儿童横纹肌肉瘤的手术治疗

横纹肌肉瘤是18岁以下儿童和青少年最常见的软组织恶性肿瘤, 约占全部儿童恶性肿瘤的4%, 软组织肉瘤的50%, 5年生存率已经从10%以下提高到70%左右。化疗、手术、放疗的综合治疗模式已经确立。最终获得广泛切除即镜下无肿瘤和无肉眼残留是外科治疗的最终目的, 由于间室切除术及截肢术对儿童病例的生存率无明显提高, 但致残率较高, 明显降低患儿的生存质量, 因此目前广泛切除是主要的治疗方式。对于儿童来说, 功能的保留似乎更加重要, 因此近年来由于化疗水平的提高, 手术趋于保守。本文回顾了原发于头颈部、躯干、四肢、泌尿生殖系统等不同部位肿瘤的手术方式。 

Surgical Treatment for Rhabdomyosarcoma in Children

Rhabdomyosarcoma accounts for approximately 4% of cancer cases among children aged 0 to 18 years, as well as among adolescents and young adults. Moreover, rhabdomyosarcoma accounts for approximately 50% of the cases of soft-tissue sarcomas. Children afflicted with this disease who received multimodality therapy experienced a 5-year event-free survival rate of approximately 70%. The multimodality therapy entails surgical resection, the success of which is feasible without major functional/cosmetic impairment, is followed by chemotherapy. Rhabdomyosarcoma arises from various sites; thus, surgical care must be tailored to the unique aspects of each site. The aim of this article is to review the surgical therapy of rhabdomyosarcoma occurring in different parts of the body. Cosmetic and functional factors are always considered. However, with modem techniques, complete resection in patients with superficial tumors need not be inconsistent with good cosmetic and functional results, especially in several sites. For head and neck tumors, cosmetic and functional factors are always considered, although narrower resection margins (< 1 mm) are acceptable because of anatomic restrictions. The definitive surgical procedure of the extremity sites involves wide local excision with en bloc removal of a cuff of normal tissue. Most patients who present with large tumors in truncal sites have a localized disease suitable for complete resection with negative margins after preoperative therapy. Lesions occurring adjacent to the testis or spermatic cord up to the internal inguinal ring should be removed by orchiectomy with resection of the entire spermatic cord. The removal process utilizes an inguinal incision with proximal vascular control. Bladder salvage is an important goal of therapy for patients with tumors arising in the prostate and bladder. After all, the basic principle of the initial surgical treatment of children with rhabdomyosarcoma is the complete resection of the primary tumor with a surrounding margin of normal tissue and lymph node sampling of the draining nodal basin, provided that major functional/cosmetic impairment is not necessary.