Plexiform Myofibroblastoma: Clinicopathologic Analysis of 36 Cases of a Distinctive Benign Tumor of Soft Tissue Affecting Mainly Children and Young Adults

The spectrum of benign superficial fibroblastic/myofibroblastic tumors continues to expand and includes entities such as plexiform fibrohistiocytic tumor, dermatomyofibroma and fibroblastic connective tissue nevus. Here, we describe a seemingly distinctive group of lesions which we have labeled “plexiform myofibroblastoma” (PM). PM is a rare superficial mesenchymal tumor of fibroblastic/myofibroblastic lineage that predominantly occurs in children and young adults. Thirty-six cases from the consultation archives of one of the authors have been studied to characterize the clinicopathologic characteristics of PM. 19 patients (53%) were female and 17 were male, with age at presentation ranging from congenital (2 cases) to 50 years of age (median: 9.5 y). Three patients had multiple lesions. Males tended to develop tumors during childhood (median: 2 y; range: congenital—37 y), while in females the age distribution was relatively uniform from childhood through adulthood (median age: 25 y; range: 4 mo to 50 y). Most tumors occurred in truncal locations (25/40), including the back (11), anterolateral chest wall (4), axilla (4), abdominal wall (4), perineum (1) and suprapubic region (1). Other tumor sites were the neck (10/40), occiput (2), lower extremity (2) and breast (1). The average greatest dimension was 2.7±1.7 cm (range: 0.6 to 8 cm). Three male patients, 2 of whom were brothers, presented between 6 months and 1 year of age with multiple lesions variabl...
Source: The American Journal of Surgical Pathology - Category: Pathology Tags: Original Articles Source Type: research