Low-grade extraskeletal osteosarcoma of the chest wall: case report and review of literature
1 Department of Medical Oncology, Institut Paoli-Calmettes, 13009 Marseille, France
2 Department of Pathology, CHU La Timone, 13005 Marseille, France
3 Department of Pathology, Hôpital Trousseau, 37000 Tours, France
4 Department of Radiology, Institut Paoli-Calmettes, 13009 Marseille, France
5 Department of Nuclear Medicine, Institut Paoli-Calmettes, 13009 Marseille, France
6 Laboratory of Solid Tumours Genetics, University of Nice-Sophia-Antipolis, CNRS UMR 6543, Nice University Hospital, 06000 Nice, France
7 Department of Pathology, Institut Paoli-Calmettes, 13009 Marseille, France
8 Department of Internal Medicine, CHU La Timone, 13005 Marseille, France
9 University of Mediterranea, Marseille, France
BMC Cancer 2010, 10:645 doi:10.1186/1471-2407-10-645Published: 24 November 2010
Low-grade extraskeletal osteosarcomas (ESOS) are extremely rare.
We present the first case of low-grade ESOS of the chest wall, which occurred in a 30-year-old man. Because of initial misdiagnosis and patient's refusal of surgery, the diagnosis was done after a 4-year history of a slowly growing mass in soft tissues, leading to a huge (30-cm diameter) calcified mass locally extended over the left chest wall. Final diagnosis was helped by molecular analysis of MDM2 and CDK4 oncogenes. Unfortunately, at this time, no surgical treatment was possible due to loco-regional extension, and despite chemotherapy, the patient died one year after diagnosis, five years after the first symptoms.
We describe the clinical, radiological and bio-pathological features of this unique case, and review the literature concerning low-grade ESOS. Our case highlights the diagnostic difficulties for such very rare tumours and the interest of molecular analysis in ambiguous cases.