Ranjith S, Muralee M, Sajeed A, Arun P M, Cherian K, Nair C K, Augustine P, Ahamed I
Division of Surgical Oncology, Regional Cancer Centre , Trivandrum , Kerala.
Division of Radiation Oncology, Regional Cancer Centre , Trivandrum , Kerala.
Ann R Coll Surg Engl. 2018 Mar;100(3):185-189. doi: 10.1308/rcsann.2017.0184. Epub 2017 Oct 19.
Introduction Mucosal malignant melanoma of the anorectum is a rare and aggressive disease, in which early diagnosis is difficult. The prognosis remains extremely poor, irrespective of the treatment. We share our experience in treating this malignancy at our centre in South India. Methods This study describes a retrospective analysis of 31 cases of anorectal melanoma presented to our centre between January 2001 and December 2013. Results Twenty-two patients (71%) presented with metastasis and had a median overall survival of nine months. None of the 22 patients survived for two years. Nine patients (29%) had curative surgery, in the form of abdominoperineal resection (six patients), abdominoperineal resection with bilateral inguinal node dissection (one patient), abdominoperineal resection with liver resection (one patient) and posterior exenteration (one patient). In patients who underwent curative surgery, the median overall survival was 15 months and disease-free survival was nine months, with a two-year overall survival of 22%. Conclusions Anorectal melanoma is an aggressive disease with a poor prognosis. The majority of patients present with distant metastases. Prognosis depends on stage at presentation. Early diagnosis and surgical resection may improve the overall outcome. Newer modalities such as immunotherapy and targeted therapies such as anti-CTLA4 monoclonal antibody and anti-programmed cell death protein 1 monoclonal antibodies have radically changed the management of mucosal melanoma and may, in the future, improve the overall prognosis of anorectal melanoma.
引言
肛管直肠黏膜恶性黑色素瘤是一种罕见且侵袭性强的疾病,早期诊断困难。无论采用何种治疗方法,其预后仍然极差。我们分享在印度南部中心治疗这种恶性肿瘤的经验。
方法
本研究对2001年1月至2013年12月期间在我们中心就诊的31例肛管直肠黑色素瘤患者进行回顾性分析。
结果
22例患者(71%)出现转移,中位总生存期为9个月。这22例患者中无一例存活超过两年。9例患者(29%)接受了根治性手术,包括腹会阴联合切除术(6例)、腹会阴联合切除术加双侧腹股沟淋巴结清扫术(1例)、腹会阴联合切除术加肝切除术(1例)和后盆腔脏器清除术(1例)。接受根治性手术的患者中位总生存期为15个月,无病生存期为9个月,两年总生存率为22%。
结论
肛管直肠黑色素瘤是一种侵袭性疾病,预后较差。大多数患者就诊时已出现远处转移。预后取决于就诊时的分期。早期诊断和手术切除可能改善总体预后。免疫治疗等新方法以及抗CTLA4单克隆抗体和抗程序性细胞死亡蛋白1单克隆抗体等靶向治疗已从根本上改变了黏膜黑色素瘤的治疗方式,未来可能改善肛管直肠黑色素瘤的总体预后。