Epithelioid Sarcoma of the Vulva in a 17-year-old Pregnant Woman

Abstract

Epithelioid sarcoma of the vulva is an extremely rare neoplasm with aggressive behavior and poor outcome. Herein, we report a case of vulvar epithelioid sarcoma presenting in a 17-year-old very early in her pregnancy. The patient presented with an asymptomatic nodule of the right labia majora of 1-year duration. Computerized tomographic scans showed enlarged inguinal lymph nodes and numerous lung nodules. Positron emission tomography was performed and revealed no suspicious lesions for metastatic disease. The patient underwent local excision of her vulvar lesion. On the basis of morphology and extensive immunohistochemistry, the lesion was classified as epithelioid sarcoma. The patient was referred to radiation therapy and upon evaluation, she was found to be in her sixth week of gestation. The patient continued with her pregnancy and underwent a lymph node dissection, which was positive. We review the literature of only the well-documented cases extensively studied by immunohistochemical analysis. We summarize the clinical presentation, clinical impression, treatment modalities, and outcomes of these cases. On account of the rarity of this disease in the vulva, there is no clear consensus on treatment modalities, but it seems that early aggressive surgical resection is the treatment of choice, with the role of adjuvant therapy to be determined.

Epithelioid sarcoma of the vulva and its clinical implication: A case report and review of the literature

“…As PES of the vulva often develops in reproductive aged women, maintenance of pregnancy and/or fertility should be considered in treating patients ( Chiyoda et al, 2011 , Moore et al, 2002 , Rai et al, 2009 ). However, the clinical behavior is unfortunately not different from that of other non-pregnant patients, and can lead to a dismal outcome in an effort to avoid surgery during pregnancy ( Moore et al, 2002 ).…”

Update on SWI/SNF‐related gynecologic mesenchymal neoplasms: SMARCA4‐deficient uterine sarcoma and SMARCB1‐deficient vulvar neoplasms

“…Epithelioid sarcoma of the vulva is rare, with fewer than 70 reported cases 59,94‐120 . However, of the116 epithelioid sarcomas diagnosed by one of us (ALF) over the past 14 years, 13% were from the vulva/perineum.…”

Vulvar sarcomas: Short guideline for histopathological recognition and clinical management. Part 1

“…23 The prognosis is poor because of the aggressive nature of the tumor. 29 According to a survey, 86% of patients relapse, 29% develop early lymph node metastases, and 64% die within 3 years after diagnosis. 21 Proximal-type epithelioid sarcoma represents a rare variant of this type of sarcoma with features of both the 'atypical' epithelioid sarcoma, and a rhabdoid tumor or undifferentiated carcinoma.…”