肝胆管囊性肿瘤临床病理特点及治疗(附8例报告)

Clinicopathological features and treatment of hepatobiliary cystic neoplasms: a report of 8 cases

目的 分析肝胆管囊性肿瘤(囊腺瘤和囊腺癌)的临床、病理特点及治疗。探讨其中卵巢样间质的免疫表型及组织来源。方法 回顾性分析7例肝胆管囊腺瘤和1例囊腺癌的临床表现、病理特征及治疗结果。对4例卵巢样间质细胞行CD117、CD34、ER、PR、Vim、SMA、S-100、Desmin等免疫组织化学检测。结果8例病人中女性7例,男性1例。表现为上腹不适,伴黄疸、腹泻或闭经等。3例为手术后复诊病例。镜下见部分囊腺瘤内衬上皮明显异型增生。检测4例囊腺瘤中的卵巢样间质细胞,4例均表达CD117、Vim及SMA;分别有3例表达CD34及ER、PR;2例表达S-100;Desmin无表达。肿瘤全部切除后无复发;部分切除或同时做内引流术均复发。结论肝胆管囊性肿瘤好发于女性。上腹不适是其常见表现。术前诊断困难。手术完全切除是最佳治疗方案。在肝胆管囊腺瘤和囊腺癌间存在交界性病变。推测肿瘤来源于内、中两个胚层。卵巢样间质成分来源于原始间质细胞,具有平滑肌和神经双向分化,其生长可能依赖神经和雌、孕激素调节。

Objective To discuss clinicopathological features of hepatobiliary cystic neoplasms (cystadenoma and cystadenocarcinoma), analyze the best methods for its treatment and explore the immune phenotype and origin of the ovarian-like stroma cells. Methods Clinical manifestations, pathological features and therapeutic outcome in 7 patients with cystadenoma and 1 with cystadenocarcinoma treated in our hospital were retrospectively analyzed. The immunohistochemical antibodies of CD117, CD34, ER, PR, Vim, SMA, S-100 and Desmin etc. were used to detect the mesenchymal stroma. Results Seven patients were women and 1 a boy. Most of the patients had epigastric upset accompanied by jaundice, diarrhea or menoschesis. Three patients were retreated. The inner lining showed obvious dysplasia and some mitotic activity in part of the area of cystadenoma in 1 patient. Mesenchymal stroma cells existed in 4 patients with cystadenoma who were all positive for CD117, Vim and SMA. Three patients were positive for CD34, ER and PR and 2 for S-100. All the patients were negative for Desmin. The patients treated by radical resection remained well with no evidence of recurrence, while recurrence appeared in those treated by incomplete excision with or without drainage. Conclusions Hepatobiliary cystic neoplasms show obvious female predominance. Epigastric upset is the most common manifestation. Preoperative diagnosis prove to be difficult. Complete excision is the best choice of treatment. Borderline pathological changes exist between cystadenoma and cystadenocarcinoma. The neoplasms might be originated from both endodermal and mesodermal cells. The o-varian-like stroma may originate from primary stromal cells and show immunophenotype evidence of smooth muscle and nervous differentiatioa Their growth may depend on the adjustment of nerve and hormone.