巴氏腺腺样囊性癌临床病理分析

目的 探讨巴氏腺腺样囊性癌的临床病理特征和治疗方法.方法 回顾性分析6例巴氏腺腺样囊性癌患者的临床及病理资料.6例患者的中位年龄为40.5岁(30～54岁).主要治疗方式为手术切除,其中单纯外阴肿物剥除术1例,外阴根治性切除术加双侧腹股沟淋巴结清扫或活检术4例,外阴局部扩大切除术加双侧腹股沟淋巴结活检术1例.有2例患者术后补充放疗.6例患者均随访至2009年4月1日,中位随访时间124.5个月(8～241个月).结果 6例巴氏腺腺样囊性癌均经病理确诊,肿瘤细胞呈筛状排列及侵犯神经是其典型的病理特点.术后病理显示,切缘阳性2例,阴性1例,邻近肿瘤1例,不详2例.腹股沟淋巴结阴性5例,不详1例.有4例患者复发,其中3例局部复发,后出现肺转移;1例仅出现肺转移.在复发患者中,死亡1例,生存时间为135个月;另3例患者分别带瘤生存120、30和36个月,总生存时间分别为241、128和103个月.2例无复发患者无瘤生存8个月和121个月.结论 巴氏腺腺样囊性癌生长缓慢,患者长期预后较好,但容易局部复发和肺转移.首选治疗方法为手术切除,对于术后切缘阳性、局部浸润较深或侵犯神经者以及复发无法手术者可行辅助放疗或姑息性放疗.

Clinicopathological characteristics of six patients with adenoid cystic carcinoma of the Bartholin gland

Objective To evaluate the clinicopathological characteristics and treatment of adenoid cystic carcinoma of the Bartholin gland.Methods The clinicopathological data of six patients with adenoid cystic carcinoma of the Bartholin gland were retrospectively analyzed.The median age was 40.8 years(range 30 to 54 years).Surgery was the primary treatment.Simple vulvar tumor resection was performed in 1 patient.Four cases underwent radical vulvectomy with bilateral inguinal lymph node dissection and 1 case underwent wide local excision of the vulva with bilateral inguinal lymph node biopsy.Two cases with high risk factors received postoperative radiotherapy.Results All patients had definite pathological diagnosis.Cribriform arrangement of tubules and gland-like elements and infiltration of perineural spaces were two main microscopic features of this type of tumor.The pathological examination after surgery revealed that two patients had positive surgical margins,one had negative margin,1 adjacent to the tumor and 1 unknown;5 cases had negative inguinal lymph nodes and 1 unknown.All the 6 patients were followed-up.Recurrence developed in 4 cases including 3 with both local recurrence and lung metastasis,and one had lung metastasis only.One patient died of lung matastasis and her total survival period was 135 months.The other 3 recurrent patients survived with tumor and the total survival period was 241,128 and 103 months,respectively.Two cases without recuccrence survived 8 and 121 months,respectively.Conclusion Adenoid cystic carcinoma of the Bartholin gland is a slow growing but locally very aggressive neoplasm with a high capacity for local recurrence and lung metastasis.Surgery is the most common and useful treatment.Radiation is a choice of treatment for patients with high risk factors after surgery such as positive surgical margin,deep local invasion and infiltration of perineural spaces or for recurrent patients without opportunity of excision.