AJSP2408 - CME/CMLE - Plexiform fibrohistiocytic tumor: a series of ten cases study-Plexiform fibrohistiocytic tumor: a series of ten cases study

Plexiform fibrohistiocytic tumor: a series of ten cases study

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The journal article explores the clinicopathologic features, differential diagnosis, and pathogenesis of plexiform fibrohistiocytic tumor (PFHT). PFHT is classified as a rare, low-grade malignant soft tissue neoplasm primarily affecting children and young adults, with a preference for females and upper extremity locations. Ten cases were analyzed from Xi Jing Hospital, evaluating clinical data, microscopic observations, and immunohistochemical studies focusing on markers like Cyclin D1 and CD10, which were significant in diagnosis and understanding the tumor's behavior.<br /><br />The study identified three histologic types in the cases: histiocytoid, fibroblastic, and mixed types. Despite the absence of CCND1 gene amplification or breaks, Cyclin D1 and CD10 were noted for their diagnostic and differential diagnostic value. Immunohistochemically, the tumors exhibited certain markers like CD68, cyclin D1, and vimentin, among others, contributing to understanding the tumor’s pathology without relying solely on genetic amplification. <br /><br />Clinically, PFHT often presents as a small, slow-growing, painless mass located subcutaneously, predominantly in the upper extremities such as fingers and wrists, but it can also affect the head and neck regions. PFHT captures interest due to its similarities with other tumors like Giant Cell Tumor of Soft Tissue and cellular neurothekeoma, complicating differential diagnosis.<br /><br />Moreover, the high rate of local recurrence is acknowledged due to the tumor’s infiltrative nature, with recommendations for thorough follow-up due to its rare potential for metastasis. This study emphasized the potential role of cell cycle regulatory pathways in PFHT pathogenesis, more specifically, the overexpression of Cyclin D1, which signifies possible new avenues for prognosis and treatment strategy discussions, albeit requiring more extensive research for validation.

Keywords

plexiform fibrohistiocytic tumor

clinicopathologic features

differential diagnosis

pathogenesis

Cyclin D1

CD10

immunohistochemical studies

soft tissue neoplasm

local recurrence

cell cycle regulatory pathways