An Association of Multiple Well Differentiated Liposarcomas, Lipomatous Tissue and Hereditary Retinoblastoma

[摘要] Well differentiated liposarcoma (atypical lipomatous tumour) is a low grade tumour, with no metastatic potential unlessdedifferentiation supervenes. When superficial, it recurs locally only occasionally after marginal excision. We present apatient in whom bilateral childhood retinoblastoma was followed by later development of massive confluent areas of lowgrade liposarcoma and lipomatous tissue affecting the upper extremities and trunk. We discuss the role of mutations in theretinoblastoma gene (RB1) in linking these conditions and demonstrate the surgical management of an extremely unusualand challenging case.

[学科分类] 肿瘤学