Diffuse ganglioneuromatosis in small intestine associated with neurofibromatosis type 1
Royal Marsden Hospital, London, United Kingdom.Annals of diagnostic pathology (Impact Factor: 1.12). 03/2009; 13(1):50-4. DOI: 10.1016/j.anndiagpath.2007.06.001
Ganglioneuromatosis of the gastrointestinal tract is a rare condition that has an established association with the multiple endocrine neoplasia syndrome, type IIb. Its occurrence in neurofibromatosis, in which the large intestine is usually affected, is much rarer. We report a case of diffuse ganglioneuromatosis of the small bowel, found incidentally during surgery for a malignant peripheral nerve sheath tumor arising in the retroperitoneum in a 32-year-old man with neurofibromatosis type 1, and review previously reported cases.
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ABSTRACT: We report a case of intra-abdominal plexiform neurofibromatosis, including periportal, mesenteric, and gastrointestinal tract involvement, in a patient with von Recklinghausen’s disease/neurofibromatosis type 1 (NF-1). A 26-year-old man with familial NF-1 was admitted to hospital for further examination of an abnormal hepatic mass along the portal vein. Esophagogastroduodenoscopy revealed antral wall thickening and swelling of the papilla of Vater. Mucosal biopsies taken from the duodenum revealed possible ganglioneuromatosis. Abdominal ultrasonography, contrast-enhanced computed tomography, and magnetic resonance imaging revealed an abnormal periportal mass with serpiginous extension into the liver along the portal vein and the mesentery, which is the typical spread pattern of plexiform neurofibromatosis. A laparotomy and cholecystectomy for gallstones were performed, and this patient was diagnosed as having intra-abdominal plexiform neurofibromatosis. This is the 15th case of intrahepatic periportal plexiform neurofibromatosis and the 16th case of diffuse ganglioneuromatosis associated with NF-1 in the English literature. The imaging findings of the lesion have been followed for 10years; there has been slight growth of the mass, but no malignant transformation has been found. The previously reported cases are reviewed. KeywordsNeurofibromatosis type 1 (von Recklinghausen’s disease)–Intestinal diffuse ganglioneuromatosis–Intrahepatic periportal plexiform neurofibromatosis
Conference Paper: GPR survey in the Taquari River, Pantanal Wetland, west-central Brazil[Show abstract] [Hide abstract]
ABSTRACT: A pioneer GPR - Ground Penetrating Radar - survey was carried out in the Pantanal of Mato Grosso State, westcentral region of Brazil. Fieldwork acquisitions were carried out in February/2001 and August/2002 in order to understand avulsion processes that are occurring within the Taquari alluvial megafan. The main subjects were to map channel, crevasses and floodplain morphology, as well as active sedimentary bedforms. Many GPR profiles were surveyed in the medium and lower Taquari River course. Subaqueous megaripples and exposed sand bars inside the Taquari channel were identified in the medium fan area. Similar features were observed in the lower fan channels, where there are also many crevasses in the marginal levees. During the flooding seasons the flow splays out in the floodplain where new distributary channels are being formed. As shown by GPR data, in the lower fan the Taquari channel is topographically higher than the adjacent floodplain, situation in which avulsion is a natural process of river course shifting. The lack of information about river morphology and dynamics is a major strain to better understand the sediment transport and the avulsion processes in the Taquari megafan. In this context, the GPR data obtained in wet and dry seasons, integrated to sedimentological information, have been very important to characterize the fluvial dynamics and the avulsion phenomena.
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