van Schaik P-M, Ernst M-F, Meijer H-A, Bosscha K
Jeroen Bosch Hospital, Department of Surgery, 's-Hertogenbosch 5211, The Netherlands.
World J Gastroenterol. 2008 Mar 14;14(10):1633-5. doi: 10.3748/wjg.14.1633.
A 41-year-old man presented with a 6-mo history of changed defecation and rectal bleeding. A 3-cm polypoid tumor of the lower rectum was found at rectosigmoidoscopy, which proved to be a leiomyosarcoma upon biopsy. Dissemination studies did not show any metastases. He was underwent to an abdomino-perineal resection (APR). Histopathology of the specimen showed a melanoma (S-100 stain positive). Two years after the resection, metastases in the abdomen and right lung were found. He died one and half years later. Primary anorectal melanoma is a rare and very aggressive disorder. According to current data, one should always perform a S-100 stain when anorectal sarcoma is suspected. A positive S-100 stain suggests the tumour to be most likely a melanoma. Subsequently, thorough dissemination studies need to be performed. Depending on the outcome of the dissemination studies, a surgical resection has to be performed. Nowadays, a sphincter-saving local excision combined with adjuvant loco-regional radiotherapy should be preferred in case of small tumors. The same loco-regional control is achieved with less "loss of function" compared to non-sphincter saving surgery. Only in the case of large and obstructing tumors an abdomino-perineal resection is the treatment of choice.
一名41岁男性,有6个月排便习惯改变及直肠出血病史。直肠乙状结肠镜检查发现距肛门3cm处有一3cm息肉样肿瘤,活检证实为平滑肌肉瘤。分期检查未发现转移。患者接受了腹会阴联合切除术(APR)。标本的组织病理学检查显示为黑色素瘤(S-100染色阳性)。切除术后两年,发现腹部及右肺转移。患者于一年半后死亡。原发性肛管直肠黑色素瘤是一种罕见且侵袭性很强的疾病。根据目前的数据,当怀疑肛管直肠肉瘤时,应常规进行S-100染色。S-100染色阳性提示肿瘤很可能是黑色素瘤。随后,需要进行全面的分期检查。根据分期检查结果,决定是否行手术切除。目前,对于小肿瘤,应首选保留括约肌的局部切除联合辅助性局部区域放疗。与不保留括约肌的手术相比,这种方法在实现相同局部区域控制的同时,“功能丧失”更少。只有在肿瘤较大且阻塞时,腹会阴联合切除术才是首选治疗方法。