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来自单一中心的15年随访结果:孤立性半侧增生病例的肿瘤发生情况及风险

Results of fifteen-year follow-up from a single center: findings and risks for tumor development in isolated hemihyperplasia cases.

作者信息

Atik T, Cogulu O, Ozkinay F

出版信息

Genet Couns. 2014;25(4):417-21.

Abstract

Isolated hemihyperplasia is abnormal asymmetric growth of one or more parts of the body without any underlying disease. The risk for the development of embryonal tumor is increased in the subjects with isolated hemihyperplasia. The study presented here retrospectively evaluated the clinical data and the risk for tumor development in the cases with isolated hemihyperplasia. 24 cases with isolated hemihyperplasia were retrospectively evaluated. An extremity segment has been involved in 16.7%, an extremity has been entirely involved in 37.5%, more than one extremity have been involved on the same side in 16.7%, and definitely half of the body including the face has been involved in 25% of the patients, whereas one side of the face has been involved in only one case. Wilms tumor in the left abdomen (4.2%) was developed in one case. Isolated hemihyperplasia is a rare clinical picture that enhances the risk for the development of embryonal tumors.

摘要

孤立性半身肥大是指身体一个或多个部位出现异常的不对称生长,且无任何潜在疾病。孤立性半身肥大患者发生胚胎性肿瘤的风险增加。本文所呈现的研究对孤立性半身肥大病例的临床资料及肿瘤发生风险进行了回顾性评估。对24例孤立性半身肥大病例进行了回顾性评估。16.7%的病例累及一个肢体节段,37.5%的病例整个肢体受累,16.7%的病例同一侧多个肢体受累,25%的患者明确累及包括面部在内的身体一侧,而仅1例患者面部一侧受累。1例患者左侧腹部发生肾母细胞瘤(4.2%)。孤立性半身肥大是一种罕见的临床表现,会增加胚胎性肿瘤发生的风险。

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