title: Myxoinflammatory fibroblastic sarcoma in children and adolescents: clinicopathologic aspects of a rare neoplasm.
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.
authors.affiliation: 1  Department of Pathology, Microbiology, and Immunology, Vanderbilt University, Nashville, TN, USA.
authors.forename: Vivian L
authors.initials: VL
authors.lastname: Weiss
authors.forename: Cristina R
authors.initials: CR
authors.lastname: Antonescu
authors.forename: Rita
authors.initials: R
authors.lastname: Alaggio
authors.forename: Justin M
authors.initials: JM
authors.lastname: Cates
authors.forename: David
authors.initials: D
authors.lastname: Gaskin
authors.forename: Camelia
authors.initials: C
authors.lastname: Stefanovici
authors.forename: Cheryl M
authors.initials: CM
authors.lastname: Coffin
mesh_terms: D000293:Adolescent; D014408:Biomarkers, Tumor / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D005260:Female; D005354:Fibrosarcoma / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D012983:Soft Tissue Neoplasms / Q000378:metabolism / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor