title: Low-grade extraskeletal osteosarcoma of the chest wall: case report and review of literature.
abstract: BACKGROUND
  Low-grade extraskeletal osteosarcomas (ESOS) are extremely rare.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Medical Oncology, Institut Paoli-Calmettes, 13009 Marseille, France.
authors.forename: Renaud
authors.initials: R
authors.lastname: Sabatier
authors.forename: Corinne
authors.initials: C
authors.lastname: Bouvier
authors.forename: Gonzague
authors.initials: G
authors.lastname: de Pinieux
authors.forename: Anthony
authors.initials: A
authors.lastname: Sarran
authors.forename: Isabelle
authors.initials: I
authors.lastname: Brenot-Rossi
authors.forename: Florence
authors.initials: F
authors.lastname: Pedeutour
authors.forename: Bruno
authors.initials: B
authors.lastname: Chetaille
authors.forename: Patrice
authors.initials: P
authors.lastname: Viens
authors.forename: Pierre-Jean
authors.initials: PJ
authors.lastname: Weiller
authors.forename: François
authors.initials: F
authors.lastname: Bertucci
mesh_terms: D000328:Adult; D051358:Cyclin-Dependent Kinase 4 / Q000096:biosynthesis; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D008297:Male; D012516:Osteosarcoma / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology*; D051736:Proto-Oncogene Proteins c-mdm2 / Q000096:biosynthesis; D012983:Soft Tissue Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology*; D035441:Thoracic Wall / Q000473:pathology*; D014057:Tomography, X-Ray Computed
chemical_list: D051736:Proto-Oncogene Proteins c-mdm2; D051358:Cyclin-Dependent Kinase 4