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It is a benign, small tumor located in the skin of the distal areas of the legs and, less commonly, the arm; it has occurred mostly in females. EWSR1-SMAD3-positive fibroblastic tumor was named based on the finding that its tumor cells express a EWSR1-SMAD3 fusion gene. Since its initial description in 2018, a total of 15 cases have been ...
Inflammatory myofibroblastic tumor was initially regarded as a benign tumor that most often developed in the lung and less commonly in almost any organ system or tissue. Over time, however, IMT cases occurred in which the tumor spread into local tissues, metastasized to distal tissues, recurred after treatment, or consisted of neoplastic cells ...
In one study, metastatic disease was detected in 23% of patients and occurred at a median of 10 months (range, 2–77 months) after resection of the primary tumor. In a review of multiple studies, the risk of developing metastases for lower grade MFS (defined in the following section) was <5% and for higher grade tumors was 25–30%. [ 11 ]
Undifferentiated pleomorphic sarcoma (UPS), also termed pleomorphic myofibrosarcoma, [1] high-grade myofibroblastic sarcoma, and high-grade myofibrosarcoma, [2] is characterized by the World Health Organization (WHO) as a rare, poorly differentiated neoplasm (i.e., an abnormal growth of cells that have an unclear identity and/or cell of origin). [3]
IMF tumors are usually painless, well-encapsulated, rubbery to hard, and freely movable-to-fixed masses. [8] They may be evident at birth in up to 60% of cases [4] but generally go undetected until they [9] are diagnosed in the first year of life, [8] uncommonly in older infants and young (<10 years/old) children, [4] or rarely in older children and adults (one individual was diagnosed with ...
Low-grade myofibroblastic sarcoma (LGMS) is a subtype of the malignant sarcomas. [1] As it is currently recognized, LGMS was first described as a rare, atypical myofibroblastic tumor (i.e. a tumor consisting of cells with the microscopic features of fibroblasts and smooth muscle cells) by Mentzel et al. in 1998. [2]
The World Health Organization in 2020 classified mammary type myofibroblastoma tumors and myofibroblastoma tumors (i.e. extramammary myofibroblastic tumors) as separate tumor forms within the category of fibroblastic and myofibroblastic tumors. [8] Mammary MFB likely represents less than 1% of all breast tumors. [9]
The World Health Organization (2020) classified LGFMS as a specific type of tumor in the category of malignant fibroblastic and myofibroblastic tumors. [3] LGFMS tumors occur in individuals of almost any age but up to 20% are less than 18 years/old. The tumors typically involve the proximal extremities but can occur virtually anywhere in the ...