目的探讨胃肠道恶性黑色素瘤的流行病学资料、病因、临床病理特征及治疗。方法结合我院2例小肠恶性黑色素瘤病例,复习相关文献并作分析报道。结果 2例患者均为男性,病程均在1年以内,病理均诊断为恶性黑色素瘤伴肠系膜淋巴结转移,手术治疗后均进行术后化疗,1例患者术后24个月无复发。胃肠道恶性黑色素瘤多为转移性,原发性胃肠道恶性黑色素瘤少见,胃肠道转移性黑色素瘤可以发生于原发病灶切除数年之后,约4%-12%没有明确的原发病灶。最常见的临床症状为间断腹痛,贫血,体重减轻和腹部肿块。外科手术是首要的治疗手段。影响手术效果的主要因素是患者的选择和能否进行根治性切除。早期诊断和进行彻底切除可以改善患者预后。患者年龄、肿瘤位置、大小、原发部位、能否进行根治性切除以及从原发病灶切除至胃肠道转移的间隔时间是独立的预后因素。靶向药物联合治疗和免疫靶向药物是未来黑色素瘤研究的新方向。结论胃肠道恶性黑色素瘤预后差,早期诊断困难,转移早,对有黑色素瘤病史的患者出现胃肠道症状应进行全面检查,对于孤立的病变应积极行根治性手术,术后应积极进行辅助药物治疗。
Objective To analyze the character of epidemiology, pathogenesis, clinical manifestation, pathology and therapy of gastrointestinal malignant melanoma(GIMM). Methods Two cases of GIMM in Peking University People's Hospital were reported and literatures were reviewed. Results Both two male patients were diagnosed with malignant melanoma pathologically with a course within 1 year. Both had chemotherapy after surgery. No recurrence of 24 months occurred in one patient. Metastatic GIMM was common while primary GIMM was rare. Metastatic GIMM could occur several years after the primary site was removed. About 4% to 12% metastatic GIMM had no definite primary cancer. The most common clinical features were intermittent abdominal pain, anemia, weight loss and abdomen mass. Surgery was the most important treatment. The main influence factors of surgery were to choose the appropriate patients and to undergo radical resection. Early diagnosis and radical resection could improve the prognosis of patients. Age, tumor location and size, the location of primary lesion and whether to have radical resection or not were independent prognostic factors, as well as the interval from removing the primary cancer to metastatic GIMM. Combing target drugs and immune targeting drugs were new directions of researches of melanoma in the future. Conclusion GIMM has poor prognosis and early distant metastasis. It is difficult to diagnose early, so it is necessary to have an overall examination for melanoma patients with gastrointestinal symptoms. Patients of isolate lesion should undergo active radical resection and auxiliary drug treatment after surgery.