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Marjolin’s Ulcer in Laron Syndrome - an Unexpected Combination: A Case Report

Marjolin’s ulcer is an atypical malignancy that develops from deep scars of chronically traumatised skin. Laron syndrome (LS) is a rare autosomal recessive growth retardation from a mutation in the growth hormone receptor (GHR) gene leading to defective GHR, growth hormone insensitivity and eventual...

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Autor principal: de la Paz, EM
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Malaysian Orthopaedic Association 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7156166/
https://www.ncbi.nlm.nih.gov/pubmed/32296486
http://dx.doi.org/10.5704/MOJ.2003.012
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author de la Paz, EM
author_facet de la Paz, EM
author_sort de la Paz, EM
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description Marjolin’s ulcer is an atypical malignancy that develops from deep scars of chronically traumatised skin. Laron syndrome (LS) is a rare autosomal recessive growth retardation from a mutation in the growth hormone receptor (GHR) gene leading to defective GHR, growth hormone insensitivity and eventual low levels of insulin-like growth factor type 1 (IGF-1). Affected individuals present with proportionate dwarfism and other characteristic physical defects, but at the same time are conferred protection against cancer due to low serum levels of IGF-1. We report an exceptional case of Marjolin’s ulcer in the foot of a female LS patient 30 years after she sustained flame burns as a 6-month-old baby. Three months before coming to us, she had a 2x3cm ulcer that turned into a rapidly enlarging fungating mass involving the leg, ankle, and foot. Histopathologic analysis of an incision biopsy showed well-differentiated squamous cell carcinoma. The extent of her lesion precluded wide excision. Below knee amputation was done. A second biopsy confirmed the histopathologic diagnosis. This is the first reported case in the literature of Marjolin’s ulcer in LS which raises the possibility that IGF-1 deficiency does not completely protect against squamous cell cancer.
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spelling pubmed-71561662020-04-15 Marjolin’s Ulcer in Laron Syndrome - an Unexpected Combination: A Case Report de la Paz, EM Malays Orthop J Case Report Marjolin’s ulcer is an atypical malignancy that develops from deep scars of chronically traumatised skin. Laron syndrome (LS) is a rare autosomal recessive growth retardation from a mutation in the growth hormone receptor (GHR) gene leading to defective GHR, growth hormone insensitivity and eventual low levels of insulin-like growth factor type 1 (IGF-1). Affected individuals present with proportionate dwarfism and other characteristic physical defects, but at the same time are conferred protection against cancer due to low serum levels of IGF-1. We report an exceptional case of Marjolin’s ulcer in the foot of a female LS patient 30 years after she sustained flame burns as a 6-month-old baby. Three months before coming to us, she had a 2x3cm ulcer that turned into a rapidly enlarging fungating mass involving the leg, ankle, and foot. Histopathologic analysis of an incision biopsy showed well-differentiated squamous cell carcinoma. The extent of her lesion precluded wide excision. Below knee amputation was done. A second biopsy confirmed the histopathologic diagnosis. This is the first reported case in the literature of Marjolin’s ulcer in LS which raises the possibility that IGF-1 deficiency does not completely protect against squamous cell cancer. Malaysian Orthopaedic Association 2020-03 /pmc/articles/PMC7156166/ /pubmed/32296486 http://dx.doi.org/10.5704/MOJ.2003.012 Text en © 2020 Malaysian Orthopaedic Association (MOA). All Rights Reserved http://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 work is properly cited
spellingShingle Case Report
de la Paz, EM
Marjolin’s Ulcer in Laron Syndrome - an Unexpected Combination: A Case Report
title Marjolin’s Ulcer in Laron Syndrome - an Unexpected Combination: A Case Report
title_full Marjolin’s Ulcer in Laron Syndrome - an Unexpected Combination: A Case Report
title_fullStr Marjolin’s Ulcer in Laron Syndrome - an Unexpected Combination: A Case Report
title_full_unstemmed Marjolin’s Ulcer in Laron Syndrome - an Unexpected Combination: A Case Report
title_short Marjolin’s Ulcer in Laron Syndrome - an Unexpected Combination: A Case Report
title_sort marjolin’s ulcer in laron syndrome - an unexpected combination: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7156166/
https://www.ncbi.nlm.nih.gov/pubmed/32296486
http://dx.doi.org/10.5704/MOJ.2003.012
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