Myofibroblastic Tumors in Children

Abstract

Pediatric myofibroblastic tumors comprise a clinically and pathologically heterogeneous group of neoplasms, including benign tumors, those of intermediate biologic potential, and frankly malignant examples (Box 1). Proper classification is critical for appropriate prognostication, clinical management, and, in some cases, identification of familial syndromes. Unlike for many other soft tissue tumors, immunohistochemistry plays a limited role in the diagnosis of pediatric myofibroblastic tumors, with notable exceptions. Understanding the molecular genetics underlying the pathogenesis of several tumor types in this group has led to improved tools for diagnosis. This article discusses the clinical and pathologic features of myofibroblastic tumors in children, with special emphasis on differential diagnosis.

Keywords: ALK; Desmoid fibromatosis; Familial adenomatous polyposis; Gardner syndrome; Inflammatory myofibroblastic tumor; Pediatric; Soft tissue tumors; β-catenin.