Anorectal amelanotic melanoma
Anorectal amelanotic melanoma
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DOI：
10.1111/j.1463-1318.2007.01400.x
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发表时间：
2008-07-01
影响因子：
3.4
通讯作者：
Formentini, A.
中科院分区：
文献类型：
--
作者：
Hillenbrand, A.;Barth, T. F. E.;Formentini, A.
目的肛门直肠黑色素瘤是一种罕见的高度恶性肿瘤，5年生存率仅为10%。大多数肛门直肠黑色素瘤具有肉眼和/或组织学色素沉着，但约30%的肛门直肠黑色素瘤是无色素性的。方法我们报告了三例无色素性肛门直肠黑色素瘤，并将我们的数据与文献中六例无色素性恶性黑色素瘤的病例报告进行整合。此外，我们比较了临床病理数据和临床结果与大系列的肛门直肠黑色素瘤（无黑色素和色素沉着）。结果有7名女性和2名男性，中位年龄62岁（范围：45-75岁）。直肠出血是所有病例的主要症状，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与长期无病生存率相关，而肿瘤厚度4 mm或以上与全身复发相关。结论早期诊断是有效治疗和提高生存率的关键，这种不寻常的黑色素瘤的患者。色素性和无色素性肛门直肠黑色素瘤在年龄、诊断时间、分期和生存率方面没有差异。
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.