Myofibroma: From protein losing enteropathy to liver tumor

Abstract

Myofibromas are rare fibrous tumors, which can be solitary (myofibroma) or multiple (myofibromatosis). They can be dermatological or visceral in origin. Usually the dermatological tumors are benign and have good prognosis while those with visceral involvement are associated with poor outcome. The extent and location of these lesions are the important prognostic factors with a mortality rate of 75% with multifocal visceral involvement. The major difficulty in diagnosis is variable clinical presentation of these tumors. We report two cases which represent the wide clinical spectrum of these tumors ranging from protein losing enteropathy in infancy to liver tumor in childhood. Delay in the diagnosis and inappropriate management can be life threatening as each requires a specific treatment approach.

Keywords

• Protein losing enteropathy of uncommon etiology
• Myofibroma
• Liver mass