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肛门直肠恶性黑色素瘤——一种罕见的实体瘤。

Malignant melanoma of the anorectum--a rare entity.

机构信息

Department of General and Colorectal Surgery, Medical University of Lodz, Plac Hallera 1, 90-647, Lodz, Poland.

出版信息

Langenbecks Arch Surg. 2010 Aug;395(6):757-60. doi: 10.1007/s00423-009-0586-5. Epub 2010 Jan 12.

Abstract

OBJECTIVE

Primary anorectal melanoma is a rare entity with a poor prognosis accounting for approximately 0.1-4.6% of anal tumours and 0.5-1.6% of all melanomas. Almost 60% of patients have already disseminated disease at initial diagnosis.

METHOD

We report four cases of anorectal melanoma treated at our department from November 2006 to September 2008, as well as a review of the literature.

RESULTS

There were two females and two males, of median age 69 years (range: 59-81 years). Most frequent complaints were rectal bleeding and/or anorectal pain. Three of our four patients had amelanotic melanomas. We found the positive expression of S-100 protein and HMB-45 in two patients and melanin A cells in one case. Abdominoperineal resection was performed in two patients, colostomy in one patient and a wide local excision also in one case. Three patients died on account of dissemination of melanoma, one patient is still alive.

CONCLUSION

Anal melanoma remains a deadly problem. Clear guidelines for the therapy of anorectal melanoma have not been established. This mainly results from the rarity of this tumour. Treatment is based on retrospective studies, which report a limited number of cases.

摘要

目的

原发性肛门直肠黑色素瘤是一种罕见的疾病,预后较差,约占肛门肿瘤的 0.1-4.6%,占所有黑色素瘤的 0.5-1.6%。几乎 60%的患者在初始诊断时已经存在疾病扩散。

方法

我们报告了 2006 年 11 月至 2008 年 9 月在我们科室治疗的 4 例肛门直肠黑色素瘤病例,并对文献进行了回顾。

结果

患者中有 2 名女性和 2 名男性,中位年龄为 69 岁(范围:59-81 岁)。最常见的症状是直肠出血和/或肛门直肠疼痛。我们发现 4 例患者中有 3 例为无色素黑色素瘤。我们在 2 例患者中发现 S-100 蛋白和 HMB-45 阳性表达,在 1 例患者中发现黑色素 A 细胞阳性表达。2 例患者行腹会阴联合切除术,1 例行结肠造口术,1 例行广泛局部切除术。3 例患者因黑色素瘤扩散而死亡,1 例患者仍存活。

结论

肛门黑色素瘤仍然是一个致命的问题。肛门直肠黑色素瘤的治疗指南尚未明确制定。这主要是由于这种肿瘤的罕见性。治疗主要基于回顾性研究,这些研究报告的病例数量有限。

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