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Intestinal ganglioneuromatosis: mucosal and transmural types. A clinicopathologic and immunohistochemical study of six cases.
Hum Pathol
March 1991
Department of Laboratory Medicine and Pathology, University of Minnesota Hospital, Minneapolis.
Six cases of intestinal ganglioneuromatosis (GN) included in this study reveal the occurrence of two morphologic patterns. Transmural GN was characterized by neural hyperplasia in all layers of the bowel wall with predominant involvement of the myenteric plexus. It was found in three patients affected by multiple endocrine neoplasia IIb.
View Article and Find Full Text PDFFamilial occurrence of neuroblastoma, von Recklinghausen's neurofibromatosis, Hirschsprung's agangliosis and jaw-winking syndrome.
Acta Paediatr Scand
September 1989
Department of Paediatrics, University Hospital of Copenhagen, Denmark.
A family is reported with ganglioneuromas in the mother and neuroblastomas in her two daughters co-existing with cases of von Recklinghausen's neurofibromatosis, Hirschsprung's agangliosis, and the jaw-winking syndrome in other family members. There were no detectable constitutional chromosomal defects in the family even when high resolution techniques were applied. Similarly, DNA-hybridization analysis did not reveal gross molecular rearrangements in the vicinity of the proto-oncogenes N-myc-, c-myc, neu, and N-ras.
View Article and Find Full Text PDFVascular dysplasia of arteries in neurocristopathies: a lesson for neurofibromatosis.
Neurofibromatosis
May 1990
Neurological Institute, University of Vienna, Austria.
In a 15-year-old girl suffering from congenital constipation, megacolon combined with a 'Ranken neuroma' of the rectum and a short aganglionic segment of distal colon was observed. The specific vascular alterations in the region of the Ranken neuroma (which has previously been described in cases of von Recklinghausen neurofibromatosis) were studied, with an emphasis on immunohistochemical methods. The results suggest that the pericytes are the cells primarily involved in the distinctive alterations of the blood vessels.
View Article and Find Full Text PDFGastrointestinal manifestations of neurofibromatosis in children: a report of two cases.
J Can Assoc Radiol
September 1982
Two patients with von Recklinghausen's disease (neurofibromatosis) involving the gastrointestinal tract are described. The manifestations of the disease were very different in each patient. In the first, the similarity of the diffuse abdominal form of plexiform neurofibromatosis to childhood sarcoma is emphasized.
View Article and Find Full Text PDFPrimary myenteric plexus alterations as a cause of megacolon in Von Recklinghausen's disease.
Pathol Biol (Paris)
November 1980
This is a case report of a megacolon in a 26 years old male patient having typical von Recklinghausen's disease. Exploratory laparotomy shows no neurofibroma on digestive tract. Myenteric plexus study of colic resection specimen following B.
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