Misago N, Kohda H
Department of Internal Medicine, Saga Medical School, Japan.
J Dermatol. 1996 Oct;23(10):689-96. doi: 10.1111/j.1346-8138.1996.tb02682.x.
A family with atypical mole syndrome which manifested as polypoid melanoma in one member is reported. A 46-year-old man presented with a polypoid tumor on his lower back, which was excised under clinical diagnosis of soft fibroma. The tumor proved to be polypoid melanoma. He was referred to our clinic, and examination revealed that he had the atypical mole syndrome phenotype. The clinicopathological screening of his first-degree relatives confirmed that his 58-year-old sister had the same phenotype. In addition to our discussion of the clinical significance of polypoid melanoma, we also attempted to elucidate the characteristics of the reported Japanese cases of melanomas with atypical moles from a survey of the literature, which revealed the following characteristics: 1) younger mean age than that of total Japanese melanoma cases, 2) sun sensitive skin, 3) unexpectedly and frequently observed familial incidence (Kraemer's D1 and D2), 4) frequent site of the melanoma on the trunk and none on the sole of the foot, and 5) high incidence of superficial spreading melanoma and nodular melanoma, and 6) no cases of acral lentiginous melanoma.
报告了一个非典型痣综合征家族，其中一名成员表现为息肉样黑色素瘤。一名46岁男性下背部出现息肉样肿瘤，在临床诊断为软纤维瘤的情况下将其切除。肿瘤病理证实为息肉样黑色素瘤。他被转诊至我们的诊所，检查发现他具有非典型痣综合征表型。对其一级亲属进行的临床病理筛查证实，他58岁的姐姐具有相同表型。除了讨论息肉样黑色素瘤的临床意义外，我们还试图通过文献调查阐明已报道的日本非典型痣黑色素瘤病例的特征，结果发现以下特征：1）平均年龄比日本黑色素瘤总病例年轻；2）皮肤对阳光敏感；3）意外且频繁地观察到家族发病率（克雷默D1和D2）；4）黑色素瘤常见于躯干，足部未见；5）浅表扩散性黑色素瘤和结节性黑色素瘤发病率高；6）无肢端雀斑样痣黑色素瘤病例。
