A treatment algorithm for adult ameloblastomas according to the Pitié-Salpêtrière Hospital experience.

During a 13-year period (from 1994 to 2007), in the Oral and Maxillofacial Surgery Department of the Pitié-Salpêtrière Hospital, 116 new cases of adult ameloblastomas, were analyzed for treatment composed against radiographic presentation, size, histological type. Follow-up and recurrence were also analyzed. Treatment was surgical consisting of enucleations (82%), segmental mandibulectomy (8.3%) resections (24.7%) 85% of them underwent reconstruction. The follow-up was documented for 97%. More than two recurrences occurred in 21% of the patients after the first enucleation: 66% with a "follicular" histological diagnosis. Lenthly, a therapeutic algorithm is suggested for adult ameloblastomas that underlines the importance of the conservative enucleation treatment as far as possible.

Coordinated expression of activated mitogen-activated protein kinases in salivary gland adenoid cystic carcinoma.

Little is known regarding the mitogen-activated protein kinase activation state in salivary gland adenoid cystic carcinoma. We evaluated the expression of the phosphorylated (activated) forms of the extracellular signal-regulated, C-jun N-terminal, and P38 kinases, as well as the Ki67 index by means of immunohistochemistry in archival paraffin-embedded material in 61 treatment-naive patients. Using a semiquantitative scoring methodology, we found that tumors were heterogeneous in their expression of phosphorylated mitogen-activated protein kinases. The rankings of scores for pairs of phosphorylated kinases across tumors were correlated (P < .01 in all comparisons). The correlation was strongest for phosphorylated P38 and C-jun N-terminal kinases (Kendall's tau, 0.39; 95% confidence interval, 0.21-0.57; P = .0007). Phosphorylated C-jun N-terminal kinases were associated with perineural invasion. In the 55 patients treated by surgery, high phosphorylated P38 (hazard ratio for death, 0.27; 95% confidence interval, 0.10-0.75; P = .01) and Ki67 (hazard ratio for death, 3.86; 95% confidence interval, 1.38-10.77; P = .01) could be used to fit a Cox regression model to overall survival. Our study provides evidence that mitogen-activated protein kinases are activated in adenoid cystic carcinoma and suggests that their activation is coordinated. High phosphorylated P38 levels predict for overall survival independently of tumor cell proliferation.

Histological features and management of a mandibular Gorham disease: a case report and review of maxillofacial cases in the literature.

Gorham disease (GD) is a rare osteolysis without sex, race, or age predilection, affecting bones in different regions. Based on clinical, histological, and molecular features, diagnosis is difficult and required exclusion of neoplastic, inflammatory, infectious, and endocrinologic disease. Etiology is still unknown. We report the case of a 36-year-old man suffering from severe progressive osteolysis located at the mandible. Histology showed massive osteolysis without malignant cells. Immunohistochemistry revealed thin-walled vessels and lymphatic ducts. These investigations lead to diagnosis of GD. Radical surgical treatment was followed by bisphophonate therapy. Recurrence occurred 4 months after surgery and alphaa-interferon therapy permitted remission. To support this case report, we reviewed the 41 maxillofacial cases published in the literature since 1928. Jaw is the main location; histology mostly shows hemangioma-like proliferation. Immune disorders are usually advanced as a cause although physiopathology is unknown. Therefore, appropriate treatment is controversial. Antiosteoclastic drugs are usually proposed in addition to surgery, but immunomodulating drugs and radiation therapy should also be considered in the treatment.

High-resolution array comparative genomic hybridization analysis of human bronchial and salivary adenoid cystic carcinoma.

Adenoid cystic carcinoma (ACC) is a rare but distinctive tumor. Oligonucleotide array comparative genomic hybridization has been applied for cataloging genomic copy number alterations (CNAs) in 17 frozen salivary or bronchial tumors. Only four whole chromosome CNAs were found, and most cases had 2-4 segmental CNAs. No high level amplification was observed. There were recurrent gains at 7p15.2, 17q21-25, and 22q11-13, and recurrent losses at 1p35, 6q22-25, 8q12-13, 9p21, 12q12-13, and 17p11-13. The minimal region of gain at 7p15.2 contained the HOXA cluster. The minimal common regions of deletions contained the CDKN2A/CDKN2B, TP53, and LIMA1 tumor suppressor genes. The recurrent deletion at 8q12.3-13.1 contained no straightforward tumor suppressor gene, but the MIRN124A2 microRNA gene, whose product regulates MMP2 and CDK6. Among unique CNAs, gains harbored CCND1, KIT/PDGFRA/KDR, MDM2, and JAK2. The CNAs involving CCND1, MDM2, KIT, CDKN2A/2B, and TP53 were validated by FISH and/or multiplex ligation-dependent probe amplification. Although most tumors overexpressed cyclin D1 compared with surrounding glands, the only case to overexpress MDM2 had the corresponding CNA. In conclusion, our report suggests that ACC is characterized by a relatively low level of structural complexity. Array CGH and immunohistochemical data implicate MDM2 as the oncogene targeted at 12q15. The gain at 4q12 warrants further exploration as it contains a cluster of receptor kinase genes (KIT/PDGFRA/KDR), whose products can be responsive to specific therapies.