Primitive neuroectodermal tumor originating in the vulva: A case report

Matsuda,Makiko; Ichimura,Tomoyuki; Kasai,Mari; Murakami,Makoto; Hoshi,Manabu; Kawamura,Naoki; Sumi,Toshiyuki

doi:10.3892/ol.2014.2073

Primitive neuroectodermal tumor originating in the vulva: A case report

Authors:
- Makiko Matsuda
- Tomoyuki Ichimura
- Mari Kasai
- Makoto Murakami
- Manabu Hoshi
- Naoki Kawamura
- Toshiyuki Sumi
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Affiliations: Department of Obstetrics and Gynecology, Osaka City University Graduate School of Medicine, Osaka 545‑8585, Japan, Department of Obstetrics and Gynecology, Osaka City Sumiyoshi Hospital, Osaka 559-0012, Japan, Department of Orthopedic Surgery, Osaka City University Graduate School of Medicine, Osaka 545‑8585, Japan, Department of Obstetrics and Gynecology, Osaka City General Hospital, Osaka 534-0021, Japan
Published online on: April 16, 2014 https://doi.org/10.3892/ol.2014.2073
Pages: 187-189

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Abstract

Primitive neuroectodermal tumors (PNETs) exhibit chromosomal translocations in common with those of Ewing's sarcoma. They usually originate in bone or soft tissue but rarely arise in the vulva. The current case report presents a case of PNET originating in the vulva in a 60‑year‑old female, who previously underwent enucleation of a vulvar tumor in another hospital. The pathologist suspected a histopathological diagnosis of PNET, and simple vulvectomy and resection of the inguinal lymph nodes were performed. An ~3 cm mass recurred in the right side of the vulva four years following the initial surgery and the tumor was excised. The tumor comprised small, round‑to‑oval nuclei and stained positively for MIC‑2, synaptophysin, neuron‑specific enolase and neurofilament antibodies. To date, the patient remains alive and with no evidence of disease four years following multidisciplinary treatment, despite PNETs usually exhibiting a poor prognosis. This is due to the small tumor size and the absence of distant metastasis.

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1	Hart NH and Earle KM: Primitive neuroectodermal tumors of the brain in children. Cancer. 32:890–897. 1973.
2	Stout AP: A tumor of the ulnar nerve. Proc NY Pathol Soc. 18:2–12. 1918.
3	Chen L: Primitive neuro ectodermal tumor of the uterus. Arch Histopathol D D. 16:33–36. 2009.
4	Fischer G, Odunsi K, Lele S and Mhawech P: Ovarian primary primitive neuroectodermal tumor coexisting with endometrioid adenocarcinoma: a case report. Int J Gynecol Pathol. 25:151–154. 2006.
5	Halil S, Kucuk M, Arvas M, et al: Peripheral primitive neuroectodermal tumor (PNET) of the vulva: a case report. Eur J Gynaecol Oncol. 32:117–118. 2011.
6	Scherr GR, d’Ablaing G III and Ouzounian JG: Peripheral primitive neuroectodermal tumor of the vulva. Gynecol Oncol. 54:254–258. 1994.
7	Takeshima N, Tabata T, Nishida H, et al: Peripheral primitive neuroectodermal tumor of the vulva: report of a case with imprint cytology. Acta Cytol. 45:1049–1052. 2001.
8	McCluggage WG, Sumathi VP, Nucci MR, et al: Ewing family of tumors involving the vulva and vagina: report of series of four cases. J Clin Pathol. 60:674–680. 2007.
9	Fong YE, López-Terrada D and Zhai QJ: Primary Ewing sarcoma/peripheral primitive neuroectodermal tumor of the vulva. Hum Pathol. 39:1535–1539. 2008.
10	Dehner LP: Primitive neuroectodermal tumor and Ewing’s sarcoma. Am J Surg Pathol. 17:1–13. 1993.
11	de Alava E and Gerald WL: Molecular biology of the Ewing’s sarcoma/primitive neuroectodermal tumor family. J Clin Oncol. 18:204–213. 2000.
12	Dagher R, Pham TA, Sorbara L, et al: Molecular confirmation of Ewing sarcoma. J Pediatr Hematol Oncol. 23:221–224. 2001.
13	Iwamoto Y: Diagnosis and treatment of Ewing’s sarcoma. Jpn J Clin Oncol. 37:79–89. 2007.
14	Esiashvili N, Goodman M and Marcus RB Jr: Changes in incidence and survival of Ewing sarcoma patients over the past 3 decades: Surveillance Epidemiology and End Results data. J Pediatr Hematol Oncol. 30:425–430. 2008.
15	Jain S and Kapoor G: Chemotherapy in Ewing’s sarcoma. Indian J Orthop. 44:369–377. 2010.
16	Tanaka K, Matsunobu T, Sakamoto A, Matsuda S and Iwamoto Y: High-dose chemotherapy and autologous peripheral blood stem-cell transfusion after conventional chemotherapy for patients with high-risk Ewing’s tumors. J Orthop Sci. 7:477–482. 2002.
17	Rodriguez-Galindo C, Billups CA, Kun LE, et al: Survival after recurrence of Ewing tumors: the St. Jude Children’s Research Hospital experience, 1979–1999. Cancer. 94:561–569. 2002.