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肛管直肠无色素性黑色素瘤。

Anorectal amelanotic melanoma.

作者信息

Hillenbrand A, Barth T F E, Henne-Bruns D, Formentini A

机构信息

Department of General, Visceral, and Transplantation Surgery, University of Ulm, Ulm, Germany.

出版信息

Colorectal Dis. 2008 Jul;10(6):612-5. doi: 10.1111/j.1463-1318.2007.01400.x. Epub 2007 Oct 18.

Abstract

OBJECTIVE

Anorectal melanoma is a rare, highly malignant tumour with a poor 5 year survival of 10%. Most anorectal melanomas have gross and/or histologic pigmentation, however about 30% of anorectal melanomas are amelanotic.

METHOD

We report three cases of amelanotic anorectal melanomas and integrate our data with six case reports of amelanotic malignant melanoma from the literature. Further we compare clinicopathological data and clinical outcome with large series of anorectal melanomas (both, amelanotic and pigmentated).

RESULTS

There were seven females and two males, of median age 62 years (range: 45-75 years). Rectal bleeding was the leading symptom in all cases with a mean duration of 4 months before diagnosis. Eight of nine patients developed distant metastases. Median survival was 14 months (range: 3-60 months). A tumour thickness of < 4 mm was correlated with long-term disease-free survival, whereas tumour thickness of 4 mm or more was correlated with systemic recurrence.

CONCLUSION

Early diagnosis is key for efficient treatment and improved survival rate for patients with this unusual variant of melanoma. There is no difference in terms of age, time of diagnosis, stage and survival between pigmented and amelanotic anorectal melanoma.

摘要

目的

肛管直肠黑色素瘤是一种罕见的高恶性肿瘤,5年生存率低至10%。大多数肛管直肠黑色素瘤有肉眼可见和/或组织学色素沉着,然而约30%的肛管直肠黑色素瘤为无色素性。

方法

我们报告3例无色素性肛管直肠黑色素瘤病例,并将我们的数据与文献中6例无色素性恶性黑色素瘤病例报告相结合。此外,我们将临床病理数据和临床结果与大量肛管直肠黑色素瘤(包括无色素性和色素沉着性)系列进行比较。

结果

共9例,其中女性7例,男性2例,中位年龄62岁(范围:45 - 75岁)。所有病例的主要症状均为直肠出血,诊断前平均持续时间为4个月。9例患者中有8例发生远处转移。中位生存期为14个月(范围:3 - 60个月)。肿瘤厚度<4 mm与长期无病生存相关,而肿瘤厚度≥4 mm与全身复发相关。

结论

早期诊断是有效治疗并提高这种罕见黑色素瘤患者生存率的关键。色素沉着性和无色素性肛管直肠黑色素瘤在年龄、诊断时间、分期和生存方面无差异。

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