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...

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Detalles Bibliográficos
Autores principales: O'Neill, J. K., Stone, C. A., Sarsfield, P., Smith, M., Smithson, S. F., Silver, D., Devaraj, V. S.
Formato: Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2005
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2395631/
https://www.ncbi.nlm.nih.gov/pubmed/18521424
http://dx.doi.org/10.1080/13577140500229596
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author O'Neill, J. K.
Stone, C. A.
Sarsfield, P.
Smith, M.
Smithson, S. F.
Silver, D.
Devaraj, V. S.
author_facet O'Neill, J. K.
Stone, C. A.
Sarsfield, P.
Smith, M.
Smithson, S. F.
Silver, D.
Devaraj, V. S.
author_sort O'Neill, J. K.
collection PubMed
description 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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spelling pubmed-23956312008-06-02 An Association of Multiple Well Differentiated Liposarcomas, Lipomatous Tissue and Hereditary Retinoblastoma O'Neill, J. K. Stone, C. A. Sarsfield, P. Smith, M. Smithson, S. F. Silver, D. Devaraj, V. S. Sarcoma Research Article 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. Hindawi Publishing Corporation 2005 /pmc/articles/PMC2395631/ /pubmed/18521424 http://dx.doi.org/10.1080/13577140500229596 Text en Copyright © 2005 Hindawi Publishing Corporation. http://creativecommons.org/licenses/by/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Research Article
O'Neill, J. K.
Stone, C. A.
Sarsfield, P.
Smith, M.
Smithson, S. F.
Silver, D.
Devaraj, V. S.
An Association of Multiple Well Differentiated Liposarcomas, Lipomatous Tissue and Hereditary Retinoblastoma
title An Association of Multiple Well Differentiated Liposarcomas, Lipomatous Tissue and Hereditary Retinoblastoma
title_full An Association of Multiple Well Differentiated Liposarcomas, Lipomatous Tissue and Hereditary Retinoblastoma
title_fullStr An Association of Multiple Well Differentiated Liposarcomas, Lipomatous Tissue and Hereditary Retinoblastoma
title_full_unstemmed An Association of Multiple Well Differentiated Liposarcomas, Lipomatous Tissue and Hereditary Retinoblastoma
title_short An Association of Multiple Well Differentiated Liposarcomas, Lipomatous Tissue and Hereditary Retinoblastoma
title_sort association of multiple well differentiated liposarcomas, lipomatous tissue and hereditary retinoblastoma
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2395631/
https://www.ncbi.nlm.nih.gov/pubmed/18521424
http://dx.doi.org/10.1080/13577140500229596
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