胆囊肉瘤样癌的诊断与治疗

Diagnosis and treatment of sarcomatoid carcinoma of the gallbladder

胆囊肉瘤样癌是一种具有上皮性癌与肉瘤样癌双相成分的少见恶性肿瘤,其生长速度快,易发生转移,手术切除率低,复发率高,预后与大多数胆囊腺癌类似或更差。由于目前文献多为散在个案报道,对其发病机制、诊断治疗及预后评估诸方面的了解尚缺少系统性认识。其临床表现缺乏特征性,影像学检查及肿瘤标志物检测无特异性,该病确诊主要在手术后依靠病理组织学与免疫组织化学染色表达结果;对化学治疗、放射治疗等均不敏感,及时彻底的手术切除是目前首选的治疗方法。探讨其病理特性,寻找有效治疗方案,改善预后,是临床面临的重要研究课题。

Sarcomatoid carcinoma of the gallbladder is a rare variant of undifferentiated carcinoma composed of both malignant epithelial and sarcomatous components. A worse or similar prognosis to the generally dismal outcome seen in most gallbladder cancer patients could be associated with the rapid growth, frequent metastasis, low resectability rate and high incidence of recurrence even after curative resection. There is a paucity of information available regarding its pathogenesis, optimal treatment and prognostic determinants of this rare cancer due to the small number of individual case reports in most institutions. Patients with sareomatoid carcinoma of the gallbladder often present with nonsepecific clinical features. It is not associated with specific imaging findings or tumor markers. The final diagnosis is only confirmed by thorough histopathological and immunohischemical staining of the resected specimen. It is not sensitive to chemotherapy or radiotherapy, the most important treatment is surgical intervention. Further studies are needed to better understand the characteristics of this deadly tumor may offer improved prognosis and survival, and to establish an effective therapeutic strategy.