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Fibrosarcoma: a challenging diagnosis

Fibrosarcoma represent a rare group of soft tissue malignancies derived from fibrous connective tissue and immature proliferating fibroblasts or undifferentiated anaplastic spindle cells. It affects patients in the fourth and fifth decade of life. Fibrosarcomas can be classified in subtypes such as...

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Autores principales: Ferreira, Cristiane Rúbia, da Fonseca, Leonardo Gomes, Piotto, Gustavo Henrique Munhoz, Geyer, Felipe Correa, de Alcântara, Paulo Sérgio Martins
Formato: Online Artículo Texto
Lenguaje:English
Publicado: São Paulo, SP: Universidade de São Paulo, Hospital Universitário 2013
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6671889/
https://www.ncbi.nlm.nih.gov/pubmed/31528615
http://dx.doi.org/10.4322/acr.2013.024
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author Ferreira, Cristiane Rúbia
da Fonseca, Leonardo Gomes
Piotto, Gustavo Henrique Munhoz
Geyer, Felipe Correa
de Alcântara, Paulo Sérgio Martins
author_facet Ferreira, Cristiane Rúbia
da Fonseca, Leonardo Gomes
Piotto, Gustavo Henrique Munhoz
Geyer, Felipe Correa
de Alcântara, Paulo Sérgio Martins
author_sort Ferreira, Cristiane Rúbia
collection PubMed
description Fibrosarcoma represent a rare group of soft tissue malignancies derived from fibrous connective tissue and immature proliferating fibroblasts or undifferentiated anaplastic spindle cells. It affects patients in the fourth and fifth decade of life. Fibrosarcomas can be classified in subtypes such as low-grade fibromyxoid sarcoma (LGFMS) and sclerosing epithelioid fibrosarcoma (SEF), and others. Histological features that overlap between types of fibrosarcomas is well known and reported in the literature. We report the case of a 53-year-old patient who presented a tumor in the axillary fossa, which was initially diagnosed as a solitary fibrous tumor. Due to recurrence of the lesion, as well as the presence of distant metastases, the histological revision considered the diagnosis of breast metaplastic carcinoma, since the tumor expressed the p63 antigen and estrogen and progesterone receptors. Unexpected resistance to chemotherapy motivated the diagnosis re-evaluation, which was due to MUC4 expression and morphological characteristics concluded by a hybrid LGFMS-SEF tumor. The authors call attention to the difficult diagnosis in cases of soft tissue tumors. A broad panel of immunohistochemical research is required as the clinical course is essential to the final diagnosis.
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spelling pubmed-66718892019-09-16 Fibrosarcoma: a challenging diagnosis Ferreira, Cristiane Rúbia da Fonseca, Leonardo Gomes Piotto, Gustavo Henrique Munhoz Geyer, Felipe Correa de Alcântara, Paulo Sérgio Martins Autops Case Rep Article / Autopsy Case Report Fibrosarcoma represent a rare group of soft tissue malignancies derived from fibrous connective tissue and immature proliferating fibroblasts or undifferentiated anaplastic spindle cells. It affects patients in the fourth and fifth decade of life. Fibrosarcomas can be classified in subtypes such as low-grade fibromyxoid sarcoma (LGFMS) and sclerosing epithelioid fibrosarcoma (SEF), and others. Histological features that overlap between types of fibrosarcomas is well known and reported in the literature. We report the case of a 53-year-old patient who presented a tumor in the axillary fossa, which was initially diagnosed as a solitary fibrous tumor. Due to recurrence of the lesion, as well as the presence of distant metastases, the histological revision considered the diagnosis of breast metaplastic carcinoma, since the tumor expressed the p63 antigen and estrogen and progesterone receptors. Unexpected resistance to chemotherapy motivated the diagnosis re-evaluation, which was due to MUC4 expression and morphological characteristics concluded by a hybrid LGFMS-SEF tumor. The authors call attention to the difficult diagnosis in cases of soft tissue tumors. A broad panel of immunohistochemical research is required as the clinical course is essential to the final diagnosis. São Paulo, SP: Universidade de São Paulo, Hospital Universitário 2013-09-30 /pmc/articles/PMC6671889/ /pubmed/31528615 http://dx.doi.org/10.4322/acr.2013.024 Text en Autopsy and Case Reports. ISSN 2236-1960. Copyright © 2013. http://creativecommons.org/licenses/by-nc/4.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the article is properly cited.
spellingShingle Article / Autopsy Case Report
Ferreira, Cristiane Rúbia
da Fonseca, Leonardo Gomes
Piotto, Gustavo Henrique Munhoz
Geyer, Felipe Correa
de Alcântara, Paulo Sérgio Martins
Fibrosarcoma: a challenging diagnosis
title Fibrosarcoma: a challenging diagnosis
title_full Fibrosarcoma: a challenging diagnosis
title_fullStr Fibrosarcoma: a challenging diagnosis
title_full_unstemmed Fibrosarcoma: a challenging diagnosis
title_short Fibrosarcoma: a challenging diagnosis
title_sort fibrosarcoma: a challenging diagnosis
topic Article / Autopsy Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6671889/
https://www.ncbi.nlm.nih.gov/pubmed/31528615
http://dx.doi.org/10.4322/acr.2013.024
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