroepithelioma(PNE)

MedGen UID:: 483352
•Concept ID:: C3489398
•: Neoplastic Process

Synonyms:	Ewing's tumor of bone (type); Extraosseous Ewing's (tumor growing outside of the bone) (type); PNE
SNOMED CT:	PNE - Peripheral neuroepithelioma (254764001); Peripheral neuroepithelioma (254764001)

Related gene: Gene(s) associated with related conditions. For conditions in a hierarchy, the parent condition will list the genes associated with the children conditions.	EWSR1

HPO:	HP:0006717
OMIM®:	612219

Definition

The Ewing sarcoma family of tumors (primitive neuroectodermal tumors; PNET) comprise morphologically heterogeneous tumors that are characterized by nonrandom chromosomal translocations involving the EWS gene on chromosome 22q12 and one of several members of the ETS family of transcription factors. The tumors include Ewing sarcoma, peripheral neuroepithelioma, and Askin tumor. In approximately 90% of cases of ESFT, the FLI1 gene (193067) on chromosome 11 is the fusion partner of EWS; in approximately 10%, the EWS fusion partner is the ERG gene (165080) on chromosome 22. Many other ETS family members have been identified as fusion partners of EWS, but these cases are rare (Khoury, 2005). [from OMIM]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVPeripheral neuroepithelioma
- CROGVAskin tumor
- CROGVEwing sarcoma

Fetal anomaly
- Congenital Systemic Disorder
  - Abnormality of the nervous system
    - Abnormal nervous system morphology
      - Neoplasm of the nervous system
        Neoplasm of the peripheral nervous system
        Peripheral neuroepithelioma
        Askin tumor
        Localized Askin Tumor
        Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
        Recurrent Ewing Sarcoma
        Recurrent Peripheral Primitive Neuroectodermal Tumor
        Ewing sarcoma
        Atypical Ewing Sarcoma
        Ewing sarcoma of bone
        Localized Bone Ewing Sarcoma
        Metastatic Bone Ewing Sarcoma
        Recurrent Bone Ewing Sarcoma
        Extraskeletal Ewing sarcoma
        Kidney Ewing Sarcoma
        Localized Extraskeletal Ewing Sarcoma
        Metastatic Extraskeletal Ewing Sarcoma
        Recurrent Extraskeletal Ewing Sarcoma
        Skin Ewing Sarcoma
        Vulvar Ewing Sarcoma
        Localized Ewing Sarcoma
        Metastatic Ewing Sarcoma
        Locally Advanced Ewing Sarcoma
        Refractory Ewing Sarcoma
        Unresectable Ewing Sarcoma

Professional guidelines

PubMed

Treatment of high-risk solid tumors of childhood with intensive therapy and autologous bone marrow transplantation.

Seeger RC, Reynolds CP
Pediatr Clin North Am 1991 Apr;38(2):393-424. doi: 10.1016/s0031-3955(16)38084-1. PMID: 2006084

Morphometry and the differential diagnosis between peripheral neuroepithelioma and neuroblastoma.

Cavazzana AO, Santopietro R, Sforza V, Miracco C, Lio R, Luzi P, Tosi P, Ninfo V
Mod Pathol 1991 Sep;4(5):615-20. PMID: 1758874

Treatment of peripheral neuroepithelioma in children and young adults.

Miser JS, Kinsella TJ, Triche TJ, Steis R, Tsokos M, Wesley R, Horvath K, Belasco J, Longo DL, Glatstein E
J Clin Oncol 1987 Nov;5(11):1752-8. doi: 10.1200/JCO.1987.5.11.1752. PMID: 3316513

See all (8)

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Malignant peripheral primitive neuroectodermal tumor-peripheral neuroepithelioma of the head and neck: a clinicopathologic study of five cases and review of the literature.

Nikitakis NG, Salama AR, O'Malley BW Jr, Ord RA, Papadimitriou JC
Head Neck 2003 Jun;25(6):488-98. doi: 10.1002/hed.10260. PMID: 12784241

Immunohistochemistry of small round-cell tumors.

Devoe K, Weidner N
Semin Diagn Pathol 2000 Aug;17(3):216-24. PMID: 10968707

013 (CD99) positivity in hematologic proliferations correlates with TdT positivity.

Robertson PB, Neiman RS, Worapongpaiboon S, John K, Orazi A
Mod Pathol 1997 Apr;10(4):277-82. PMID: 9110287

Soft tissue small round cell tumors: morphological parameters.

d'Amore ES, Ninfo V
Semin Diagn Pathol 1996 Aug;13(3):184-203. PMID: 8875709

Treatment of peripheral neuroepithelioma in children and young adults.

Miser JS, Kinsella TJ, Triche TJ, Steis R, Tsokos M, Wesley R, Horvath K, Belasco J, Longo DL, Glatstein E
J Clin Oncol 1987 Nov;5(11):1752-8. doi: 10.1200/JCO.1987.5.11.1752. PMID: 3316513

See all (23)

Diagnosis

Malignant peripheral primitive neuroectodermal tumor-peripheral neuroepithelioma of the head and neck: a clinicopathologic study of five cases and review of the literature.

Nikitakis NG, Salama AR, O'Malley BW Jr, Ord RA, Papadimitriou JC
Head Neck 2003 Jun;25(6):488-98. doi: 10.1002/hed.10260. PMID: 12784241

Immunohistochemistry of small round-cell tumors.

Devoe K, Weidner N
Semin Diagn Pathol 2000 Aug;17(3):216-24. PMID: 10968707

Soft tissue small round cell tumors: morphological parameters.

d'Amore ES, Ninfo V
Semin Diagn Pathol 1996 Aug;13(3):184-203. PMID: 8875709

Extraskeletal Ewing's sarcoma.

Huntrakoon M
Ultrastruct Pathol 1987;11(4):411-9. doi: 10.3109/01913128709048435. PMID: 3617227

Peripheral neuroepithelioma in childhood.

Voss BL, Pysher TJ, Humphrey GB
Cancer 1984 Dec 15;54(12):3059-64. doi: 10.1002/1097-0142(19841215)54:12<3059::aid-cncr2820541241>3.0.co;2-4. PMID: 6498781

See all (49)

Therapy

Malignant peripheral primitive neuroectodermal (pPNET) of tongue.

Ulutin C, Cetinayak O, Aksu G, Fayda M, Ataergin S, Beyzadeoğlu M
Auris Nasus Larynx 2007 Mar;34(1):115-8. Epub 2007 Jan 19 doi: 10.1016/j.anl.2006.11.001. PMID: 17240098

Peripheral neuroepithelioma with ganglion cells: report of two cases and review of the literature.

Williams S, Parham DM, Jenkins JJ 3rd
Pediatr Dev Pathol 1999 Jan-Feb;2(1):42-9. doi: 10.1007/s100249900088. PMID: 9841705

Peripheral primitive neuroectodermal tumor (peripheral neuroepithelioma) in children. A review of the St. Jude experience and controversies in diagnosis and management.

Marina NM, Etcubanas E, Parham DM, Bowman LC, Green A
Cancer 1989 Nov 1;64(9):1952-60. doi: 10.1002/1097-0142(19891101)64:9<1952::aid-cncr2820640931>3.0.co;2-w. PMID: 2551479

Treatment of peripheral neuroepithelioma in children and young adults.

Miser JS, Kinsella TJ, Triche TJ, Steis R, Tsokos M, Wesley R, Horvath K, Belasco J, Longo DL, Glatstein E
J Clin Oncol 1987 Nov;5(11):1752-8. doi: 10.1200/JCO.1987.5.11.1752. PMID: 3316513

Ifosfamide with mesna uroprotection and etoposide: an effective regimen in the treatment of recurrent sarcomas and other tumors of children and young adults.

Miser JS, Kinsella TJ, Triche TJ, Tsokos M, Jarosinski P, Forquer R, Wesley R, Magrath I
J Clin Oncol 1987 Aug;5(8):1191-8. doi: 10.1200/JCO.1987.5.8.1191. PMID: 3114435

See all (16)

Prognosis

Malignant peripheral primitive neuroectodermal (pPNET) of tongue.

Ulutin C, Cetinayak O, Aksu G, Fayda M, Ataergin S, Beyzadeoğlu M
Auris Nasus Larynx 2007 Mar;34(1):115-8. Epub 2007 Jan 19 doi: 10.1016/j.anl.2006.11.001. PMID: 17240098

Collision tumor: a peripheral neuroepithelioma and a transitional-cell carcinoma occurring simultaneously in the renal pelvis.

Phan ST, Meng M, Weidner N
Ann Diagn Pathol 1997 Dec;1(2):91-8. doi: 10.1016/s1092-9134(97)80013-5. PMID: 9869830

Cytogenetic and pathologic aspects of Ewing's sarcoma and neuroectodermal tumors.

Stephenson CF, Bridge JA, Sandberg AA
Hum Pathol 1992 Nov;23(11):1270-7. doi: 10.1016/0046-8177(92)90295-e. PMID: 1330877

Treatment of peripheral neuroepithelioma in children and young adults.

Miser JS, Kinsella TJ, Triche TJ, Steis R, Tsokos M, Wesley R, Horvath K, Belasco J, Longo DL, Glatstein E
J Clin Oncol 1987 Nov;5(11):1752-8. doi: 10.1200/JCO.1987.5.11.1752. PMID: 3316513

Peripheral neuroepithelioma in childhood.

Voss BL, Pysher TJ, Humphrey GB
Cancer 1984 Dec 15;54(12):3059-64. doi: 10.1002/1097-0142(19841215)54:12<3059::aid-cncr2820541241>3.0.co;2-4. PMID: 6498781

See all (22)

Clinical prediction guides

Constitutive activation of the neuregulin-1/erbB signaling pathway promotes the proliferation of a human peripheral neuroepithelioma cell line.

Fallon KB, Havlioglu N, Hamilton LH, Cheng TP, Carroll SL
J Neurooncol 2004 Feb;66(3):273-84. doi: 10.1023/b:neon.0000014521.28294.84. PMID: 15015657

Primitive neuroectodermal tumors arising in testicular germ cell neoplasms.

Michael H, Hull MT, Ulbright TM, Foster RS, Miller KD
Am J Surg Pathol 1997 Aug;21(8):896-904. doi: 10.1097/00000478-199708000-00003. PMID: 9255252

Peripheral neuroepithelioma (peripheral primitive neuroectodermal tumor) of the uterine cervix.

Sato S, Yajima A, Kimura N, Namiki T, Furuhashi N, Sakuma H
Tohoku J Exp Med 1996 Oct;180(2):187-95. doi: 10.1620/tjem.180.187. PMID: 9111767

The establishment and characterization of a peripheral neuroepithelioma cell line in soft tissue of extremity.

Tanaka K, Iwamoto Y, Noguchi Y, Oda Y, Sugioka Y
Lab Invest 1995 Feb;72(2):237-48. PMID: 7853855

Peripheral neuroepithelioma in childhood.

Voss BL, Pysher TJ, Humphrey GB
Cancer 1984 Dec 15;54(12):3059-64. doi: 10.1002/1097-0142(19841215)54:12<3059::aid-cncr2820541241>3.0.co;2-4. PMID: 6498781

See all (17)

MedGen

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Peripheral neuroepithelioma(PNE)

Definition

Term Hierarchy

Professional guidelines

PubMed

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Curated

Consumer resources

Reviews

Related information

Recent activity