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Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour
BACKGROUND: Liposarcoma is an extremely rare primary bone sarcoma. CASE PRESENTATION: We report a case of primary pleomorphic liposarcoma that arose in an 18 year old male in the metaphysis of the left tibia. Plain radiographs showed a partly sclerotic lesion and MR imaging a heterogeneous tumour pr...
Autores principales: | , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5807841/ https://www.ncbi.nlm.nih.gov/pubmed/29449935 http://dx.doi.org/10.1186/s13569-018-0089-7 |
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author | Tiemeier, G. L. Brown, J. M. Pratap, S. E. McCarthy, C. Kastrenopoulou, A. Bradley, K. Wilson, S. Orosz, Z. Gibbons, C. L. M. H. Oppermann, U. Athanasou, N. A. |
author_facet | Tiemeier, G. L. Brown, J. M. Pratap, S. E. McCarthy, C. Kastrenopoulou, A. Bradley, K. Wilson, S. Orosz, Z. Gibbons, C. L. M. H. Oppermann, U. Athanasou, N. A. |
author_sort | Tiemeier, G. L. |
collection | PubMed |
description | BACKGROUND: Liposarcoma is an extremely rare primary bone sarcoma. CASE PRESENTATION: We report a case of primary pleomorphic liposarcoma that arose in an 18 year old male in the metaphysis of the left tibia. Plain radiographs showed a partly sclerotic lesion and MR imaging a heterogeneous tumour predominantly isointense on T1- and high-signal on T2-weighted sequences with focal areas of increased T1 signal that suppressed with fat saturation. PET/CT showed marked FDG uptake (SUV = 17.1) in the primary tumour as well as a metastasis in the right distal femur and multiple small pulmonary metastases. Histologically, the tumour was a pleomorphic liposarcoma containing large tumour cells with vacuolated cytoplasm and hyperchromatic pleomorphic nuclei as well as numerous lipoblasts and scattered brown fat-like cells. Tumour cells strongly expressed FABP4/aP2, a marker of adipocyte differentiation, and UCP1, a marker of brown fat, but not S100. The case was treated with neoadjuvant MAP chemotherapy, resulting in extensive (> 95%) necrosis in the primary tumour and almost complete resolution of the femoral and pulmonary metastases. CONCLUSIONS: Pleomorphic liposarcoma can present as a sclerotic primary malignant bone tumour; markers of adipose differentiation are useful in histological diagnosis and neoadjuvant MAP chemotherapy results in significant tumor necrosis. |
format | Online Article Text |
id | pubmed-5807841 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-58078412018-02-15 Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour Tiemeier, G. L. Brown, J. M. Pratap, S. E. McCarthy, C. Kastrenopoulou, A. Bradley, K. Wilson, S. Orosz, Z. Gibbons, C. L. M. H. Oppermann, U. Athanasou, N. A. Clin Sarcoma Res Case Report BACKGROUND: Liposarcoma is an extremely rare primary bone sarcoma. CASE PRESENTATION: We report a case of primary pleomorphic liposarcoma that arose in an 18 year old male in the metaphysis of the left tibia. Plain radiographs showed a partly sclerotic lesion and MR imaging a heterogeneous tumour predominantly isointense on T1- and high-signal on T2-weighted sequences with focal areas of increased T1 signal that suppressed with fat saturation. PET/CT showed marked FDG uptake (SUV = 17.1) in the primary tumour as well as a metastasis in the right distal femur and multiple small pulmonary metastases. Histologically, the tumour was a pleomorphic liposarcoma containing large tumour cells with vacuolated cytoplasm and hyperchromatic pleomorphic nuclei as well as numerous lipoblasts and scattered brown fat-like cells. Tumour cells strongly expressed FABP4/aP2, a marker of adipocyte differentiation, and UCP1, a marker of brown fat, but not S100. The case was treated with neoadjuvant MAP chemotherapy, resulting in extensive (> 95%) necrosis in the primary tumour and almost complete resolution of the femoral and pulmonary metastases. CONCLUSIONS: Pleomorphic liposarcoma can present as a sclerotic primary malignant bone tumour; markers of adipose differentiation are useful in histological diagnosis and neoadjuvant MAP chemotherapy results in significant tumor necrosis. BioMed Central 2018-02-09 /pmc/articles/PMC5807841/ /pubmed/29449935 http://dx.doi.org/10.1186/s13569-018-0089-7 Text en © The Author(s) 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Case Report Tiemeier, G. L. Brown, J. M. Pratap, S. E. McCarthy, C. Kastrenopoulou, A. Bradley, K. Wilson, S. Orosz, Z. Gibbons, C. L. M. H. Oppermann, U. Athanasou, N. A. Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour |
title | Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour |
title_full | Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour |
title_fullStr | Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour |
title_full_unstemmed | Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour |
title_short | Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour |
title_sort | pleomorphic liposarcoma of bone: a rare primary malignant bone tumour |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5807841/ https://www.ncbi.nlm.nih.gov/pubmed/29449935 http://dx.doi.org/10.1186/s13569-018-0089-7 |
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