An extremely rare elderly case of proximal epithelioid sarcoma of the vulva: case report with a review of literatures

Abstract

We experienced an extremely rare case of proximal epithelioid sarcoma (PES) of the vulva in a 77-year-old woman. After history taking and physical examination, the patient was tentatively diagnosed as having Bartholin's cyst in the right labium. Based on histopathological and immunohistochemical (IHC) findings, however, a final diagnosis of PES of the vulva was made. After receiving CyberKnife treatment, the patient survived but with recurrent episodes and poor prognosis. In conclusion, our case indicates that patients with PES of the vulva should be appropriately managed with radiotherapy after a differential diagnosis based on histopathological and IHC findings.

Keywords: Diagnosis, differential; Immunohistochemistry; Sarcoma; Vulva; Vulvar neoplasms.

Fig. 1.

Gross, histopathological, and immunohistochemical findings. (A) Shown is the gross finding of a mass of 3×2 cm in size in the skin over the right lower labia, which is suggestive of Bartholin’s cyst in the right labium. (B) The histopathological examination result shows a poorly differentiated malignant neoplasm with a plump cytoplasm and eccentric nuclei. (C) From left to right are the positive immunohistochemical findings for CD34, pan-cytokeratin, and vimentin.

Fig. 2.

Findings from imaging studies. (A) The chest computed tomography (CT) scans show multiple bone metastases. (B) The abdominal and pelvic CT and chest CT scans show findings suggestive of multiple peritoneal carcinomatosis. (C) The chest CT scans show a caval lymph node metastasis.