Myxoinflammatory fibroblastic sarcoma is a rare soft tissue tumor with most occurring in the distal extremities of adult patients. It has a high rate of local recurrence and a low rate of metastasis. Because it may appear benign on clinical examination, and because the microscopic features are generally underrecognized, it is often inadequately treated and misdiagnosed. In this review, based upon experience and that of the literature, the intent is to highlight salient clinicopathologic features, detail the broad microscopic spectrum including high-grade aggressive variants, review the molecular features, and discuss its relation to hemosiderotic fibrolipomatous tumor.
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High-Grade Sarcomatous Transformation of Hybrid Hemosiderotic Fibrolipomatous Tumor-Myxoinflammatory Fibroblastic Sarcoma with Novel Gene Fusion.
Int J Surg Pathol
January 2025
Department of Laboratory Medicine and Pathology, University of Washington, Seattle, WA, USA.
Hemosiderotic fibrolipomatous tumor (HFLT) and myxoinflammatory fibroblastic sarcoma (MIFS) are rare, locally aggressive soft tissue tumors with a predilection for distal extremities of middle-aged adults. Hybrid tumors (HFLT-MIFS) demonstrate overlapping features of both and share recurrent translocation (1;10) (p22; q24). We describe a tumor with high-grade sarcomatous transformation of a hybrid HFLT-MIFS, with a novel gene fusion, presenting as a right foot soft tissue mass in an 85-year-old woman.
View Article and Find Full Text PDFMyxoinflammatory fibroblastic sarcoma of the liver: Case report and literature review.
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Department of Radiology, Hebei General Hospital, Shijiazhuang, Hebei, China.
Rationale: Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade malignant soft tissue sarcoma that primarily affects the distal extremities in adults, with the highest incidence in patients in their 40s and 50s. It has a high local recurrence rate and a low metastasis rate. Although MIFSs have been documented in other sites, an MIFS in the liver is highly unusual.
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Department of Human Genetics, KU Leuven, University Hospitals Leuven, Leuven, Belgium.
Soft-tissue and bone tumors represent a heterogeneous group of tumors encompassing more than 100 histologic subtypes today. Identifying genetic aberrations increasingly is important in these tumors for accurate diagnosis. Although gene mutations typically are detected by second-generation sequencing, the identification of structural variants (SVs) and copy number alterations (CNAs) remains challenging and requires various cytogenetic techniques including karyotyping, fluorescence in situ hybridization, and arrays, each with important limitations.
View Article and Find Full Text PDFMyxoinflammatory Fibroblastic Sarcoma.
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Department of Pathology, Yale University School of Medicine, New Haven, CT 06520, USA. Electronic address:
MIFS is a low-grade fibroblastic sarcoma that predilects to superficial distal extremity soft tissue. It is composed of plump spindled and epithelioid cells, inflammatory infiltrates, and mucin deposits in a fibrosclerotic stroma. Large epithelioid cells harboring bizarre nuclei and virocyte-like macronucleoli and pleomorphic pseudolipoblasts are characteristic.
View Article and Find Full Text PDFRecent Advances in the Diagnosis, Pathogenesis, and Management of Myxoinflammatory Fibroblastic Sarcoma.
Int J Mol Sci
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Department of Pathology, Faculty of Medicine, Fukuoka University, 7-45-1 Nanakuma, Jonan-ku, Fukuoka 814-0180, Japan.
Myxoinflammatory fibroblastic sarcoma (MIFS) is an infiltrative, locally aggressive fibroblastic neoplasm of intermediate malignancy that typically arises in the distal extremities of middle-aged adults. It can histologically be confused with a number of benign and malignant conditions. Recently, high-grade examples of MIFS have been described.
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