Acral FibroChondroMyxoid tumor: imaging features of a new entity
| Autor: | Corinne Bouvier, Thomas Le Corroller, Hugo Nihous, Nicolas Macagno, Pierre Champsaur |
|---|---|
| Přispěvatelé: | Institut des Sciences du Mouvement Etienne Jules Marey (ISM), Centre National de la Recherche Scientifique (CNRS)-Aix Marseille Université (AMU), Assistance Publique - Hôpitaux de Marseille (APHM), Marseille medical genetics - Centre de génétique médicale de Marseille (MMG), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Gall, Valérie, Aix Marseille Université (AMU)-Centre National de la Recherche Scientifique (CNRS), Institut Marseille Maladies Rares (MarMaRa), Aix Marseille Université (AMU), Service d'Anatomo-Cyto-Pathologie et de NeuroPathologie [Hôpital de la Timone - APHM] (ACPNP), Aix Marseille Université (AMU)- Hôpital de la Timone [CHU - APHM] (TIMONE) |
| Rok vydání: | 2020 |
| Předmět: |
Adult
Pathology medicine.medical_specialty Radiography CD34 Soft Tissue Neoplasms [SDV.GEN] Life Sciences [q-bio]/Genetics 030218 nuclear medicine & medical imaging Diagnosis Differential 03 medical and health sciences 0302 clinical medicine Biopsy medicine Humans Radiology Nuclear Medicine and imaging 030203 arthritis & rheumatology [SDV.GEN]Life Sciences [q-bio]/Genetics [SDV.MHEP] Life Sciences [q-bio]/Human health and pathology Tumor medicine.diagnostic_test business.industry Soft tissue Magnetic resonance imaging Toes medicine.disease Immunohistochemistry 3. Good health Female Neoplasm Recurrence Local Differential diagnosis Extremity business Chondroma [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology |
| Zdroj: | Skeletal Radiology Skeletal Radiology, Springer Verlag, 2020, 50 (3), pp.603-607. ⟨10.1007/s00256-020-03592-9⟩ Skeletal Radiology, 2020, 50 (3), pp.603-607. ⟨10.1007/s00256-020-03592-9⟩ |
| ISSN: | 1432-2161 0364-2348 |
| Popis: | International audience; "Acral FibroChondroMyxoid tumor" (AFCMT) is a recently described distinctive subtype of acral soft tissue tumor that typically arises on the fingers and toes. We herein present the unreported imaging features of AFCMT in a 44-year-old woman. This otherwise healthy patient was referred for a painful, slow-growing, soft tissue mass in the middle finger of her right hand. Initial radiographs and computed tomography showed a small lesion centered in the soft tissue of the ulnar aspect of the proximal phalanx, associated with scalloping of the underlying bone. Magnetic resonance imaging confirmed the presence of a well-circumscribed soft tissue tumor that exhibited relatively high T2-weighted signal intensity and marked enhancement after contrast administration. Subsequent excisional biopsy was performed. Histologically, the tumor was characterized by an abundant stroma displaying fibrous, chondroid, and myxoid areas. By immunohistochemistry, tumor cells stained for CD34, ERG, and focally S100 protein. RNA-sequencing allowed detection of THBS1-ADGFR5 gene fusion which confirmed the diagnosis of AFCMT. At 2-year follow-up, the patient remains free of recurrence. AFCMT is a previously unrecognized entity that may mimic chondroma and should be considered in the differential diagnosis of soft tissue tumors with cartilaginous or myxoid stroma in the extremities. |
| Databáze: | OpenAIRE |
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