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Macrodystrophia Lipomatosa: Radiologic-Pathologic Correlation

机译:大型营养不良性脂肪瘤:放射病理相关性

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Macrodystrophia lipomatosa (MDL) is a rare cause of congenital macrodactyly, characterised by progressive proliferation of all mesenchymal elements, with disproportionate increase in fibro-adipose tissue. It occurs most frequently in lower limbs along the distribution of the medial plantar nerve. MDL presents as localised gigantism of the hand or foot and comes to clinical attention for cosmetic reasons, mechanical problems secondary to degenerative joint disease, or development of neurovascular compression. Here, we report a case of MDL, with altered soft tissue growth due to an earlier surgery, making clinical diagnosis difficult. However, with a complete radio-clinical work-up and review of the history, a provisional diagnosis of MDL was made, which was confirmed by histopathology and during surgery.Keywords: Fat, local gigantism, macrodystrophia lipomatosa, macrodactyly
机译:脂溢性脂肪营养不良(MDL)是先天性巨指的罕见病因,其特征在于所有间充质成分的逐步增殖,纤维脂肪组织的增加不成比例。它最常发生在沿内侧plant神经分布的下肢中。 MDL表现为手或脚的局部巨人症,并出于美容原因,退行性关节疾病继发的机械问题或神经血管压迫的发展而引起临床关注。在这里,我们报告一例MDL,由于早期手术导致软组织生长改变,从而使临床诊断变得困难。然而,经过全面的放射线临床检查和病史回顾,对MDL进行了初步诊断,这在组织病理学和手术过程中得到了证实。关键词:脂肪,局部巨人症,脂溢性大肌营养不良,巨乳

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