Herghelegiu Cătălin Gabriel, Neacşu Adrian, Oprescu Nuţi Daniela, Cărbunaru Ana Elena, Brăila Anca Daniela, Curea Fabiana Georgiana, Marcu Mădălina Lucia, Ioan Raluca Gabriela, Bohîlţea Roxana Elena
Department of Obstetrics and Gynecology, "Polizu" Clinical Hospital, "Alessandrescu-Rusescu" National Institute for Healthcare of Mother and Child, Bucharest, Romania;
Rom J Morphol Embryol. 2018;59(4):1233-1237.
Clear cell carcinoma (CCC) of the female genital tract usually arises in the ovary, endometrium, cervix and vagina. A rare site for CCC is the vulva, and moreover even rarer are the cases involving the Bartholin gland. A 54-year-old female was admitted for a 1.5×2 cm tumor at the level of the right Bartholin gland. The magnetic resonance imaging (MRI) exam revealed enlarged inguinal, pelvic and para-aortic lymph nodes but no other primary tumor. Microscopic examination revealed CCC. The tumor was positive for cytokeratin 7 (CK7), paired-box 8 (Pax8), napsin A and vimentin, negative for estrogen receptor (ER), progesterone receptor (PR), calretinin, cluster of differentiation 10 (CD10), carcinoembryonic antigen (CEA), p16 and p63. Also, p53 was expressed in 30-40% and Ki67 in 70% of the malignant cells. Given the clinical, imagistic, histological and immunohistochemical features of the tumor, we concluded that the tumor is a CCC of the Bartholin gland. Aim of the study is to signal a rare case of CCC of Bartholin gland. Since there are only two other cases reported in literature, the natural history and prognosis of the disease is not known, also there are no therapeutic guidelines regarding this rare tumor so appropriate treatment is uncertain. Therefore, it is important that new cases are reported for a better understanding of this rare condition. Bartholin gland carcinoma is a pathology quite rarely encountered in practice. The positive diagnosis is eminently histological and immunohistochemistry. Bartholin gland CCC is an extremely rare diagnosis with, to our knowledge, only two other cases reported in literature, but with a potential aggressive clinical behavior and poor outcome.
女性生殖道透明细胞癌（CCC）通常发生于卵巢、子宫内膜、宫颈和阴道。CCC发生于外阴较为罕见，而累及巴氏腺的病例更是罕见。一名54岁女性因右侧巴氏腺处有一个1.5×2 cm的肿瘤入院。磁共振成像（MRI）检查显示腹股沟、盆腔和腹主动脉旁淋巴结肿大，但未发现其他原发肿瘤。显微镜检查显示为CCC。肿瘤细胞角蛋白7（CK7）、配对盒8（Pax8）、 napsin A和波形蛋白呈阳性，雌激素受体（ER）、孕激素受体（PR）、钙视网膜蛋白、分化簇10（CD10）、癌胚抗原（CEA）、p16和p63呈阴性。此外，p53在30%-40%的恶性细胞中表达，Ki67在70%的恶性细胞中表达。根据肿瘤的临床、影像学、组织学和免疫组化特征，我们得出结论，该肿瘤为巴氏腺CCC。本研究的目的是报告一例罕见的巴氏腺CCC病例。由于文献中仅报道了另外两例，该病的自然史和预后尚不清楚，对于这种罕见肿瘤也没有治疗指南，因此合适的治疗方法尚不确定。因此，报告新病例对于更好地了解这种罕见疾病很重要。巴氏腺癌在实际临床中很少见。确诊主要依靠组织学和免疫组化。巴氏腺CCC是一种极其罕见的诊断，据我们所知，文献中仅报道了另外两例，但具有潜在的侵袭性临床行为和不良预后。
