Abstract
Intestinal ganglioneuromatosis are rare benign tumour of ganglionic cell origin, most of them associated with neurofi bromatosis type I and multiple endocrine neoplasia type IIB. They are benign tumours with rare malignant transformation, have a varied clinical presentation and long indolent course. Surgical resection is the only modality of treatment and diagnosis is based on histopathological examination and immunohistochemistry. We report a case, 55 year old male who was diagnosed as a case of isolated intestinal ganglioneuromatosis, i.e no association with neurofibromatosis type I and multiple endocrine neoplasia type IIB. He is on regular follow up with normal daily activities and no evidence of recurrence.Journal of Kathmandu Medical College, Vol. 5, No. 4, Issue 18, Oct.-Dec., 2016, Page: 131-135
Highlights
Ganglioneuromas are well differentiated tumours, arising from ganglion cells, nerve fibres and supporting cells
Ganglioneuromatous polyposis has been found to be associated with neurofibromatosis type I (NF I), multiple endocrine neoplasia type IIB (MEN IIB), Cowden syndrome and even as isolated lesions[2,3]
Sections from intestine revealed prominent proliferation of nerve fibres with ganglion cells in submucosa that extended throughout the muscular layer into subserosa. (Figure 4 and 5) Sections from mesenteric nodules showed well circumscribed masses with proliferation of Figure 4: 40X H&E Nerve fibres and ganglion cells
Summary
Ganglioneuromas are well differentiated tumours, arising from ganglion cells, nerve fibres and supporting cells. Ganglioneuromatous polyposis has been found to be associated with neurofibromatosis type I (NF I), multiple endocrine neoplasia type IIB (MEN IIB), Cowden syndrome and even as isolated lesions[2,3] These have a long course, varied clinical presentation and none of the radiological investigations are conclusive. A 55 year old male presented with complaints of pain in right lower abdomen for 10 years, weight loss of 10 kg in last 4 months, constipation, melena on and off for 2 years, and his symptoms were increasing in severity in last 4 months He is a smoker, consumes Gutkha, and is an occasional alcohol consumer, has no similar illness in family. Extra 10 cm of distal ileum was removed which was involved by disease
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