Iberoamerican Journal of Medicine
Iberoamerican Journal of Medicine
Case Report

Proximal epithelioid sarcoma of the vulva. A rare neoplasm. A clinicopathological case

Sarcoma epitelioide proximal de la vulva. Una neoplasia rara. Un caso clínico-patológico

Rosalía Sarabia Ochoa, Juan Pablo García de la Torre, Antonio Amezcua Recover

Downloads: 0
Views: 509


Epithelioid sarcoma is a rare aggressive soft tissue sarcoma, which can be distal or proximal types. The classic form (distal-type) of epithelioid sarcoma mainly occurs in teenagers and young adults. A rarer form, called large-cell (proximal-type) epithelioid sarcoma, tends to be more aggressive and mainly affects adults. The proximal subtype mostly arises from the proximal pelvis, limbs, and genital tract.
We report a case of a 59 -year-old female, presented with a progressively growing mass in the left labia majora. Gynecologic examination revealed a 2 cm mobile and painless mass that was not attached to deep planes.
The histological study showed a multinodular tumor was seen comprising sheets of oval to polygonal cells with moderate amount of cytoplasm. Interspersed were larger, rhabdoid cells with abundant eosinophilic cytoplasm and prominent nucleoli. On IHC, the tumor cells showed positivity for EMA and CKAE1/AE3 and do not expressed INI-1 in the nucleus. All tumor cells were negative for S-100 protein and CD34. The histopathological diagnosis was soft tissue of the vulvar region with proximal epithelioid sarcoma.
The patient received adjuvant external pelvic radiotherapy and brachytherapy in the vulvar bed. Currently, 3 years after diagnosis, the patient does not present signs of tumor recurrence in her controls.
Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment. The prognosis is generally poor. A wide excision with clear margins is imperative with options of post-operative CT/RT in individual cases during a close follow-upbehavior, as seen in our case.


Vulva; Epithelioid sarcoma; Proximal epithelioid sarcoma


El sarcoma epitelioide es un sarcoma de tejido blando agresivo poco frecuente, que puede ser de tipo distal o proximal. La forma clásica (tipo distal) de sarcoma epitelioide se presenta principalmente en adolescentes y adultos jóvenes. Una forma más rara, llamada sarcoma epitelioide de células grandes (tipo proximal), tiende a ser más agresiva y afecta principalmente a adultos. El subtipo proximal surge principalmente de la pelvis proximal, las extremidades y el tracto genital.
Presentamos el caso de una mujer de 59 años, que presentó una masa de crecimiento progresivo en labios mayores izquierdos. El examen ginecológico reveló una masa móvil e indolora de 2 cm que no estaba adherida a planos profundos.
El estudio histológico mostró un tumor multinodular compuesto por láminas de células de forma ovalada a poligonal con moderada cantidad de citoplasma. Intercaladas había células rabdoides más grandes con abundante citoplasma eosinófilo y nucléolos prominentes. En IHC, las células tumorales mostraron positividad para EMA y CKAE1/AE3 y no expresaron INI-1 en el núcleo. Todas las células tumorales fueron negativas para la proteína S-100 y CD34. El diagnóstico histopatológico fue tejido blando de la región vulvar con sarcoma epitelioide proximal.
La paciente recibió radioterapia pélvica externa adyuvante y braquiterapia en el lecho vulvar. Actualmente, a 3 años del diagnóstico, la paciente no presenta signos de recurrencia tumoral en sus controles.
Debido a su baja incidencia, no existen algoritmos de diagnóstico basados en evidencia ni recomendaciones de tratamiento publicadas. El pronóstico es generalmente malo. Es imperativa una escisión amplia con márgenes claros con opciones de CT/RT postoperatoria en casos individuales durante un seguimiento cercano, como se observa en nuestro caso.

Palabras clave

Vulva; Sarcoma epitelioide; Sarcoma epitelioide proximal


1. Guillou L, Wadden C, Coindre JM, Krausz T, Fletcher CD. "Proximal-type" epithelioid sarcoma, a distinctive aggressive neoplasm showing rhabdoid features. Clinicopathologic, immunohistochemical, and ultrastructural study of a series. Am J Surg Pathol. 1997;21(2):130-46. doi: 10.1097/00000478-199702000-00002.
2. Frezza AM, Sbaraglia M, Lo Vullo S, Baldi GG, Simeone N, Frenos F, et al. The natural history of epithelioid sarcoma. A retrospective multicentre case-series within the Italian Sarcoma Group. Eur J Surg Oncol. 2020;46(7):1320-6. doi: 10.1016/j.ejso.2020.03.215.
3. Czarnecka AM, Sobczuk P, Kostrzanowski M, Spalek M, Chojnacka M, Szumera-Cieckiewicz A, et al. Epithelioid Sarcoma-From Genetics to Clinical Practice. Cancers (Basel). 2020;12(8):2112. doi: 10.3390/cancers12082112.
4. Han CH, Li X, Khanna N. Epithelioid sarcoma of the vulva and its clinical implication: A case report and review of the literature. Gynecol Oncol Rep. 2016;15:31-3. doi: 10.1016/j.gore.2016.01.001.
5. Yahiro S, Fujimoto T, Fujita I, Takai T, Sakuma T, Sudo T, et al. Proximal-type epithelioid sarcoma in pubic region expressing L-type amino acid
transporter 1: A case report. SAGE Open Med Case Rep. 2022;10:2050313X211067917. doi: 10.1177/2050313X211067917.
6. Noh JJ, Jeon JE, Jung H, Kim HS, Lee YY, Choi CH, et al. Vulvar epithelioid sarcoma: A case report with literature review. Taiwan J Obstet Gynecol. 2021;60(1):132-5. doi: 10.1016/j.tjog.2020.11.020.
7. Chiyoda T, Ishikawa M, Nakamura M, Ogawa M, Takamatsu K. Successfully treated case of epithelioid sarcoma of the vulva. J Obstet Gynaecol Res. 2011;37(12):1856-9. doi: 10.1111/j.1447-0756.2011.01637.x.
8. Jeon SE, Lee J, Jung HJ, Kim BJ, Ryu SY, Park SI, et al. An extremely rare elderly case of proximal epithelioid sarcoma of the vulva: case report with a review of literatures. Obstet Gynecol Sci. 2020;63(5):670-4. doi: 10.5468/ogs.20076.
9. Ong AC, Lim TY, Tan TC, Wang S, Raju GC. Proximal epithelioid sarcoma of the vulva: a case report and review of current medical literature. J Obstet Gynaecol Res. 2012;38(7):1032-5. doi: 10.1111/j.1447-0756.2011.01819.x.
10. Sundaram A, Elangovan A, Rajwanshi A, Srinivasan R, Kapoor R. Proximal-type epithelioid sarcoma of the vulva: Cytopathological diagnosis of a rare neoplasm. Cytopathology. 2018;29(5):471-3. doi: 10.1111/cyt.12561.
11. Li Y, Cao G, Tao X, Guo J, Wu S, Tao Y. Clinicopathologic features of epithelioid sarcoma: report of seventeen cases and review of literature. Int J Clin Exp Pathol. 2019;12(8):3042-8.
12. Kim HJ, Kim MH, Kwon J, Kim JY, Park K, Ro JY. Proximal-type epithelioid sarcoma of the vulva with INI1 diagnostic utility. Ann Diagn Pathol. 2012;16(5):411-5. doi: 10.1016/j.anndiagpath.2011.04.002.

Submitted date:

Reviewed date:

Accepted date:

Publication date:

653cf50ca953956bb84e9bb5 iberoamericanjm Articles
Links & Downloads

Iberoam J Med

Share this page
Page Sections