Abstract
Background
Ewing’s sarcoma (ES)/primitive neuroectodermal tumor (PNET) of the genital tract of women is uncommon. Presentation in vagina is exceedingly rare.
Case
A 54-year-old Chinese woman presented with complaints of vaginal bleeding with fragmented bits occasionally. Physical examination revealed two nodules about 4 cm × 3 cm in the vagina. The immunohistochemical stains revealed a rare presentation of a primitive neuroectodermal tumor. The patient was treated with whole-pelvis external beam radiation, vaginal cylinder intracavitary brachytherapy, intracavitary afterloading radiotherapy and bilateral groin area radiotherapy. She died of clinical recurrence after 18 months of treatment.
Conclusion
This is the oldest but virgin case, treated with radiation completely, of primitive neuroectodermal tumor located in the vagina reported in the literature.
Similar content being viewed by others
References
Fukunaga M, Nomura K, Endo Y, Ushigome S, Aizawa S (1996) Carcinosarcoma of the uterus with extensive neuroectodermal differentiation. Histopathology 29:565–570
Tsao AS, Roth LM, Sandler A, Hurteau JA (2001) Cervical primitive neuroectodermal tumor. Gynecol Oncol 83:138–142
Kleinman GM, Young RH, Scully RE (1993) Primary neuroectodermal tumors of the ovary. A report of 25 cases. Am J Surg Pathol 17:764–778
Vang R, Taubenberger JK, Mannion CM, Bijwaard K, Malpica A, Ordonez NG, Tavassoli FA, Silver SA (2000) Primary vulvar and vaginal extraosseous Ewing’s sarcoma/peripheral neuroectodermal tumor: diagnostic confirmation with CD99 immunostaining and reverse transcriptase-polymerase chain reaction. Int J Gynecol Pathol 19:103–109
Farley J, O’Boyle JD, Heaton J, Remmenga S (2000) Extraosseous Ewing sarcoma of the vagina. Obstet Gynecol 96:832–834
Petković M, Zamolo G, Muhvić D, Coklo M, Stifter S, Antulov R (2002) The first report of extraosseous Ewing’s sarcoma in the rectovaginal septum. Tumori 88:345–346
Liao X, Xin X, Lü X (2004) Primary Ewing’s sarcoma-primitive neuroectodermal tumor of the vagina. Gynecol Oncol 92:684–688
McCluggage WG, Sumathi VP, Nucci MR, Hirsch M, Dal Cin P, Wells M, Flanagan AM, Fisher C (2007) Ewing family of tumours involving the vulva and vagina: report of a series of four cases. J Clin Pathol 60:674–680
Al-Tamimi H, Al-Hadi AA, Al-Khater AH, Al-Bozom I, Al-Sayed N (2009) Extraskeletal neuroectodermal tumour of the vagina: a single case report and review. Arch Gynecol Obstet 280:465–468
Rekhi B, Qureshi S, Basak R, Desai SB, Medhi S, Kurkure P, Menon S, Maheshwari A, Jambhekar NA (2010) Primary vaginal Ewing’s sarcoma or primitive neuroectodermal tumor in a 17-year-old woman: a case report. J Med Case Rep 4:88
Peres E, Mattoo TK, Poulik J, Warrier I (2005) Primitive neuroectodermal tumor (PNET) of the uterus in a renal allograft patient: a case report. Pediatr Blood Cancer 44:283–285
Angervall L, Enzinger FM (1975) Extraskeletal neoplasm resembling Ewing’s sarcoma. Cancer 36:240–251
Grier HE, Krailo MD, Tarbell NJ, Link MP, Fryer CJ, Pritchard DJ, Gebhardt MC, Dickman PS, Perlman EJ, Meyers PA, Donaldson SS, Moore S, Rausen AR, Vietti TJ, Miser JS (2003) Addition of ifosfamide and etoposide to standard chemotherapy for Ewing’s sarcoma and primitive neuroectodermal tumor of bone. N Engl J Med 348:694–701
de Alava E, Gerald WL (2000) Molecular biology of the Ewing’s sarcoma/primitive neuroectodermal tumor family. J Clin Oncol 18:204–213
Raney RB, Asmar L, Newton WA Jr, Bagwell C, Breneman JC, Crist W, Gehan EA, Webber B, Wharam M, Wiener ES, Anderson JR, Maurer HM (1997) Ewing’s sarcoma of soft tissues in childhood: a report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991. J Clin Oncol 15:574–582
Gururangan S, Marina NM, Luo X, Parham DM, Tzen CY, Greenwald CA, Rao BN, Kun LE, Meyer WH (1998) Treatment of children with peripheral primitive neuroectodermal tumor or extraosseous Ewing’s tumor with Ewing’s-directed therapy. J Pediatr Hematol Oncol 20:55–61
Ahmad R, Mayol BR, Davis M, Rougraff BT (1999) Extraskeletal Ewing’s sarcoma. Cancer 85:725–731
Conflict of interest
None.
Author information
Authors and Affiliations
Corresponding authors
Additional information
The authors Fang Wen and Yi Zhang contributed equally to this work.
Rights and permissions
About this article
Cite this article
Pang, X., Chen, P., Wen, F. et al. Primary Ewing’s sarcoma/primitive neuroectodermal tumor of the vagina in a 54-year-old woman: a case report. Arch Gynecol Obstet 285, 1031–1033 (2012). https://doi.org/10.1007/s00404-011-2084-6
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00404-011-2084-6