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.

【摘要】本研究报道1例伴高级别转化（high-grade transformation, HGT）的原发性巴氏腺（Bartholin gland）腺样囊性癌（adenoid cystic carcinoma, ACC）。巴氏腺腺样囊性癌为罕见肿瘤，而高级别转化此前仅见于头颈部肿瘤病例。

病例报告：患者为77岁女性，右侧巴氏腺区域出现非溃疡性外阴病灶。患者接受肿瘤切除术，随后行V-Y岛状皮瓣修复，并辅以术后辅助放疗。组织病理学检查显示为原发性巴氏腺腺样囊性癌，可见高级别转化灶及广泛神经周围浸润。p53免疫组化检测结果显示，高级别转化区域呈弥漫性强阳性表达。经24个月随访，尽管患者接受了化疗，仍出现远处转移并死亡。

结论：据我们所知，本病例为文献中首次报道巴氏腺腺样囊性癌伴高级别转化的病例，且高级别转化似乎与肿瘤侵袭性密切相关。