Acral Fibrochondromyxoid Tumor: A Clinicopathologic and Molecular Genetic Study of 37 Cases.

Autor:	Dehner CA; Department of Anatomic Pathology and Laboratory Medicine, Indiana University, Indianapolis, Indiana., Pearson H; Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio., Almohsen SS; Department of Pathology and Laboratory Medicine, Mount Sinai Hospital and Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada., Lo YC; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota., Thangaiah JJ; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota., Torres-Mora J; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota., Guo RR; Department of Laboratory Medicine and Pathology, Mayo Clinic, Jacksonville, Florida; Department of Dermatology, Mayo Clinic, Jacksonville, Florida., Baker JC; Mallinckrodt Institute of Radiology, Musculoskeletal Section, Washington University School of Medicine, St. Louis, Missouri., Folpe AL; Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota., Alomari AK; Department of Anatomic Pathology and Laboratory Medicine, Indiana University, Indianapolis, Indiana., Dickson BC; Department of Pathology and Laboratory Medicine, Mount Sinai Hospital and Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada., Billings SD; Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio., Michal M; Department of Pathology, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czech Republic; Bioptical Laboratory Ltd, Pilsen, Czech Republic., Demicco EG; Department of Pathology and Laboratory Medicine, Mount Sinai Hospital and Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada., Fritchie KJ; Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio., Chrisinger JSA; Department of Pathology and Immunology, Division of Anatomic and Molecular Pathology, Washington University School of Medicine, St. Louis, Missouri. Electronic address: jschrisi@wustl.edu.
Jazyk:	angličtina
Zdroj:	Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc [Mod Pathol] 2024 Aug 23; Vol. 37 (12), pp. 100599. Date of Electronic Publication: 2024 Aug 23.
DOI:	10.1016/j.modpat.2024.100599
Abstrakt:	Acral fibrochondromyxoid tumor (AFCMT) is a recently described likely benign mesenchymal neoplasm arising in the distal extremities with distinctive histologic features and a recurrent THBS1::ADGRF5 fusion. We studied an additional 37 cases of AFCMT and expanded on the so-far reported clinicopathologic and molecular findings. Tumors occurred in 21 females and 16 males, ranging in age from 17 to 78 years (median age: 47), and solely involved the hands (24/37, 65%) or feet (13/37, 35%). Histologic examination revealed well-delineated uni- or multinodular tumors with prominent vasculature-rich septa and bland, chondrocyte-like tumor cells set within abundant chondromyxoid stroma. Immunohistochemical studies showed that tumor cells were positive for CD34 (25/27; 93%) and ERG (27/27; 100%), whereas negative for S100 protein (0/31). Molecular analysis revealed evidence of a THBS1::ADGRF5 fusion in 17 of 19 (89%) successfully tested tumors. Clinical follow-up was available in 8 cases (median: 97 months), with multiple local recurrences in 1 case at 276, 312, and 360 months. We conclude that AFCMT is a distinct entity with reproducible morphologic, immunohistochemical, and molecular genetic features that should be differentiated from other similar appearing acral mesenchymal neoplasms. (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)
Databáze:	MEDLINE
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