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.

Acral myxoinflammatory fibroblastic sarcoma with hybrid features of hemosiderotic fibrolipomatous tumor occurring 10 years after renal transplantation

“…However, these abnormalities have not been found in all cases of MIFS. 18 , 19 Antonescu et al 15 documented consistent t(1;10) with TGFBR3 and MGEA5 gene rearrangements in both MIFS and HFLT, including all three hybrid MIFS/HFLT studied, suggesting that these might represent different clinical and morphologic spectra of the same biologic entity. TGFBR3 and/or MGEA5 rearrangements have also been shown in 3/3 hybrid MIFS/HFLT by Carter et al 18 However, this group recently assessed a large series including MIFS, HFLT and MIFS and found only 2/31 MIFS to contain MGEA5 rearrangements with no MIFS harboring TGFBR3 rearrangements, while 1/1 HFLT showed TGFBR3 and MGEA5 rearrangements and 6/8 hybrid HFLT/MIFS contained TGFBR3 and/or MGEA5 rearrangements.…”

Myxoinflammatory Fibroblastic Sarcoma of the Scalp

“…MIFS on the scalp has only been previously described once, and never in adultsthe previous case was in a 5-year-old child. 8 Of over 120 cases in the literature, nodal metastases have only been described in 4 cases. MIFS was first characterized as "inflammatory myxohyaline tumor" in a large case series by Montgomery et al in 1998, 3 and almost simultaneously by Meis-Kindblom et al as "acral myxoinflammatory fibroblastic sarcoma" 2 and Michal as "inflammatory myxoid tumor of the soft parts with bizarre giant cells."…”

The t(1;10)(p22;q24) TGFBR3/MGEA5 Translocation in Pleomorphic Hyalinizing Angiectatic Tumor, Myxoinflammatory Fibroblastic Sarcoma, and Hemosiderotic Fibrolipomatous Tumor

“…The frequency of MIFS is significantly higher than those of PHAT and HFLT; more than 200 cases have been reported. [2][3][4][43][44][45] Most patients are adults in the fifth or sixth decade of life (range, 4-93 years), with equal sex distribution. The common clinical presentation is a slowly growing, illdefined mass at the dorsal part of distal extremities (fingers, hands, wrists, toes, feet, ankles), with or without pain or tenderness.…”

“…The very low percentage of TGFBR3/ MGEA5 rearrangements in classical MIFS in their prospective studies (3 of 37) and in another study (0 of 3) further supports the authors' notion. 8,9,45 Personally, we find it difficult and subjective to define the difference between classical MIFS and MIFS-like tumors, because of the nonspecificity of these morphologic features. Other experts do not deem hyalinized stroma to be a critical feature for MIFS, in contrast to the Mayo Clinic group.…”