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Primary Leiomyosarcoma of Bone: Analysis of Prognosis

Leiomyosarcoma of bone is just one of the variants of spindle cell sarcoma of bone characterised by the expression of desmin and other markers indicating a significant element of smooth muscle in the tumour, without osteoid production we have investigated the management and outcome of this rare type...

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Autores principales: Brewer, P., Sumathi, V., Grimer, R. J., Carter, S. R., Tillman, R. M., Abudu, A., Jeys, L.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3329678/
https://www.ncbi.nlm.nih.gov/pubmed/22550421
http://dx.doi.org/10.1155/2012/636849
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author Brewer, P.
Sumathi, V.
Grimer, R. J.
Carter, S. R.
Tillman, R. M.
Abudu, A.
Jeys, L.
author_facet Brewer, P.
Sumathi, V.
Grimer, R. J.
Carter, S. R.
Tillman, R. M.
Abudu, A.
Jeys, L.
author_sort Brewer, P.
collection PubMed
description Leiomyosarcoma of bone is just one of the variants of spindle cell sarcoma of bone characterised by the expression of desmin and other markers indicating a significant element of smooth muscle in the tumour, without osteoid production we have investigated the management and outcome of this rare type of primary malignant bone tumour. Method. Retrospective review of data stored on a prospective database. Results. In a database of 3364 patients with primary malignant bone sarcomas, 31 patients were identified with a primary leiomyosarcoma of bone. There were 12 males and 19 females with a mean age of 46 and tumour size of 8 cm. The most common site was the distal femur followed by the proximal tibia. Treatment was with chemotherapy and surgical resection. Seven of the patients had metastases at diagnosis. Surgery was carried out in 28 patients, 8 having amputation and 20 limb salvage. Three patients developed local recurrence, but half developed metastases. All patient disease-specific survival was 57% at five years and 44% at 10 yrs but for those without metastases was 82% and 60%, respectively. The only prognostic factors were metastases at diagnosis. Conclusion. Leiomyosarcoma of bone is a very rare primary malignant bone tumour affecting a predominantly older population. Despite the high incidence of metastases, survival is better than for other bone sarcomas for those without metastases at diagnosis.
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spelling pubmed-33296782012-05-01 Primary Leiomyosarcoma of Bone: Analysis of Prognosis Brewer, P. Sumathi, V. Grimer, R. J. Carter, S. R. Tillman, R. M. Abudu, A. Jeys, L. Sarcoma Research Article Leiomyosarcoma of bone is just one of the variants of spindle cell sarcoma of bone characterised by the expression of desmin and other markers indicating a significant element of smooth muscle in the tumour, without osteoid production we have investigated the management and outcome of this rare type of primary malignant bone tumour. Method. Retrospective review of data stored on a prospective database. Results. In a database of 3364 patients with primary malignant bone sarcomas, 31 patients were identified with a primary leiomyosarcoma of bone. There were 12 males and 19 females with a mean age of 46 and tumour size of 8 cm. The most common site was the distal femur followed by the proximal tibia. Treatment was with chemotherapy and surgical resection. Seven of the patients had metastases at diagnosis. Surgery was carried out in 28 patients, 8 having amputation and 20 limb salvage. Three patients developed local recurrence, but half developed metastases. All patient disease-specific survival was 57% at five years and 44% at 10 yrs but for those without metastases was 82% and 60%, respectively. The only prognostic factors were metastases at diagnosis. Conclusion. Leiomyosarcoma of bone is a very rare primary malignant bone tumour affecting a predominantly older population. Despite the high incidence of metastases, survival is better than for other bone sarcomas for those without metastases at diagnosis. Hindawi Publishing Corporation 2012 2012-03-11 /pmc/articles/PMC3329678/ /pubmed/22550421 http://dx.doi.org/10.1155/2012/636849 Text en Copyright © 2012 P. Brewer et al. https://creativecommons.org/licenses/by/3.0/ 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
Brewer, P.
Sumathi, V.
Grimer, R. J.
Carter, S. R.
Tillman, R. M.
Abudu, A.
Jeys, L.
Primary Leiomyosarcoma of Bone: Analysis of Prognosis
title Primary Leiomyosarcoma of Bone: Analysis of Prognosis
title_full Primary Leiomyosarcoma of Bone: Analysis of Prognosis
title_fullStr Primary Leiomyosarcoma of Bone: Analysis of Prognosis
title_full_unstemmed Primary Leiomyosarcoma of Bone: Analysis of Prognosis
title_short Primary Leiomyosarcoma of Bone: Analysis of Prognosis
title_sort primary leiomyosarcoma of bone: analysis of prognosis
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3329678/
https://www.ncbi.nlm.nih.gov/pubmed/22550421
http://dx.doi.org/10.1155/2012/636849
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