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Rare Lower Extremity Fibrosarcomatous Variant of Dermatofibrosarcoma With Myxoid Features Treated With Transtibial Amputation

Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor originating from the dermis, with high rates of local recurrence and invasive growth but low likelihood of distant metastasis. Fibrosarcomatous transformation (FS-DFSP) of DFSP accounts for approximately 5-15% of DFSP tumors, is...

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Autores principales: Pryor, Elizabeth K, Sinkler, Margaret A, Ullah, Asad, Martin, Elizabeth, Homlar, Kelly
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8523476/
https://www.ncbi.nlm.nih.gov/pubmed/34692297
http://dx.doi.org/10.7759/cureus.18079
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author Pryor, Elizabeth K
Sinkler, Margaret A
Ullah, Asad
Martin, Elizabeth
Homlar, Kelly
author_facet Pryor, Elizabeth K
Sinkler, Margaret A
Ullah, Asad
Martin, Elizabeth
Homlar, Kelly
author_sort Pryor, Elizabeth K
collection PubMed
description Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor originating from the dermis, with high rates of local recurrence and invasive growth but low likelihood of distant metastasis. Fibrosarcomatous transformation (FS-DFSP) of DFSP accounts for approximately 5-15% of DFSP tumors, is a higher-grade tumor, with higher chances of metastasis and poorer prognosis. We present a case of a 66-year-old female presented with a large fungating mass on the left dorsal foot. Ultrasound-guided core needle biopsy with immunohistochemistry suggested a spindle cell neoplasm, favoring myxofibrosarcoma with intermediate grade. The patient elected for below-knee amputation over limb salvage with wide resection and free flap reconstruction. Based on clinical presentation, radiologic, histologic features and fluorescence in situ hybridization (FISH) studies confirmed the diagnosis of fibrosarcomatous variant of dermatofibrosarcoma protuberans with myxoid change. FS-DFSP with myxoid change is a rare soft tissue tumor that requires aggressive treatment due to its high rates of recurrence. This case presents a rare tumor in a unique location that was successfully treated with limb amputation, which is not documented in current literature.
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spelling pubmed-85234762021-10-22 Rare Lower Extremity Fibrosarcomatous Variant of Dermatofibrosarcoma With Myxoid Features Treated With Transtibial Amputation Pryor, Elizabeth K Sinkler, Margaret A Ullah, Asad Martin, Elizabeth Homlar, Kelly Cureus Pathology Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue tumor originating from the dermis, with high rates of local recurrence and invasive growth but low likelihood of distant metastasis. Fibrosarcomatous transformation (FS-DFSP) of DFSP accounts for approximately 5-15% of DFSP tumors, is a higher-grade tumor, with higher chances of metastasis and poorer prognosis. We present a case of a 66-year-old female presented with a large fungating mass on the left dorsal foot. Ultrasound-guided core needle biopsy with immunohistochemistry suggested a spindle cell neoplasm, favoring myxofibrosarcoma with intermediate grade. The patient elected for below-knee amputation over limb salvage with wide resection and free flap reconstruction. Based on clinical presentation, radiologic, histologic features and fluorescence in situ hybridization (FISH) studies confirmed the diagnosis of fibrosarcomatous variant of dermatofibrosarcoma protuberans with myxoid change. FS-DFSP with myxoid change is a rare soft tissue tumor that requires aggressive treatment due to its high rates of recurrence. This case presents a rare tumor in a unique location that was successfully treated with limb amputation, which is not documented in current literature. Cureus 2021-09-18 /pmc/articles/PMC8523476/ /pubmed/34692297 http://dx.doi.org/10.7759/cureus.18079 Text en Copyright © 2021, Pryor et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Pathology
Pryor, Elizabeth K
Sinkler, Margaret A
Ullah, Asad
Martin, Elizabeth
Homlar, Kelly
Rare Lower Extremity Fibrosarcomatous Variant of Dermatofibrosarcoma With Myxoid Features Treated With Transtibial Amputation
title Rare Lower Extremity Fibrosarcomatous Variant of Dermatofibrosarcoma With Myxoid Features Treated With Transtibial Amputation
title_full Rare Lower Extremity Fibrosarcomatous Variant of Dermatofibrosarcoma With Myxoid Features Treated With Transtibial Amputation
title_fullStr Rare Lower Extremity Fibrosarcomatous Variant of Dermatofibrosarcoma With Myxoid Features Treated With Transtibial Amputation
title_full_unstemmed Rare Lower Extremity Fibrosarcomatous Variant of Dermatofibrosarcoma With Myxoid Features Treated With Transtibial Amputation
title_short Rare Lower Extremity Fibrosarcomatous Variant of Dermatofibrosarcoma With Myxoid Features Treated With Transtibial Amputation
title_sort rare lower extremity fibrosarcomatous variant of dermatofibrosarcoma with myxoid features treated with transtibial amputation
topic Pathology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8523476/
https://www.ncbi.nlm.nih.gov/pubmed/34692297
http://dx.doi.org/10.7759/cureus.18079
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