Clear Cell Carcinoma Arising in Vulvar Endometriosis

Abstract

We report a case of vulvar clear cell adenocarcinoma in a woman presenting with a lump and pain in the right side of the labia majora. Three years prior to this visit, she underwent a total abdominal hysterectomy with bilateral salpingooophorectomy and excision of a labial mass. Pathological examination revealed adenomyosis and multiple leiomyomas in the uterus, endometriotic cysts in both ovaries, and foci of atypical endometriosis in the labial mass. The results of an incision biopsy performed before referral indicted only apocrine hidrocystoma. Physical examination revealed a hard mass at the right labia majora extending to the right groin. The mass seemed to be in continuity with the pubic symphysis that would require pubic bone excision and reconstruction with flap surgery to achieve complete resection. However, the patient refused such extensive surgery. Based on previous diagnosis of vulvar endometriosis, she had been treated with GnRH agonists and depot medroxyprogesterone acetate. However, the mass developed into an ulcer and increased in size. A second biopsy of the mass was undertaken, and the pathological diagnosis was clear cell carcinoma with coexisting atypical endometriosis. Computed tomography of the abdominopelvic region showed an ulcerative mass at the right labia majora and nodal metastasis at the external iliac and inguinal regions. Systemic chemotherapy was administered. The growth of the tumors stabilized during the first two cycles of chemotherapy but rapidly progressed thereafter. At 17 months after her initial presentation, the patient passed away due to the progression of the disease.

Diagnosis and Treatment of Vulvo-Perineal Endometriosis: A Systematic Review

“…It is difficult to distinguish benign from malignant perineal endometriosis based on symptoms and clinical examination and a biopsy or surgical excision will always be necessary to confirm the diagnosis of malignant transformation (13). Histological observations are dominated by endometrioid carcinoma and sarcoma (107), but can also present as dermatosarcoma, clear cell carcinoma and serous papillary cystadenocarcinoma (87,106,(108)(109)(110)(111)(112)(113)(114)(115)(116). As malignant transformation appears uncertain, unpredictable and may be very delayed, long-term follow-up is recommended.…”

PD-L1 Expressing Recurrent Clear Cell Carcinoma of the Vulva with Durable Partial Response to Pembrolizumab: A Case Report

“…A single case report has previously reported on a patient with recurrent clear cell vulvar cancer who received paclitaxel and carboplatin with stable disease as best response followed by disease progression and OS of 17 months. 1 Novel approaches are urgently required to improve outcomes for patients with advanced and recurrent vulvar cancer.…”

“… 1–3 This disease has been reported to arise in two distinct settings, either from transformation within a glandular appendage such as the Bartholin’s gland or sweat gland, or from extragonadal endometriosis. 1 , 3–6 Unlike squamous cell carcinomas of the vulva and vagina, no clear association with human papilloma virus infection has been reported. 7 Owing to its rarity, the biologic behavior and genomic make up of VCCC are poorly understood.…”

Primary Clear Cell Adenocarcinoma of the Vulva: A Case Study With Mutation Analysis and Literature Review

“…Only 8 other cases of primary vulvar CCC have been reported in the English literature. [3][4][5][6][7][8][9][10] The clinicopathological characteristics of these cases, including the current case, are summarized in Table 1. The age of the patients ranged from 36 to 70 years (median age, 46 years), and the episiotomy scar was the most commonly involved site (56%, 5 of 9 patients).…”