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

De La Paz Em

doi:10.5704/moj.2003.012

Abstract

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.

Highlights

Marjolin’s ulcer is an atypical malignant degeneration, usually to squamous cell carcinoma[1], of chronic deep scar tissue from previously traumatised or burned 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)
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 6month-old baby

Summary

Introduction

Marjolin’s ulcer is an atypical malignant degeneration, usually to squamous cell carcinoma[1], of chronic deep scar tissue from previously traumatised or burned skin. Marjolin’s ulcer is an atypical malignancy that develops from deep scars of chronically traumatised skin. 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.

Results

Conclusion