[Leprosy with an unusual course]. / Une lèpre d'évolution atypique.

In developed countries, Hansen disease, or leprosy, is a rare and little-known disease. Over the last few years, its prevalence in New Caledonia has remained stable (0.35 per 10,000 inhabitants). We report the case of an 11-year-old child who presented lepromatous leprosy complicated by a type 2 reaction. Despite appropriate treatment, the course was unusual with fever lasting a few weeks associated with asthenia, weight loss, and biological perturbations such as inflammatory syndrome, anemia, and hyperferritinemia. After a brief review of Hansen disease and its complications, we discuss the different hypotheses that can explain the clinical and biological progression of our patient (hemolytic anemia secondary to dapsone, type 2 reaction, and aspects of hemophagocytic syndrome) and describe therapeutic management, which led to a good outcome.

[Results of resection for ductal adenocarcinoma of the pancreatic head]. / Résultats carcinologiques de l'exérèse chirurgicale du cancer glandulaire céphalique du pancréas.

INTRODUCTION: Pancreaticoduodenectomy (PD) is the only curative treatment for adenocarcinoma of the pancreatic head but is associated with a significant early morbidity and a poor long term survival. Therefore, its value is still debated. The aim of this study was to evaluate early and distant results of PD for pancreatic adenocarcinoma, and to identify prognostic factors. SUMMARY: Seventy-nine patients who underwent PD with curative intent for adenocarcinoma of the pancreatic head from 1982 to 2002 were studied retrospectively. The following data were evaluated: operative mortality, long-term survival, prognostic factors (through univariate and multivariate analysis), and characteristics of 5-year survivors. RESULTS: Mortality rate was 1.3%. Survival at 1, 3 and 5 years was 46%, 26% and 11%. The median survival was 12 months. The prognostic factors were the T stage (T.N.M. classification) and radicality of resection. After multivariate analysis, radicality of resection was the only independent prognostic factor. Five patients survived for more than 5 years. They did not differ of the other patients but none had positive margin or venous invasion. CONCLUSIONS: These results (low mortality, significant distant survival including some long term survivors) suggest that PD for pancreatic adenocarcinoma must be indicated in most low-risk patients. PD remains the only curative treatment allowing prolonged survival.

Small cell neuroendocrine carcinoma of the nasal cavity and paranasal sinuses.

INTRODUCTION: Small cell neuroendocrine carcinoma (SNEC) of the sinonasal tract is a rare disease. OBJECTIVE: Report a descriptive study of a relatively large cohort of SNEC of the nasal cavity and paranasal sinuses. METHOD: The medical records of 21 patients presenting with nasal and paranasal SNEC to various French hospitals, from 1989 to 2003, were analysed to determine the clinical features and current treatment of the disease. RESULTS: Patient data were obtained from eight French hospitals. Twelve of the patients were male and nine were female, with a mean age at presentation of 55 years (range: 27 to 79 years). Patients' staging for nasal cavity malignancy was: T1, four; T2, three; T3, one; T4, 13; N0, 18; N2, three; M0, 20; and M1, one. None of the patients suffered from SNEC of the sinonasal tract with ectopic hormone production. Immunohistochemistry proved useful for diagnosis in 20 cases. Twelve cases were positive for cytokeratin, 14 for chromogranin, eight for neuron-specific enolase and 11 for neuron-specific synaptophysin. One patient had an adenocarcinoma and an inverted papilloma associated with neuroendocrine carcinoma. Patients underwent surgery (11 cases), radiotherapy (14 cases) and chemotherapy (12 cases). Recurrence occurred in 10 cases. Five patients had visceral metastases or cervical lymph node involvement. Nine of the patients died within four years of onset of the disease. CONCLUSION: Small cell neuroendocrine carcinoma of the sinonasal tract is an uncommon neoplasm with aggressive clinical behaviour. Recurrence is frequent and the prognosis is poor. However, the current treatment of these neuroendocrine neoplasms varies widely.

[Laryngeal schwannoma: a case report and review of the literature]. / Schwannome laryngé: a propos d'un cas et revue de la littérature.

OBJECTIVES: In presenting a case of laryngeal schwannoma, the authors emphasize the difficulties in diagnosing this rare pathology and review treatment options. The recent literature concerning this benign tumour is reviewed. MATERIALS AND METHOD: The authors present a case of laryngeal schwannoma in a 29 year old man, revealed by exertional inspiratory dyspnea. RESULTS: The patient was treated by a conservative surgery with no recurrence after two years follow-up. CONCLUSION: CT scan and magnetic resonance imaging provide essentiel data on the spread of this benign tumour. The possibility of malignant change and recurrence warrant long-term follow-up.