Malignant perivascular epithelioid tumor of the vagina: Report of a rare case with brief review of literature

Abstract

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors with immunohistochemical co‐expression of melanocytic and myoid markers. Vaginal PEComas have been described in only nine cases so far. We describe the case of a 65‐year‐old female with a large growth in the left lateral vaginal wall. Biopsy imprint smears showed dispersed tumor cells with anisonucleosis, multinucleation, and bizarre forms, suggestive of a malignant tumor. Histopathology, however, showed perivascular arrangement of clear epithelioid cells, focal necrosis, intracellular brown pigment in few cells, and mitotic activity at 2 to 3 per 50 high power fields. Immunohistochemical positivity for vimentin, HMB‐45, S‐100 protein, desmin, and MyoD1 assisted in rendering a final pathological diagnosis of malignant PEComa of the vagina. Further work‐up revealed metastatic deposits in liver and retroperitoneal lymph nodes. PEComa arising in vagina is an unusual phenomenon with the malignant variant being an extremely rare tumor. Awareness of the characteristic morphology and utilization of a panel of immunohistochemical stains are mandatory to be able to make a precise diagnosis and appropriate prognostication.

Effect of High-Quality Nursing Intervention on the Quality of Life and Psychological State of Tumor Patients Undergoing First Chemotherapy

“…A malignant tumor is a health concern that imposes considerable threats on human beings [ 1 , 2 ]. The malignant tumor features infinite proliferation, and it would wildly proliferate if effective measures are not provided.…”

Perivascular epithelioid cell tumors (PEComa) of the gynecologic tract

“…The uterine corpus is the most common site for gynecologic PEComas; however, they have also been reported in the cervix, 4‐18 vagina, 5,19‐26 broad ligament, 5,27‐33 ovary, 23,34‐38 and rarely, the vulva 39 . Overall, most gynecologic PEComas present in patients in their fifth to sixth decades (range 6‐79 years), 5,17,23,24,40‐42 but those in the vagina, broad ligament, and vulva may occur at an earlier age (mean 28, 25, and 20 years, respectively) 43 .…”

Tumor de células epitelioides perivasculares (PEComa) uterino en una paciente pediátrica: reporte de caso

“…El cuerpo uterino es el sitio más común de PEComas ginecológicos; sin embargo, también se han reportado en el cuello uterino (4,5) vagina (6,7,8), ligamento ancho (6,9,10), ovario (11,12) y la vulva (13). La presentación clínica es inespecífica e incluye sangrado uterino anormal, dolor abdominopélvico o identificación de una masa en imágenes diagnósticas (14,15).…”