Proximal epithelioid sarcoma of the vulva

Epithelioid sarcoma (ES) is an extremely rare disease that, according to morphological data, can be divided into proximal and distal types. The proximal type of ES (PES) of the vulva arises from the superficial and deep layers of the external genital organs, manifests as single or multiple soft tissue tumor nodules with areas of necrosis and hemorrhage, is characterized by aggressive behavior, and has an unfavorable prognosis due to its high tendency for local recurrence and hematogenous metastasis. Differential diagnosis is performed with various benign and malignant neoplasms, cysts or abscesses of the Bartholin gland, and inguinal and femoral hernias. The final diagnosis and histological type of the tumor are established based on morphological, immunohistochemical, and molecular genetic studies. PES is characterized by solid tumor growth composed of large and pleomorphic epithelioid cells with large vesicular nuclei and distinct eosinophilic nucleoli. Immunohistochemically, loss of SMARCB1 (INI1, BAF47) expression is observed, along with positive expression of EMA, vimentin, and cytokeratins; a positive reaction for CD34 staining is often noted in the absence of expression of other endothelial markers such as CD31 and FLI‑1. The clinical presentation and disease characteristics may suggest PES and justify the expansion of the immunohistochemical marker panel. Timely and accurate detection of this tumour plays a key role in improving treatment outcomes, enhancing patients’ quality of life, and reducing mortality. However, due to its rarity, PES of the vulva presents objective diagnostic difficulties in both clinical and morphological aspects. Surgical intervention remains the main method of treatment for this pathology, and to date, there are no clearly established recommendations regarding the optimal management strategy for patients with PES of the vulva. This paper presents a clinical case of a 65‑year-old patient whose atypical clinical presentation of recurrent vulvar tumor served as the basis for expanding the panel of immunohistochemical markers, which allowed for the diagnosis of PES of the vulva.

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