Abstract
Ganglioneuromatous proliferation in the gastrointestinal tract is a rare occurrence and is usually associated with specific syndrome complexes such as multiple endocrine neoplasia Type 2B or von Recklinghausen's disease. We report here a case of diffuse intestinal ganglioneuromatosis, presenting as intestinal obstruction and chronic constipation in an 11-year-old boy. Sporadic cases of intestinal ganglioneuromatosis in the absence of any systemic manifestations are a very rare cause of enteric motility disorders in childhood, and we discuss the pathological and clinical significance of this finding. Histopathological identification of this uncommon cause of a common pediatric problem is important since the condition is amenable to surgical treatment.
MeSH terms
-
Biomarkers, Tumor / analysis
-
Child
-
Constipation / etiology
-
Digestive System Neoplasms / diagnosis*
-
Digestive System Neoplasms / diagnostic imaging
-
Digestive System Neoplasms / pathology*
-
Ganglioneuroma / diagnosis*
-
Ganglioneuroma / diagnostic imaging
-
Ganglioneuroma / pathology*
-
Histocytochemistry
-
Humans
-
Ileal Neoplasms / diagnosis*
-
Ileal Neoplasms / diagnostic imaging
-
Ileal Neoplasms / pathology*
-
Immunohistochemistry
-
Intestinal Obstruction / etiology
-
Intestinal Obstruction / pathology
-
Male
-
Microscopy
-
Multiple Endocrine Neoplasia Type 2b / diagnosis*
-
Multiple Endocrine Neoplasia Type 2b / diagnostic imaging
-
Multiple Endocrine Neoplasia Type 2b / pathology*
-
Radiography, Abdominal
-
S100 Proteins / analysis
-
Tomography, X-Ray Computed
Substances
-
Biomarkers, Tumor
-
S100 Proteins
Supplementary concepts
-
Ganglioneuromatosis of the Alimentary Tract