Title of article :
Marjolin’s Ulcer in Laron Syndrome - an Unexpected Combination: A Case Report
Author/Authors :
EM, de la Paz Department of Orthopaedics - The Medical City Clark - Angeles City - Philippines
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.
Keywords :
Marjolin's ulcer , Laron syndrome , IGF-1 deficiency , cancer protection , below knee amputation
Journal title :
Malaysian Orthopaedic Journal