High-Grade Transformation in Adenoid Cystic Carcinoma of the Bartholin Gland: Case Report

Abstract

Introduction In the present study, we report a case of primary adenoid cystic carcinoma (ACC) of the Bartholin gland with high-grade transformation (HGT). Adenoid cystic carcinoma of the Bartholin gland is a rare tumor and HGT has only been reported in head and neck tumors. Case Report A 77-year-old woman with a non-ulcerated vulvar lesion on the topography of the right Bartholin gland. The patient was submitted to tumor resection followed by V–Y island flap and adjuvant radiotherapy. The histopathological examination revealed primary ACC of the Bartholin gland, with areas of HGT and extensive perineural invasion. The immunohistochemical study with p53 showed a diffuse and strong positive reaction in areas with HGT. After 24 months of follow-up, the patient presented distant metastases and died, despite having undergone to chemotherapy. Conclusion As far as we know, this case is the first description in the literature of HGT in ACC of the Bartholin gland, and HGT appears to be associated with tumor aggressiveness.

Management of primary and recurrent Bartholin’s gland carcinoma: A systematic review on behalf of MITO Rare Cancer Group

“…Details about number of patients, age, FIGO stage at presentation and histotype are reported in Table 1. 6 -41…”

“…14 Distant recurrent disease was managed only with RT for liver and pulmonary relapses 14,17 and with surgery for pulmonary relapses, 25,31 but required CHT alone or in association in all other cases. 9,11,14,26,30,35…”

“…Detailed data on patients’ recurrence site, secondary treatment and outcome are summarized in Table 2. 6 -41…”

“…14 Distant recurrent disease was managed only with RT for liver and pulmonary relapses 14,17 and with surgery for pulmonary relapses, 25,31 but required CHT alone or in association in all other cases. 9,11,14,26,30,35 Survival status was reported only in 106 patients (28.8%). In the majority of the considered cases women had no evidence of disease at follow up (68%), while about 20% of patients were alive with disease and 12% had died with disease.…”