Danikas D, Theodorou S J, Singh R, Camal D E
Department of Surgery, Monmouth Medical Center, Long Branch, New Jersey USA.
Am Surg. 2001 Sep;67(9):873-4.
Primary sarcoma of the gallbladder is a rare disease. The tumor occurs more frequently in women. Usually gallstones are present. Symptoms resemble those of cholelithiasis or cholecystitis. The diagnosis is rarely made preoperatively. The patient was a 51-year-old woman with a 2-month history of right upper quadrant pain, nausea, vomiting, and a 10-pound weight loss. Ultrasound showed cholelithiasis and cholecystitis. Laparoscopic cholecystectomy was converted to open as a result of dense tissue in the middle to distal gallbladder. Exploration by a right subcostal incision revealed multiple implants on the surface of the liver and the peritoneum of the upper abdomen. The wall of the gallbladder was very thick and inflamed. Cholecystectomy with liver biopsy was performed. Pathology revealed poorly differentiated epithelioid leiomyosarcoma of the gallbladder with extension to the liver. The disease followed a very aggressive course and the patient died 3 weeks after the procedure. Recommended treatment is extensive surgical resection that can be followed by radiotherapy or chemotherapy. The tumor follows a very aggressive course, which often lasts a few weeks. Prognosis is poor with rare reported 5-year survivals.
原发性胆囊肉瘤是一种罕见疾病。该肿瘤在女性中更为常见。通常存在胆结石。症状类似于胆石症或胆囊炎。术前很少能做出诊断。患者为一名51岁女性，有2个月右上腹疼痛、恶心、呕吐病史，体重减轻10磅。超声显示胆石症和胆囊炎。由于胆囊中至远端组织致密，腹腔镜胆囊切除术转为开腹手术。经右肋下切口探查发现肝脏表面和上腹部腹膜有多处种植灶。胆囊壁非常厚且发炎。进行了胆囊切除术并取肝脏活检。病理显示为胆囊低分化上皮样平滑肌肉瘤并累及肝脏。该疾病进展非常迅速，患者在手术后3周死亡。推荐的治疗方法是广泛手术切除，术后可进行放疗或化疗。肿瘤进展非常迅速，通常持续几周。预后很差，很少有报道5年生存率。
