Pheochromocytoma and paraganglioma are rare in the pediatric population occurring in approximately 1 in 50,000 children. While some cases are sporadic, they have commonly been associated with syndromes such as von Hippel-Lindau, multiple endocrine neoplasia types IIa and IIb, neurofibromatosis type 1, and hereditary pheochromocytoma-paraganglioma syndromes. In children less than 18 years of age approximately 60% of pheochromocytomas and paragangliomas are associated with a germline mutation. We present an 11-year-old child with an abdominal paraganglioma related to a succinate dehydrogenase subunit B gene mutation whose father had a previously resected abdominal paraganglioma and was found to carry the same mutation. In addition, we review the etiology, genetics, diagnostic approach, and challenges of preoperative management of secretory pheochromocytomas and paragangliomas in children.
Four patients are described in whom pneumoconiosis was diagnosed towards the end of a lifetime's work in shale mines. All developed complicated pneumoconiosis, diagnosed in two cases at necropsy, in one by lobectomy, and in one radiologically. Two ofthe patients were found at necropsy also to have peripheral squamous lung cancer. The clinical and histological features of the disease resembled the pneumoconioses of coalminers and kaolin workers and the lungs of three of the patients were shown to contain dust composed predominantly of kaolinite, mica, and silica. Shale
Four men who mined barytes in Scotland and who developed pneumoconiosis are described. Three developed progressive massive fibrosis, from which two died; and one developed a nodular simple pneumoconiosis after leaving the industry. The radiological and pathological features of the men's lungs were those of silicosis and high proportions of quartz were found in two of them post mortem. The quartz was inhaled from rocks associated with the barytes in the mines. The features of silicosis in barium miners are contrasted with the benign pneumoconiosis, baritosis, that occurs in workers exposed to crushed and ground insoluble barium salts. Diagnostic difficulties arise when silicosis develops in workers mining minerals known to cause a separate and benign pneumoconiosis. These difficulties are compounded when, as not infrequently happens, the silicotic lesions develop or progress after exposure to quartz has ceased.
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