22例肛管直肠恶性黑色素瘤的临床分析并文献复习

背景与目的：肛管直肠恶性黑色素瘤恶性度高,其治疗方法尚存在争议．本研究旨在总结肛管直肠恶性黑色素瘤的诊断及治疗经验。方法：回顾性分析1977年至2003年江苏省肿瘤医院收治的22例肛管直肠恶性黑色素瘤患者的临床及病理资料．包括临床表现、诊断、手术方式和预后,并复习相关文献。结果：本组22例．占同期收治的大肠恶性肿瘤的0．4％（22／5205）,男性6例,女性16例．中位年龄61岁。主要临床表现为：便血86％（19／22）、肛门疼痛或不适59％（13／22）、肛门口肿物27％（6／22）等。首次就诊误诊率86％（19／22）,术前病理诊断确诊率48％（10／21）。无远处转移16例,伴远处转移6例。行腹会阴联合切除术11例．局部广泛切除术5例（2例复发后补充腹会阴联合切除术）,Park’s术2例．Hartmann’s术1例,乙状结肠双腔造瘘术3例。术后1、3、5年生存率分别为45．4％、18．1％、9．1％,中位生存时间12个月（95％CI：6～18个月）。结论：肛管直肠恶性黑色素瘤少见,易误诊,预后差。腹会阴联合切除术可作为无远处转移的肛管直肠恶性黑色素瘤患者的首选术式。

Diagnosis and treatment of anorectal malignant melanoma--a report of 22 cases with literature review

BACKGROUND & OBJECTIVE: Anorectal malignant melanoma (AMM) is an aggressive malignant tumor, and its treatment still remains controversial. This study was to summarize our experience on diagnosis and treatment of AMM. METHODS: Clinicopathologic records, including clinical feature, diagnosis, operation patterns, and prognosis, of 22 patients with AMM, treated in Jiangsu Provincial Cancer Hospital from 1977 to 2003, were analyzed retrospectively with literature review. RESULTS: The 22 patients with AMM accounted for 0.04% of all the patients diagnosed as malignant tumors of large bowel simultaneously in our hospital. Of the 22 patients, 6 were men, and 16 were women, with the median age of 61 (ranged 37-72). The most common complaints of AMM patients were hematochezia (86%), anus pain or discomfort (59%), local mass (27%), and so on. The misdiagnosis rate was 86%. The definite pathologic diagnosis rate before surgery was 48%. Of the 22 patients, 6 had distant metastasis; 11 underwent abdominoperineal resection, 5 underwent wide local excision (2 underwent salvage abdominoperineal resection who suffered from local recurrence after wide local excision), 2 underwent Park's procedure, 1 underwent Hartmann's procedure, and 3 underwent sigmoid colostomy. The 1-, 3-, and 5-year survival rates of the 22 patients were 45.4%, 18.1%, and 9.1%, respectively. The median survival time was 12 months (95% confidence interval: 6-18 months). CONCLUSIONS: AMM is a rare disease with poor prognosis. It tends to be misdiagnosed. Abdominoperineal resection may be considered as the first choice for the AMM patients without distant metastasis.