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

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: IGF-1 deficiency; Laron syndrome; Marjolin’s ulcer; below knee amputation; cancer protection.

Fig. 1:

Medial view of the foot. Measuring 6x10x12cm at its widest dimensions, the fungating mass extends from 6cm cephalad to the malleoli to the plantar aspect of the heel. It started as a recurrent blister at the heel similar to the lesion indicated by the arrow. Burn scars involve the entire foot and distal leg.

Fig. 2:

Lateral view of the foot. Involving the entire heel and the insertion of the tendon of Achilles, the ulcerating mass is foul-smelling with beginning necrosis in some areas. Rapid growth is noticeable at its most proximal and medial margin. Scarring affects the entire foot, ankle, and distal leg. The forefoot is abducted and everted due to the contractures. Post-burn bent contracture deformities are evident on the 2nd-5th toes.