Korenkov M, Gönner U, Dünschede F, Junginger T
Klinik und Poliklinik für Allgemein- und Abdominalchirurgie, Johannes-Gutenberg-Universität Mainz, Eschwege.
Zentralbl Chir. 2008 Dec;133(6):564-7. doi: 10.1055/s-2008-1076876. Epub 2008 Dec 17.
Rectal melanoma is a rare disease. There is much controversy concerning cause, incidence and treatment of the disease and the spreading of recurrence. In this article, we discuss actual aspects of diagnostic, therapy and prognosis on the basis of our series of seven patients as well as a literature review. The surgical therapy in the form of local tumour excision with a disease-free margin of up to 1-2 cm is the initial therapeutic modality of choice. Large tumours that obviously could not be removed in sano should be treated with a multimodal concept. Such tumours should be treated by a combination of neoadjuvant radiation and chemotherapy for down-staging with subsequent local excision (LE) or abdomino-perineal rectum extirpation (APR). An inguinal lymphadenectomy should only be performed if the lymph nodes are enlarged on clinical or radiological examination. The prognosis of rectal melanoma is markedly poor and is primarily related with the stage of disease. The 5-year survival rate is estimated at about 24% for patients with stage I tumours. Patients with stage II and III tumours have appreciably shorter survival times of 12 months on the average.
直肠黑色素瘤是一种罕见疾病。关于该疾病的病因、发病率、治疗以及复发扩散存在诸多争议。在本文中,我们基于我们的7例患者系列以及文献综述,讨论诊断、治疗和预后的实际情况。以局部肿瘤切除形式进行手术治疗,切缘无瘤可达1 - 2厘米,是首选的初始治疗方式。明显无法完整切除的大肿瘤应以多模式理念治疗。此类肿瘤应采用新辅助放疗和化疗联合以降期,随后进行局部切除(LE)或腹会阴直肠切除术(APR)。仅当临床或影像学检查发现淋巴结肿大时才应进行腹股沟淋巴结清扫术。直肠黑色素瘤的预后明显较差,主要与疾病分期相关。I期肿瘤患者的5年生存率估计约为24%。II期和III期肿瘤患者的平均生存时间明显较短,约为12个月。