Primary extraskeletal myxoid chondrosarcoma of bone: Report of three cases and review of the literature
| Autor: | C. Ghinelli, D. Vanel, P. Picci, Stefania Benini, Tommaso Frisoni, L. Finos, Marco Gambarotti, Alberto Righi |
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| Přispěvatelé: | Finos, L., Righi, A., Frisoni, T., Gambarotti, M., Ghinelli, C., Benini, S., Vanel, D., Picci, P. |
| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Male Pathology medicine.medical_specialty Receptors Steroid Proximal humerus Myoepithelioma DNA-Binding Protein Chondrosarcoma Bone and Bones Translocation Genetic Pathology and Forensic Medicine Extraskeletal myxoid chondrosarcoma Lesion Diagnosis Differential 03 medical and health sciences 0302 clinical medicine medicine Humans Bone In Situ Hybridization Fluorescence Aged Receptors Thyroid Hormone medicine.diagnostic_test Molecular analysi business.industry Soft tissue Magnetic resonance imaging Cell Biology Extraskeletal Myxoid Chondrosarcoma Middle Aged Magnetic Resonance Imaging DNA-Binding Proteins Contrast medium 030104 developmental biology 030220 oncology & carcinogenesis Female Differential diagnosis medicine.symptom Neoplasm Recurrence Local RNA-Binding Protein EWS business Neoplasms Connective and Soft Tissue Bone and Bone Human |
| Zdroj: | Pathology, research and practice. 213(5) |
| ISSN: | 1618-0631 |
| Popis: | Extraskeletal myxoid chondrosarcoma is a rare neoplasm of soft tissue. The usual location is in deep parts of the proximal extremities and limb girdles in middle-aged adults. The bone location as primary location is extremely rare and few cases are reported. We present three cases arising in bone with molecular confirmation using both RT-PCR and FISH analysis. Patients include two men and one woman with an age of 62, 69 and 73 years old. The mean size of the lesion was 13 cm (range 8–18 cm). Tumors arose in the iliac bone in two cases and in the proximal humerus in the other case. At time of diagnosis the three cases show bone cortex and soft tissue involvement. On imaging, lesions have a lobular pattern, are purely lytic, but take up contrast medium after injection. Two patients are alive with disease (local recurrence and lung metastasis) after five years and five years and six months, respectively and one patient died of disease two years after the diagnosis. The primary extraskeletal myxoid chondrosarcoma of bone seems to have a more aggressive behavior than the soft tissue counterpart. The molecular confirmation of diagnosis using RT-PCR is necessary to do the differential diagnosis with other entities, in particular with myoepithelioma that shows similar morphological features and EWSR1 and FUS genes rearrangement. |
| Databáze: | OpenAIRE |
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