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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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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Malaysian Orthopaedic Association
2020
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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 |
collection | PubMed |
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. |
format | Online Article Text |
id | pubmed-7156166 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | Malaysian Orthopaedic Association |
record_format | MEDLINE/PubMed |
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 |
work_keys_str_mv | AT delapazem marjolinsulcerinlaronsyndromeanunexpectedcombinationacasereport |