Myofibromatosis: a case report with a unique clinical presentation

Abstract

Myofibroma and myofibromatosis have been described under different names since 1951. These lesions are a benign fibroblastic and myofibroblastic proliferation containing a biphasic presentation of spindle-shaped cells surrounding a central zone of less differentiated cells focally arranged in a hemangiopericytoma-like pattern. Classically, these lesions are described in children younger than 2, with two thirds present at birth, and rarely in adults. The typical clinical presentation shows variable growth pattern of a painless purple to pink soft tissue mass, often showing secondary ulceration. Controversy exists as to an autosomal dominant or recessive inheritance versus sporadic occurrence. Presented here is a unique case of myofibromatosis presenting first as a superficial scalp lesion at age 2, followed by other primary lesions of the right mandibular vestibule, right temple, and left mandibular vestibule at ages 9, 12, and 23, respectively. All were treated with excision, without recurrence at the primary site.