Adenoid cystic carcinoma of the female genital tract – Case series with review of literature

Abstract

Abstract Introduction/Objective Adenoid cystic carcinoma (ACC) of the female genital tract is rare and the published literature is sparse. In the vulva it is considered to arise from Bartholin’s gland whereas, in cervix it arises from the reserve cells. Although morphologically ACC of female genital tract is similar to its counterparts in salivary glands, the clinical behavior is different. Methods/Case Report We describe the clinicopathologic and immunohistochemical features of three cases of adenoid cystic carcinoma of female genital tract with review of literature. The age of our patients ranged from 48 to 75 years with tumors ranging from 1.9 to 3.9 cm. The tumor sites were vulva (2 cases) and cervix (1 case). Two of the 3 patients presented with mass lesions and one patient presented with postmenopausal bleeding. Microscopically, all tumors showed classic morphologic features with cribriform, tubular and solid patterns. On immunohistochemistry, CD117 was consistently reactive in all 3 cases. In addition, the ductal and myoepithelial cell population were highlighted by keratin cocktail and p63 respectively. P16 was diffuse and block-like positive in two cases (one vulvar and cervical ACC). High risk HPV (HPV 33) was detected in one case of vulvar ACC. Two patients underwent surgical excision while one patient was deemed unfit for surgical treatment. All 3 patients received radiation therapy. On follow- up that ranged from 17 to 76 months, one patient is alive without evidence of disease. One patient died of disease with local recurrence and lung metastasis. The other patient died of complications (radiation proctocolitis with colonic perforation). Results (if a Case Study enter NA) NA Conclusion Our cases highlight the aggressive behavior of adenoid cystic carcinoma in the female genital tract and the need for close follow-up. Also high risk HPV has been demonstrated in cervical ACC previously, but our series demonstrates high risk HPV association with vulvar ACC.