Myxoinflammatory Fibroblastic Sarcoma in Children and Adolescents: Clinicopathologic Aspects of a Rare Neoplasm. (November 2013)

• Record Type: Journal Article
• Authors: Weiss, Vivian L.; Antonescu, Cristina R.; Alaggio, Rita; Cates, Justin M.; Gaskin, David; Stefanovici, Camelia; Coffin, Cheryl M.
• Is Part Of: Pediatric and developmental pathology. Volume 16:Number 6(2013)
• Page Start: 425
• Page End: 431
• Publication Date: 2013-11
• Subjects: acral myxoinflammatory fibroblastic sarcoma -- fibroma -- fibromatosis -- inflammatory myxohyaline tumor -- myxoinflammatory fibroblastic sarcoma -- myxoma; Pediatric pathology -- Periodicals; Children -- Diseases -- Periodicals; Diagnosis, Laboratory -- Periodicals; Abnormalities, Human -- Periodicals; Child development -- Periodicals; Pediatrics -- Periodicals; 616.07
• DOI: 10.2350/13-06-1353-CR.1
• Languages: English
• ISSNs: 1093-5266

Abstract

Myxoinflammatory fibroblastic sarcoma (MIFS), originally described as a low-grade malignant soft-tissue tumor in adults, has recently been reported in children and in non-acral sites. This report describes the clinicopathologic features of a series of 5 MIFS in children and adolescents (3 males, 2 females), ranging in age from 5 to 17 years (mean, 13 years). These tumors presented as small, superficial, slowly growing soft-tissues masses of the scalp, neck, middle finger, forearm, and thigh. Histologically, the tumors were composed of spindled and plump polygonal cells with prominent nuclear pleomorphism, nuclear pseudoinclusions; large eosinophilic nucleoli; myxoid foci intermingled with spindled foci; and an accompanying inflammatory infiltrate of lymphocytes, plasma cells, and variable neutrophils. Immunohistochemical analysis revealed variable reactivity for CD34 and smooth muscle actin in the tumor cells. Genetic analysis in 3 cases showed no rearrangements of TGFBR3 or MGEA5 . Follow up in 4 cases revealed no recurrence or metastasis. These 5 cases of childhood and adolescent MIFS demonstrate an expanded age range and topographic distribution and a favorable outcome. The differential diagnosis and importance of recognizing this rare neoplasm in young patients are discussed.