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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: 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]

Professional guidelines

PubMed

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

Curated

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

Recent clinical studies

Etiology

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
Devoe K, Weidner N
Semin Diagn Pathol 2000 Aug;17(3):216-24. PMID: 10968707
d'Amore ES, Ninfo V
Semin Diagn Pathol 1996 Aug;13(3):184-203. PMID: 8875709
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
Dehner LP
Arch Pathol Lab Med 1986 Nov;110(11):997-1005. PMID: 3535732

Diagnosis

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
Devoe K, Weidner N
Semin Diagn Pathol 2000 Aug;17(3):216-24. PMID: 10968707
Helton KJ, Fletcher BD, Kun LE, Jenkins JJ 3rd, Pratt CB
Cancer 1993 May 1;71(9):2847-53. doi: 10.1002/1097-0142(19930501)71:9<2847::aid-cncr2820710928>3.0.co;2-e. PMID: 8467462
Huntrakoon M
Ultrastruct Pathol 1987;11(4):411-9. doi: 10.3109/01913128709048435. PMID: 3617227
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

Therapy

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
Williams S, Parham DM, Jenkins JJ 3rd
Pediatr Dev Pathol 1999 Jan-Feb;2(1):42-9. doi: 10.1007/s100249900088. PMID: 9841705
Kelessis NG, Apostolikas NN, Filopoulos EJ, Griniatsos JK, Vassilopoulos PP
Anticancer Res 1996 Sep-Oct;16(5B):3193-5. PMID: 8920788
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
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

Prognosis

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
Stephenson CF, Bridge JA, Sandberg AA
Hum Pathol 1992 Nov;23(11):1270-7. doi: 10.1016/0046-8177(92)90295-e. PMID: 1330877
Llombart-Bosch A, Terrier-Lacombe MJ, Peydro-Olaya A, Contesso G
Hum Pathol 1989 Mar;20(3):273-80. doi: 10.1016/0046-8177(89)90136-6. PMID: 2656493
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
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

Clinical prediction guides

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
Tanaka K, Iwamoto Y, Noguchi Y, Oda Y, Sugioka Y
Lab Invest 1995 Feb;72(2):237-48. PMID: 7853855
Füzesi L, Heller R, Schreiber H, Mertens R
Pathol Res Pract 1993 Mar;189(2):235-41; discussion 241-4. doi: 10.1016/S0344-0338(11)80104-4. PMID: 8321753
Whang-Peng J, Freter CE, Knutsen T, Nanfro JJ, Gazdar A
Cancer Genet Cytogenet 1987 Nov;29(1):155-7. doi: 10.1016/0165-4608(87)90043-4. PMID: 3478125
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

Supplemental Content

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    Curated

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

    Consumer resources

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