Pollono D G, Tomarchio S, Berghoff R, Drut R, Urrutia A, Cédola J
Oncology Unit, Hospital de Niños Superiora Sor María Ludovica, La Plata, Argentina.
Med Pediatr Oncol. 1998 May;30(5):290-3. doi: 10.1002/(sici)1096-911x(199805)30:5<290::aid-mpo5>3.0.co;2-a.
The very low frequency of rhabdomyosarcoma (RMS) of the extrahepatic biliary tree has impeded the development of a standardized form of treatment.
Based on the good response of embryonal RMS to chemotherapy, we used a multi-drug protocol as the initial treatment of a 3-year-old girl after obtaining adequate transparietohepatic biliary drainage. The treatment achieved complete remission. Later conservative laparoscopic surgery revealed only residual scar tissue. The patient is alive and well 1 year after remission.
Management of this difficult tumor using modern less invasive techniques for diagnosis and treatment, with the help of preoperative chemotherapy, prevented extensive damage to the biliary tree and allowed complete recovery.
肝外胆管横纹肌肉瘤（RMS）的极低发病率阻碍了标准化治疗方案的制定。
鉴于胚胎型RMS对化疗反应良好，我们在对一名3岁女童进行充分的经皮肝穿刺胆管引流后，采用多药联合方案作为初始治疗。治疗取得了完全缓解。随后的保守性腹腔镜手术仅发现残留的瘢痕组织。缓解后1年，患者存活且状况良好。
借助术前化疗，采用现代微创技术进行诊断和治疗这种难治性肿瘤，避免了对胆管树的广泛损伤，并实现了完全康复。
