Inflammatory pseudotumour of the nasal cavity and sinuses.

The term inflammatory pseudotumour encompasses a very diverse group of conditions, with different sites, whose only common feature is the absence of a specific histological diagnosis. The histopathological and aetiopathogenic data have been derived from experience in the thoracic, orbital and abdominal forms, which are much more frequent than disease affecting the nasal cavities and sinuses. However, there is no evidence that these data are applicable to sino-nasal pseudotumour. We present a case of inflammatory pseudotumour of the nasal cavity and a review of the literature (19 cases in 30 years) with respect to the clinical and radiological findings, the natural history and the specific management of disease at these sites. Surgery seems to be the best option for this localization. Corticosteroids and radiotherapy may be of value in cases of residual tumour after the histological confirmation of the diagnosis and the exclusion of neoplasia.

Malignant lymphoma of mucosa associated lymphoid tissue: a new etiology of amyloidosis.

Non Hodgkin Malignant lymphomas (NHML) of mucosa associated lymphoid tissue (MALT) are known to have multiple involvement of the digestive tract. We report one case, presenting with an infiltrative process of the jejuno-ileum, associating lymphoplasmacytoid proliferating cells and amyloidosis. The plasmacytoid cells expressed Alpha and scarce Mu heavy chains, and lambda light chain. Lympho-epithelial lesions were more obvious at the second site of involvement, in the gastric mucosa. The amyloid substance was negative with the Amyloid A component antibody and gave a background noise with Alpha, Mu and lambda chains. No similar report of amyloidosis associated with MALT NHML has been found in the literature.

[Intraductal mucin-secreting tumors of the pancreas. Nosologic discussion of two cases of villous tumor of Wirsung's duct and a case of mucinous ductal ectasia]. / Les tumeurs mucosécrétantes intracanalaires du pancréas. Discussion nosologique à propos de deux cas de tumeur villeuse du canal de Wirsung et d'un cas d'ectasie canalaire mucineuse.

Intraductal mucin-producing tumors of the pancreas are rare, recently described tumors; they have a better prognosis than usual ductal carcinomas of the pancreas. We report two cases of villous tumor of the main pancreatic duct and one case of mucinous ductal ectasia, with histochemical and immunohistochemical study.

Immunological study in primary intestinal lymphangiectasia.

Primary intestinal lymphangiectasia is a rare congenital condition associated with protein-losing enteropathy. Hypogammaglobulinemia and lymphopenia secondary to this condition are frequent but infectious complications are not. So far few immunological studies have been made in these patients. We report here the results of such a study carried out in two adolescents. Both patients presented with a dramatic decrease in serum gammaglobulins, especially IgG and IgA, and in peripheral blood lymphocytes, especially CD4 T helper cells. From a functional standpoint, the proliferative response to certain mitogens was reduced. A decrease in in vitro production of immunoglobulins by B lymphocytes may be due to a faulty T/B cell cooperation. Histological examination of duodenal biopsy specimens revealed a decreased number of intraepithelial lymphocytes. Colonoscopy revealed nodular lymphoid hyperplasia in the terminal ileum, confirmed by endoscopic biopsy. The role of these abnormalities in the development of infectious complications and lymphoma is underscored.