[Primary interstitial pancreatitis or dyschylic pancreatitis in children. Apropos of 13 cases]. / Les pancréatites interstitielles primitives ou pancréatites dyschyliques chez l'enfant. A propos de 13 observations.

During a retrospective review of more than 1,000 pediatric pancreas specimens obtained by autopsy or biopsy, 13 cases of primitive interstitial pancreatitis (PIP) were identified. The morphologic diagnosis of PIP is based on the following histological features: presence of abundant, inspissated, PAS-negative intraductal secretions, overdistension and focal rupture of the intrapancreatic ducts, and presence of a focal, extensive inflammatory infiltrate. PIP should be distinguished from cystic fibrosis, necrotizing pancreatitis, and passive secondary interstitial infiltrates associated with extensive retroperitoneal cellulitis caused by septicemia or abdominal surgery. Clinical diagnosis is difficult and was not considered in the ten children under four years of age. In the three children over five, the presence of recurrent abdominal pain with mild ascitis and jaundice led to the correct diagnosis. Pathological findings suggest two possible pathophysiologic hypotheses: lesions may develop proximal to a mechanical malformative obstruction that is, however, only very rarely found, according to a more likely alternative, quantitative and qualitative modifications of pancreatic secretions may occur as a result of severe dehydration or use of drugs (corticosteroids, diuretics), justifying the term "dyschylic pancreatitis" coined by G. Seifert.

[Intracranial tumor developing from the vitelline sac. Apropos of a case]. / Tumeur intracrânienne développée à partir du sac vitellin. A propos d'un cas.

The authors report a case of a primary intracranial yolk sac tumor which is a quite rare eventuality. The patient, an 18 months old girl was referred for severe intracranial hypertension. Neurological examination revealed a right hemiparesis, a left abducens nerve palsy and a bilateral papilledema. The serum and C.S.F. levels of alpha fetoprotein were at 2100 Ugr/l and 2500 Ugr/l, respectively. The computerized tomography showed a mass with a low density area in the left temporoparietal lobe and the carotid angiogram a highly hyper-vascular tumor. The child underwent a left temporo-parietal craniotomy and the friable vascular tumor was totally removed. A radiotherapy was associated to the surgical treatment. Histologically, glomerular like structures (Shiller Duval body), intra and extra cellular hyaline globules PAS positive were frequently seen. The tumor marker was demonstrated immunohistochemically.

Xanthogranulomatous pyelonephritis in children. Report of 7 cases and one associated with nephroblastoma.

8 cases of XGP in children are reported. The presence of urolithiasis together with xanthogranulomatous lesion in 7 cases and of nephroblastoma in one may be of help in understanding the aetiopathogenesis. In all the cases the involved kidney did not function thus necessitating subcapsular nephrectomy via retroperitoneal approach.

Abdominal Burkitt-type lymphomas in Algeria.

In a previous retrospective analysis from the principal paediatric centres of Algeria, Burkitt-type lymphomas (BL) were shown to account for around 46.5% of the total childhood non-Hodgkin's malignant lymphomas in that country. In the present study, a series of 49 abdominal BL from the Paediatric Clinic of Surgery, Mustapha Hospital, Algiers, has been studied. The age distribution shows a peak between 4 and 5 years of age, and the sex ratio is (M:F) 2.26:1. The disease is characterized by a rapid evolution in the absence of therapy. The major problem is an explosive form of the disease, which at present seems difficult to control in this country. Fifteen of the 49 patients (30.6%) died before completion of the first course of chemotherapy; however, complete remission (CR) was obtained for 30 patients (61%). Overall survival was 42.85% (21/49), whereas survival of patients who reached CR is 70% (21/30). When CR was obtained, deaths were related to cerebrospinal fluid involvement, local recurrence, secondary bone marrow involvement or therapeutic accidents. All patients alive with no evidence of disease (NED) 8-months after CR can be considered definitively cured. Epstein-Barr virus (EBV) serology performed on 31 BL patients and on a control group of 25 children with other malignant tumours showed that most Algerian BL have elevated EBV titres. A search for viral markers within malignant cells in 17 patients indicated that 88% (15/17) of the BL cases were EBV-associated. Analysis of the immunological and cytogenetic data showed that, as in the rest of the world, these BL cases involve proliferation of B-cell-type lymphocytes, with characteristic cytogenetic translocations involving chromosome 8. This report represents the most detailed description so far of BL from an area in non-equatorial Africa and the first report of a large series from North Africa.