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Keywords = myxoinflammatory fibroblastic sarcoma

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11 pages, 264 KiB  
Review
Recent Advances in the Diagnosis, Pathogenesis, and Management of Myxoinflammatory Fibroblastic Sarcoma
by Jun Nishio, Shizuhide Nakayama and Mikiko Aoki
Int. J. Mol. Sci. 2024, 25(2), 1127; https://doi.org/10.3390/ijms25021127 - 17 Jan 2024
Cited by 4 | Viewed by 2885
Abstract
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 [...] Read more.
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. Immunohistochemistry plays a very limited role in the diagnosis of MIFS. Several genetic alterations have been identified in MIFS, including a t(1;10)(p22;q24) translocation with TGFBR3 and/or OGA rearrangements, BRAF rearrangement, and VGLL3 amplification. Although it appears that VGLL3 amplification is the most consistent alteration, the molecular pathogenesis of MIFS remains poorly understood. A wide resection is considered the standard treatment for MIFS. Radiotherapy may be a viable option in cases with inadequate surgical margins or cases where surgery is likely to cause significant functional impairment. The systemic treatment options for advanced or metastatic disease are very limited. This review provides an updated overview of the clinicoradiological features, pathogenesis, histopathology, and treatment of MIFS. Full article
(This article belongs to the Special Issue Pathogenesis and Novel Therapeutic Approaches for Sarcomas)
6 pages, 1276 KiB  
Review
Pleomorphic Hyalinizing Angiectatic Tumor (PHAT): Review of the Literature with Case Presentation
by Gerardo Cazzato, Anna Colagrande, Antonietta Cimmino, Teresa Lettini, Maria Teresa Savino, Carmen Martella, Giuseppe Ingravallo and Leonardo Resta
Dermatopathology 2021, 8(2), 97-102; https://doi.org/10.3390/dermatopathology8020015 - 4 Apr 2021
Cited by 10 | Viewed by 4440
Abstract
Pleomorphic hyalinizing angiectatic tumor (PHAT) is a very rare entity of soft tissue considered a “neoplasm of uncertain behaviour of connective or other soft tissue” by the World Health Organization (2020). It develops in subcutaneous tissue of the lower extremities, more frequently in [...] Read more.
Pleomorphic hyalinizing angiectatic tumor (PHAT) is a very rare entity of soft tissue considered a “neoplasm of uncertain behaviour of connective or other soft tissue” by the World Health Organization (2020). It develops in subcutaneous tissue of the lower extremities, more frequently in the region of the ankle and foot, and rarely as a deep-seated soft tissue mass in locations such as the perineum, buttock, arms, head and neck, and viscera. Although inconsistent cytogenetic data have been reported on PHAT so far, there are potential morphological and genetic overlaps with hemosiderotic fibrolipomatous tumor (HFLT) and myxoinflammatory fibroblastic sarcoma (MIFS). Here we report a case of PHAT at the level of the upper third of the right thigh in a 48-year-old patient and we also focus on the differential diagnoses of these entities and conduct a literature review of reported cases. Full article
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