An Association of Multiple Well Differentiated Liposarcomas, Lipomatous Tissue and Hereditary Retinoblastoma
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 fol...
| Main Authors: | , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Hindawi Limited
2005-01-01
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| Series: | Sarcoma |
| Online Access: | http://dx.doi.org/10.1080/13577140500229596 |
| Summary: | 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. |
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| ISSN: | 1357-714X 1369-1643 |