疑似直腸癌之原發性外陰巴氏腺亮細胞腺癌

Primary vulvar clear cell carcinoma of a Bartholin's gland mimicking rectal carcinoma

原發性外陰巴氏腺亮細胞腺癌是罕見且機轉未明的。有效的治療選項目前也未知。關於這個主題，這是第一個文獻回顧。58歲女性抱怨一個的無痛性肛門腫塊有20年，初步懷疑為直腸癌;然而，檢體報告為婦科起源的腺癌。隨後，該患者接受根除性外陰切除術。病理報告為外陰巴氏腺亮細胞腺癌。術後輔助性放療正在計畫中。亮細胞腺癌一般發生在更年期附近。會陰內子宮內膜異位症的惡性轉化為可能的機轉。治療包括根除性外陰切除術及腹股溝淋巴結清除術。建議使用輔助放療和鉑金類化學治療以降低最大程度的復發風險。

Primary vulvar clear cell carcinoma (CCC) of a Bartholin's gland is rare and poorly understood. There is uncertainly regarding effective treatment options. This is the first literature review on this topic. Our patient, a 58-year-old woman complained of a painless lump for 20 years. We initially suspected rectal carcinoma; however, her biopsy report indicated an adenocarcinoma of gynecologic origin. Subsequently, the patient underwent a radical vulvectomy. Analysis indicated a CCC originating from the Bartholin's gland. Adjuvant radiotherapy was subsequently planned. A CCC generally occurs during peri-menopausal years. Malignant transformation of endometriosis within the vulva may be the possible mechanism. Primary treatment includes radical total vulvectomy with inguinal lymphadenectomy (LN). Adjuvant radiation therapy and platinum-based chemotherapy are suggested to minimize risk of recurrence.