[Pediatric keratocystic odontogenic tumor and nevoid basal cell carcinoma syndrome. Predictive factors for recurrence and aggressiveness]. / Tumeurs kératokystiques odontogènes de l'enfant et syndrome de Gorlin. Comment expliquer les récidives et l'agressivité des lésions ?

INTRODUCTION: Keratocystic odontogenic tumors (KOT), as complications in Nevoid Basal Cell Carcinoma Syndrome (NBCCS), occur early (before 20 years of age) and are usually more aggressive. The aim of this retrospective study was to determine the clinical, histological, and genetic phenotype, of these lesions and to define predictive features of aggressiveness. PATIENTS AND METHODS: We retrospectively studied five patients presenting with one or several KOT with NBCCS. We collected their clinical, radiological, and therapeutic data, rate of recurrence or new localization. Anatomopathological examinations were reviewed systematically. Somatic PTCH, SMO and SMAD 4 sequencing were completed. RESULTS: The average age at diagnosis was 11.2 years. The average number of KOT was 3.2 most often located in the molar region. All the cysts were enucleated. Anatomopathological examination revealed the presence of satellite cysts and daughter cysts and epithelial expansion in more than 80% of cases. No somatic mutation was observed among KOT. DISCUSSION: KOT develop in the first 10 years, in patients presenting with NBCCS, and recurrence is observed in the second and third decade. KOT are typically aggressive and have a tendency to recur, especially in patients with NBCCS. Anatomopathological examination may be predictive of the lesion's aggressiveness. Understanding the genetic and immunological mechanisms should open the way for new medical treatment.

[Jaw osteosarcomas]. / Ostéosarcomes des mâchoires.

INTRODUCTION: Osteosarcoma (OS) is the most frequent bone malignant tumor. It is usually found on long bones, 5 to 10% are located on jaws, accounting for 0.5 to 1% of all facial tumors. There is little published data which concerns only few patients. Our aim was to study retrospectively cases of facial bone OS in adults, and to compare our results with published data to suggest an optimal management scheme. PATIENTS AND METHOD: Thirty-three patients were managed for an OS, from January 1997 to January 2007. Fourteen patients with a maxillary and mandibular OS, treated in first-intention in our unit, were included. The following data were analyzed: age; personal history; circumstance of discovery; clinical, functional, and physical signs; loco-regional extension and metastasis radiological investigation. The histological slides were systematically reviewed. The protocol, therapeutic outcome, and follow-up were studied. RESULTS: The mean age at diagnosis was 43. Swelling was the most frequent functional sign. The mean delay before management was 3.4 months. The most frequent radiological presentation was a lytic and hyperdense image. The diagnosis was suggested after CT scan in 57.1% of cases. The biopsy was correlated to the anatomopathological analysis in 78.6% of cases. The most common treatment was surgical exeresis completed by chemotherapy. The 5-year survival rate was 50%. DISCUSSION: Jaw OS are specific because of their localization and specific bone ultrastructure. Their management remains controversial: should they be managed like limb OS or treated more specifically? Neoadjuvant chemotherapy, even if it delays exeresis for 3 months, seems to stop the growth or reduce the tumor. An early anatomopathological analysis of the surgical piece determines adjuvant therapy. The negative prognostic factors are: maxillary localization because of limited exeresis margins, tumoral size, and osteoblastic sub-type.

[Ameloblastoma of the jaws. A retrospective analysis from 1994 to 2007]. / Améloblastomes des mâchoires. Analyse rétrospective de 1994 à 2007.

INTRODUCTION: Ameloblastomas and keratocysts are the most frequent epithelial odontogenic tumors of the jaws. They have a high recurrence rate. This retrospective study reviews the features of ameloblastomas operated on in our unit from 1994 to 2007. PATIENTS AND METHODS: The studied parameters were sex, ethnic origin, age at diagnosis, clinical signs, radiographic presentation, site distribution, histological type, treatment, and follow-up records. RESULTS: One hundred and sixteen patients were included (with 239 surgical samples). The mean age was 36 years, with a majority of Europeans, 60% of multilocular radiolucent lesions with root resorption, mandibular location (93%). Twenty-one percent of the patients presented with an impacted tooth, the third molar in 79% of cases. Fifty percent of the lesions were from 5 to 13cm in length, 10% longer than 13cm. The most common histological type was follicular ameloblastoma. Patients were treated by enucleation in 82% of cases and radical mandibular resection with reconstruction in 11% of cases. The follow-up was documented for 96% of the patients with a 44% recurrence rate. Seventy-four percent of patients with a double recurrence presented with a "follicular" ameloblastoma. DISCUSSION: We prefer a well-performed enucleation which preserves surrounding bone. The high rate of follicular type recurrence should more systematically lead to a combined treatment: periostectomy and tooth extraction. Our data was compared with previously published large series.

[Keratocysts (or keratocystic epithelial odontogenic tumors)]. / Kératokystes (ou tumeurs odontogéniques kératokystiques).

The aim of this study was to describe the management of keratocysts based on our own experience and on a large literature review. Keratocysts are benign odontogenic epithelial tumors. The main aspects of this lesion are described (definition, epidemiology, clinic, radiology, histology, treatment, and prognosis). In small intra-osseous tumors, surgical procedure must be as conservative as possible, most often enucleation. In large tumors with destruction of the cortical bone, or with destruction of coronoid process or notch, extensive resection can be indicated, with sometimes a transfacial approach for an accurate control, especially in soft tissues. Strict follow-up is mandatory because of the high risk of recurrence.

[Post-traumatic taste and smell disorders: forensic aspects]. / Dysosmies et dysgueusies post-traumatiques: aspects médicolégaux.

The impact of anosmia or ageusia may be considerable for some occupations. Expert assessment shows how difficult it is to prove the diagnosis. A specialist is usually needed to demonstrate the asserted disorder. Damage compensation is based on the patient's occupation and coverage.

A treatment algorithm for managing giant mandibular ameloblastoma: 5-year experiences in a Paris university hospital.

AIMS: To review our experiences with giant mandibular ameloblastoma (GMA) over a 5-year period, and to formulate a treatment algorithm for managing this tumour. METHODS: We retrospectively reviewed all GMA patients who underwent segmental mandibulectomy and immediate free fibular osteoseptocutaneous flap reconstruction (SM-IFFOFR) by a single reconstructive team from 2002 to 2006. All treatment methods and outcomes were analysed. FINDINGS: Forty-four ameloblastoma patients were operated upon during this study period. Sixteen cases had GMA, of which 9 patients were included in this series (mean age: 35 years). The defects in the mandible ranged from 7 to 16 cm in length (mean: 12 cm). The average length of the harvested fibula was 11 cm, and the number of osteotomies ranged from 1 to 2. The mean ischemic time was 137 min (range: 90-180 min). Neck recipient vessels were used for flap perfusion in all cases. All but one flaps were viable without any complications, whilst partial skin-island necrosis occurred in 2 patients. Hospital stay was 2 weeks in most of the patients. No tumour recurrence was found during the follow-up period (range: 26-73 months). Dental implants were placed in 2 patients. CONCLUSIONS: Despite several limitations of this study, we suggest that a radical approach with the SM-IFFOFR is an effective treatment for GMA. Further well-designed, larger series with longer follow-up periods are still encouraged.