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Background: Plexiform fibrohistiocytic tumor (PFH) is a rarely metastasizing slowly growing neoplasm usually affecting children and young adults. The tumor usually has a dermal-subcutaneous location, is poorly circumscribed, and is comprised of a plexiform or multinodular proliferation of a variable admixture of fibroblasts and histiocytoid cells with a distinctive biphasic morphology. Myxoid change in PFH is extremely rare with only five cases of myxoid variant of PFH reported to date in the English literature.

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Article Synopsis
  • - The study investigated 10 cases of plexiform fibrohistiocytic tumor (PFHT) to analyze its features, diagnose methods, and understand its origins using clinical data and microscopic techniques from patients at Xi Jing Hospital over a 14-year span.
  • - Findings revealed various types of PFHT, with most cases showing histiocytoid characteristics, and immunohistochemical markers like CD68 and cyclin D1 were prominently expressed, aiding in diagnosis.
  • - The study concluded that PFHT is a low-grade malignant tumor, with the diagnosis relying heavily on histopathology and immunohistochemistry, particularly noting the role of cyclin D1 in its pathogenesis.
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[Plexiform fibrohistiocytic tumor].

Dermatologie (Heidelb)

June 2023

Hautklinik, Universitätsklinikum Heidelberg, Ruprecht-Karls-Universität Heidelberg, Im Neuenheimer Feld 440, 69120, Heidelberg, Deutschland.

Plexiform fibrohistiocytic tumors are rare, low-to-moderate malignant soft tissue tumors that occur primarily in children and adolescents and are located on the upper extremity. The diagnosis must be made histologically. We report on a young woman who presented a growing, painless lesion on the cubital fossa.

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Plexiform fibrohistiocytic tumor (PFHT) is a mesenchymal tumor of intermediate malignancy, typically occurring in the superficial soft tissues of young patients and displaying a biphasic pattern, with nodules of histiocytoid cells surrounded by fascicles of myofibroblastic spindled cells. The pathogenesis of PHFT is unknown. We comprehensively studied 39 PFHT, occurring in 25 females (66%) and 13 males (34%), ranging from 2 to 55 years of age (median 21 years).

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