儿童横纹肌肉瘤23例临床分析

Childhood rhabdomyosarcoma: a retrospective review of 23 cases

摘要:

目的：探讨儿童横纹肌肉瘤的临床特点、治疗和转归。方法：对1998年1月至2008年10月收治的23例横纹肌肉瘤患儿临床资料进行回顾性分析。结果：23例患儿中，男15例，女8例，平均发病年龄5岁(7个月至12岁)。依据美国横纹肌肉瘤研究组(IRS)的分期标准，I期2例，Ⅱ期4例，Ⅲ期8例，Ⅳ期9例。原发于头颈部14例，四肢4例，膀胱2例，肾脏、腹膜后及胆道各1例。所有患儿均经病理活检及免疫组织化学染色确诊。临床表现无特异性,主要为肿瘤组织占位、压迫、浸润后引起。治疗严格依照患儿IRS分期进行。2002年前化疗方案以VDCA、VAC 和VadrC 为主，2002年后采用美国肿瘤学中心研究组（COG）横纹肌肉瘤化疗方案。其中19例接受手术、化疗和放疗综合治疗的患儿2年生存率为63%，4例接受单纯手术或手术结合单一化疗或放疗的患儿生存期均未超过2年。结论：儿童横纹肌肉瘤临床表现无特异性；联合手术、放疗、化疗是治疗横纹肌肉瘤的有效方法。

Abstract:

OBJECTIVE: To study the clinical characteristics, treatment and outcome of childhood rhabdomyosarcoma. METHODS: The clinical data of 23 children with rhabdomyosarcoma from January, 1998 to October, 2008 were retrospectively reviewed. RESULTS: Of the 23 cases, 15 were male and 8 were female, with a mean age of 5 years old (7 months to 12 years old). Based on the American IRS staging system, 2 cases were in stage I, 4 cases in stage II, 8 cases in stage III, and 9 were in stage IV. The primary sites were found in head and neck (14 cases), extremities (4 cases), bladder (2 cases), kidney (1 case), postperitoneum (1 case) and bile duct (1 case). All of the children were confirmed with rhabdomyosarcoma by biopsy and immunohistochemistry. The clinical manifestations were related to the tumor tissues-induced space occupying, compression and erosion and were aspecific. The patients in different IRS stages were given different treatment regimens. The chemotherapy regimens VDCA, VAC or VadrC were used before 2002. After 2002, the Children′s Oncology Group (COG) protocol was employed. The two-year survival rate was 63% in 19 patients who received a combination of surgery, chemotherapy and radiotherapy, but none of 4 patients who received a surgery alone or a combination of surgery and chemotherapy or radiotherapy survived more than two years. CONCLUSIONS: The clinical manifestations of childhood rhabdomyosarcoma are not specific. A combination therapy including surgery, chemotherapy and radiation is effective to the improvement of the survival rate in children with rhabdomyosarcoma.