Evidence of multicentric origin of the multiple endocrine neoplasia syndrome type 2A (Sipple's syndrome) in a large family in the Netherlands. Diagnostic and therapeutic implications

1978 ◽  
Vol 64 (4) ◽  
pp. A79
Surgery ◽  
2020 ◽  
Author(s):  
Constantinos Nastos ◽  
Dimitrios Papaconstantinou ◽  
Efstratios Kofopoulos-Lymperis ◽  
Melpomeni Peppa ◽  
Andreas Pikoulis ◽  
...  

2020 ◽  
Vol 50 (2) ◽  
Author(s):  
Marcos Leites ◽  
Alejandra Arriola ◽  
Yéssica Pontét ◽  
Iván Trochansky ◽  
Marta Otero ◽  
...  

Intestinal ganglioneuromatosis is an unusual pathology, especially in adults. It is associated with multiple endocrine neoplasia type IIb and Von Recklinghausen´s neurofibromatosis. Histologically, it is characterized by hyperplasia of the intramural plexuses of the gastrointestinal tract and enteric nerve fibres. Clinical manifestations include abdominal pain, distension and changes in bowel habits. We report the case of a 38-year-old patient, with a multiple endocrine neoplasia syndrome type IIb, presenting with repeated partial or complete intestinal obstruction that requires partial colectomy. Pathology of the resected colon revealed intestinal ganglioneuromatosis.


Sign in / Sign up

Export Citation Format

Share Document