Wanebo H J, Woodruff J M, Farr G H, Quan S H
Cancer. 1981 Apr 1;47(7):1891-900. doi: 10.1002/1097-0142(19810401)47:7<1891::aid-cncr2820470730>3.0.co;2-k.
Primary malignant melanoma of the anorectum is a rare and virulent malignancy associated with an extremely poor prognosis in spite of aggressive initial therapy. Fifty-one patients with this disease were treated at Memorial Sloan-Kettering Cancer Center during the last 50 years and only six (12%) survived five years. This report views in detail 36 of the patients treated since 1950. In this group there was a female predominance (21 females, 15 males), and median age was in the 6th decade (range 27-75). Common presenting symptoms were pain, bleeding, mass or "hemorrhoids" of 1-12 months duration. In two-thirds, of the cases, a radical surgical approach was attempted. Other therapy included local excision alone or combined with groin dissection, local excision followed by delayed rectal resection and local tumor destruction by cryosurgery or fulguration. Mean survival was 21.5 months. Three patients had palliative treatment only with radiation therapy. Histopathologic study of 30 lesions showed that two-thirds were bulky or polypoid lesions and two-thirds showed junctional changes. The virulent prognosis of primary anorectal melanoma appears directly related to tumor size and thickness. Although all four of the five-year survivors had radical surgery, the three whose tumors could be measured had superficial lesions. In general, curative efforts by radical surgery were no more effective than local treatment by excision or cryosurgery. One patient, however, did have a thin lesion but also had nodal metastases and survived over five years after radical surgery. Although these data suggest that radical resection may cure patients with lesions thinner than 3 mm, it does not exclude the possibility that local excision might also be curative. For larger lesions that have not been cured by radical surgery, more conservative local approaches by excision or cryotherapy might be the optimum way of achieving local palliation.
原发性直肠肛管恶性黑色素瘤是一种罕见且恶性程度高的肿瘤,尽管初始治疗积极,但预后极差。在过去50年中,纪念斯隆 - 凯特琳癌症中心共治疗了51例该疾病患者,只有6例(12%)存活了5年。本报告详细回顾了自1950年以来接受治疗的36例患者。该组中女性占优势(21名女性,15名男性),中位年龄在60岁左右(范围27 - 75岁)。常见的首发症状为疼痛、出血、肿物或“痔疮”,持续时间为1 - 12个月。三分之二的病例尝试了根治性手术方法。其他治疗包括单纯局部切除或联合腹股沟淋巴结清扫、局部切除后延迟直肠切除以及通过冷冻手术或电灼进行局部肿瘤破坏。平均生存期为21.5个月。3例患者仅接受了放射治疗作为姑息治疗。对30个病变的组织病理学研究表明,三分之二为体积较大或息肉样病变,三分之二显示交界性改变。原发性直肠肛管黑色素瘤的恶性预后似乎与肿瘤大小和厚度直接相关。虽然5名5年幸存者均接受了根治性手术,但其中3名肿瘤可测量的患者病变较表浅。总体而言,根治性手术的治愈效果并不比局部切除或冷冻手术更有效。然而,有1例患者病变较薄但有淋巴结转移,根治性手术后存活超过了5年。虽然这些数据表明根治性切除可能治愈病变厚度小于3mm的患者,但并不排除局部切除也可能治愈的可能性。对于根治性手术未能治愈的较大病变,更保守的局部切除或冷冻治疗方法可能是实现局部姑息的最佳方式。