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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. ORCID: https://orcid.org/0000-0003-1911-3382, Wilson, S., Orosz, Z., Gibbons, C. L. M. H., Oppermann, U. and Athanasou, N. A. 2018. Pleomorphic liposarcoma of bone: a rare primary malignant bone tumour. Clinical Sarcoma Research 8 (1) , 2. 10.1186/s13569-018-0089-7

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Abstract

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.

Item Type: Article
Date Type: Published Online
Status: Published
Schools: Medicine
Publisher: BioMed Central
ISSN: 2045-3329
Date of First Compliant Deposit: 1 June 2020
Date of Acceptance: 5 February 2018
Last Modified: 07 Nov 2022 10:21
URI: https://orca.cardiff.ac.uk/id/eprint/131993

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