title: [De novo (type 3) primary intraosseous carcinoma of the jaws].
abstract: BACKGROUND
  Primary intraosseous carcinoma (PIOC) of the jaws is a rare epidermoid carcinoma from epithelial origin and initially strictly localized within the bone. Histologically, type 3 PIOC (PIOC3) is a de novo primary intraosseous carcinoma. Because of the rarity of this illness, we propose an analysis of a personal case and a revue of the literature.
  
  
  METHODS
  Two search engines (Pubmed®, Sciencedirect®) were questioned over the period 1976-February 2016 by using following keywords carcinoma, intraosseous, jaws, squamous cell carcinoma. Articles reporting proven PIOC3 and mentioning a precise treatment were selected.
  
  
  RESULTS
  Thirty articles concerning 54 patients (sex ratio: 2.4; mean age: 56.8; extreme: 24-78) met the inclusion criterions. The most common symptoms were swelling (53%), pain (44.9%) and infra-alveolar nerve paresthesia (30.6%). The time to diagnosis was 13 weeks. Classification of Zwetyenga et al. showed more than 80% of T2 and T3 stages. The lesions were predominantly mandibular (85.2%) and posterior. Less than a third of patients had lymph node and 10% had distant metastasis. Treatment consisted mostly in a combination of surgery and radiotherapy. With a mean follow-up of 74.8 months, 70.8% were in remission with no evidence of recurrence. We report the case of a 58-year-old patient, with no medical history, complaining since several months about periodontitis with teeth mobility in the right mandibular area. The panoramic X-ray showed a bone lysis at the place of tooth No. 46. In the absence of alveolar healing after extraction and antibiotherapy, a biopsy was made that diagnosed a differentiated keratinizing squamous cell carcinoma. CT scan and MRI showed a mandibular cortical bone loss with involvement of adjacent structures and lymphadenopathy in the ipsilateral IB area. The patient was treated with a combination of chemotherapy and surgery. Postoperative chemo- and radiotherapy is still going on.
  
  
  CONCLUSIONS
  The PIOC3 is a rare tumor, mainly arising in males around 50. Diagnosis should be evoked in the presence of painful swelling and nervous symptoms. The time to diagnosis is long. Tumors are usually seen at late stages. Treatment classically combines surgery and radiotherapy.
authors.affiliation: Service de chirurgie maxillo-faciale, hôpital de la Conception, 147, boulevard Baille, 13385 Marseille, France; Aix-Marseille université, jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France; UMR 6057, laboratoire parole et langage (LPL), 13604 Aix-en-Provence, France. Electronic address: anne-claire.ray@ap-hm.fr.
authors.forename: A C
authors.initials: AC
authors.lastname: Ray
authors.affiliation: Service de chirurgie maxillo-faciale, hôpital Nord, chemin des Bourrely, 13015 Marseille, France; Aix-Marseille université, jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France; Aix-Marseille université, IFSTTAR, LBA UMR_T 24, 13916 Marseille, France.
authors.forename: J M
authors.initials: JM
authors.lastname: Foletti
authors.affiliation: Service de chirurgie maxillo-faciale, hôpital de la Conception, 147, boulevard Baille, 13385 Marseille, France; Aix-Marseille université, jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France.
authors.forename: N
authors.initials: N
authors.lastname: Graillon
authors.affiliation: Service de chirurgie maxillo-faciale, hôpital Nord, chemin des Bourrely, 13015 Marseille, France; Aix-Marseille université, jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France.
authors.forename: L
authors.initials: L
authors.lastname: Guyot
authors.affiliation: Service de chirurgie maxillo-faciale, hôpital de la Conception, 147, boulevard Baille, 13385 Marseille, France; Aix-Marseille université, jardin du Pharo, 58, boulevard Charles-Livon, 13284 Marseille cedex 07, France; UMR 6057, laboratoire parole et langage (LPL), 13604 Aix-en-Provence, France.
authors.forename: C
authors.initials: C
authors.lastname: Chossegros
mesh_terms: D000328:Adult; D000368:Aged; D002294:Carcinoma, Squamous Cell / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D005260:Female; D006801:Humans; D007573:Jaw Neoplasms / Q000453:epidemiology / Q000473:pathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D055815:Young Adult
keywords: Carcinoma; Carcinome; Intra-osseux; Intraosseous; Jaws; Mâchoires