Abstract
Well differentiated liposarcoma (atypical lipomatous tumour) is a low grade tumour, with no metastatic potential unless dedifferentiation supervenes. When superficial, it recurs locally only occasionally after marginal excision. We present a patient in whom bilateral childhood retinoblastoma was followed by later development of massive confluent areas of low grade liposarcoma and lipomatous tissue affecting the upper extremities and trunk. We discuss the role of mutations in the retinoblastoma gene (RB1) in linking these conditions and demonstrate the surgical management of an extremely unusual and challenging case.
Highlights
Well differentiated liposarcoma is a low grade tumour, with no metastatic potential unless dedifferentiation supervenes
A spectrum is seen within the tumours: areas typical of well differentiated liposarcoma merge imperceptibly towards the periphery with histologically normal adipose tissue
Some further debulking to non-specific areas of lipodystrophy is currently being planned at the patient’s request to aid mobility and cosmesis. This is a case of a man with bilateral retinoblastomas, multiple liposarcomas
Summary
An association of multiple well differentiated liposarcomas, lipomatous tissue and hereditary retinoblastoma.
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