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

CA. Dehner, H. Pearson, SS. Almohsen, YC. Lo, JJ. Thangaiah, J. Torres-Mora, RR. Guo, JC. Baker, AL. Folpe, AK. Alomari, BC. Dickson, SD. Billings, M. Michal, EG. Demicco, KJ. Fritchie, JSA. Chrisinger

. 2024 ; 37 (12) : 100599. [pub] 20240823

Language English Country United States

Document type Journal Article

Persistent link   https://www.medvik.cz/link/bmc25003296

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.

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$a Acral fibrochondromyxoid tumor (AFCMT) is a recently described likely benign mesenchymal neoplasm arising in the distal extremities with distinctive histologic features and a recurrent THBS1::ADGRF5 fusion. We studied an additional 37 cases of AFCMT and expanded on the so-far reported clinicopathologic and molecular findings. Tumors occurred in 21 females and 16 males, ranging in age from 17 to 78 years (median age: 47), and solely involved the hands (24/37, 65%) or feet (13/37, 35%). Histologic examination revealed well-delineated uni- or multinodular tumors with prominent vasculature-rich septa and bland, chondrocyte-like tumor cells set within abundant chondromyxoid stroma. Immunohistochemical studies showed that tumor cells were positive for CD34 (25/27; 93%) and ERG (27/27; 100%), whereas negative for S100 protein (0/31). Molecular analysis revealed evidence of a THBS1::ADGRF5 fusion in 17 of 19 (89%) successfully tested tumors. Clinical follow-up was available in 8 cases (median: 97 months), with multiple local recurrences in 1 case at 276, 312, and 360 months. We conclude that AFCMT is a distinct entity with reproducible morphologic, immunohistochemical, and molecular genetic features that should be differentiated from other similar appearing acral mesenchymal neoplasms.
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$a Pearson, Hadley $u Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio
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$a Almohsen, Shahd S $u Department of Pathology and Laboratory Medicine, Mount Sinai Hospital and Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada
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$a Lo, Ying-Chun $u Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
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$a Guo, Ruifeng Ray $u Department of Laboratory Medicine and Pathology, Mayo Clinic, Jacksonville, Florida; Department of Dermatology, Mayo Clinic, Jacksonville, Florida
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$a Baker, Jonathan C $u Mallinckrodt Institute of Radiology, Musculoskeletal Section, Washington University School of Medicine, St. Louis, Missouri
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$a Folpe, Andrew L $u Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota
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$a Alomari, Ahmed K $u Department of Anatomic Pathology and Laboratory Medicine, Indiana University, Indianapolis, Indiana
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$a Michal, Michael $u Department of Pathology, Faculty of Medicine in Pilsen, Charles University, Pilsen, Czech Republic; Bioptical Laboratory Ltd, Pilsen, Czech Republic
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$a Demicco, Elizabeth G $u Department of Pathology and Laboratory Medicine, Mount Sinai Hospital and Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada
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$a Fritchie, Karen J $u Robert J Tomsich Pathology and Laboratory Medicine Institute, Cleveland Clinic, Cleveland, Ohio
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