[Anatomo-clinical study of elastic dystrophy in so-called idiopathic pulmonary hemosiderosis].
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Biomedical subjects
Publications and source records attributed to J Floquet.
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We report two cases of aggressive fibromatosis of the mediastinum with esophageal involvement in adults. CT showed the posterior mediastinal mass involving the esophagus. In both cases, surgical resection was not feasible. Histopathologic examination showed a fibrous tissue. The patients died two years later.
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A clear cell rhabdomyosarcoma was studied by light microscopy, histochemistry, immunohistochemistry, and electron microscopy. It was a large, painful left parapharyngeal mass in a 10-year-old boy with intracranial extension and cervical metastatic enlarged lymph nodes. Tumor tissue was macroscopically grayish. At microscopic examination, the architecture was diffuse and focally alveolar. Tumor cells were of three types. Most cells were large, round or polygonal, with abundant clear vacuolated cytoplasm. Fibrils were sometimes found to be present around the nucleus. Nuclei often had irregular outlines and multiple nucleoli. Mitotic activity was high. Some round or elongated cells had eosinophilic fibrillar cytoplasm and were found to have a few double striations. A few cells were round and medium sized with a high nucleocytoplasmic ratio. Periodic acid-Schiff stain demonstrated huge amounts of intracytoplasmic glycogen in clear cells. Tumor cells showed positive immunostaining for muscle markers (desmin, muscle actins, dystrophin). Electron microscopy showed large lakes of glycogen, lipid droplets, and striated muscle features.
A case report of endocrine pancreatic tumour associated with diabetes and hyperparathyroidism is presented. Some arguments are in favour of a glucagonoma. Despite the absence of beta-cells in tumour, an exceptional result was obtained by use of Streptozotocin. This case brings up the relations between endocrine pancreatic tumours and parathyroid, and may be included in polyglandular adenoma syndrome.
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Report of a child with disseminated ganglioneuromatosis of the gut. The complexity of the intestinal nervous system malformation is proved by histochemical, histoenzymological and ultrastructural studies. The malformation is characterized by: hyperplasia and hypertrophy of enteric plexus and nerves bundles in the meso, high acetylcholinesterase activity, aplasia of the sympathetic innervation with the exception of perivascular plexus, qualitative and likely quantitative integrity of the endocrine digestive system. These data are compared with similar observations in the literature.