Colonic Ganglioneuromatosis in a Horse

Department of Pathobiology, College of Veterinary Medicine and Biomedical Sciences, Texas A&M University, College Station, TX 77843-4467, USA.
Veterinary Pathology (Impact Factor: 1.87). 04/2007; 44(2):207-10. DOI: 10.1354/vp.44-2-207
Source: PubMed


Ganglioneuromas are complex tumors that arise in peripheral ganglia and are composed of well-differentiated neurons, nerve processes, Schwann cells, and enteric glial cells. The term ganglioneuromatosis (GN) denotes a regional or segmental proliferation of ganglioneuromatous tissue. This report describes an 8-year-old mixed breed horse with GN in a 25-cm segment of small colon. Grossly, the lesion consisted of numerous sessile to pedunculated nodules extending from the serosal surface. Histologic examination revealed the nodules to consist of fascicles of spindle-shaped cells consistent with Schwann cells, clusters of neurons, supporting enteric glial cells, and thick bands of perineurial collagen. Most of the nodules coincided with the location of the myenteric plexus and extended through the outer layer of the tunica muscularis to the serosal surface. Neuronal processes were demonstrated within the lesion with electron microscopy. With immunohistochemistry neurons were positive for neuron specific enolase (NSE) and S-100 and the Schwann cells and enteric glial cells were positive for S-100 and glial fibrillary acidic protein (GFAP). The pathogenesis of GN is poorly understood. GN, although rare, should be included in the differential diagnosis of gastrointestinal tumors in the horse.

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Available from: Harold Ross Payne, Dec 20, 2014
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    • "Both conditions are composed of well-differentiated ganglion cells, nerve fibres, Schwann cells and enteric glial cells (Head et al., 2002; Porter et al., 2007; Hazell et al., 2011). Single cases of GNM have been reported in a dog (Hazell et al., 2011; Paris et al., 2013), a steer (Cole et al., 1990) and a horse (Porter et al., 2007). The purpose of this report is to describe the first case of intestinal GNM in a piglet. "
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    ABSTRACT: Ganglioneuromatosis (GNM) is a rare condition characterized by the benign proliferation of ganglion cells, nerve fibres and supporting cells of the enteric nervous system. Necropsy examination of a female piglet weighing 4 kg revealed a well-demarcated 20 cm segment of terminal ileum with thickening of the wall. Microscopically, the lamina propria was infiltrated by enteric glial cells and large ganglion cells. Within the submucosal and muscular layers, aggregates of neurons were interlaced by Schwann cells and enteric glial cells arranged in concentric rings. Immunohistochemically, the neurons were weakly labelled for S-100 and neuron-specific enolase, Schwann cells expressed S-100 and vimentin and enteric glial cells expressed glial fibrillary acidic protein and S-100. Pathological and immunohistochemical findings supported the diagnosis of ileal GNM. Copyright © 2014 Elsevier Ltd. All rights reserved.
    Full-text · Article · Nov 2014 · Journal of Comparative Pathology
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    • "We found GFAP-positive neuropil-like elements resembling cells of the neurohypophysis located at the edge of the tumor mass. Schwann cells, satellite cells, and glial cells of ganglioneuromas have been demonstrated to express GFAP in human and animal cases (Pace et al. 2002; Porter et al. 2007; Scheithauer et al. 1997). Glial fibrillary acidic proteinpositive neuropil-like elements in the present case may have represented one of the components of the tumor tissue rather than of the neurohypophysis. "
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    ABSTRACT: In a carcinogenicity study, a neuronal tumor in the cranial cavity was observed in a 110-week-old female B6C3F1 mouse. At necropsy, the tumor was seen at the site of the pituitary gland. Histologically, the tumor consisted of well-differentiated ganglion cells, nerve fiber/neuropil-like elements and ganglion-like cells. The tumor was composed mainly of ganglion-like cells, which were arranged in solid sheets interspersed with thin fibrovascular stroma. Nissl substance was detected at the margin in the cytoplasm of well-differentiated ganglion cells, and nerve fibers were identified by the Kluever-Barrera method. Immunohistochemically, the well-differentiated ganglion cells were positive for S-100, neurofilament protein (NF), neuron-specific enolase (NSE), synaptophysin, and chromogranin A. The nerve fiber/neuropil-like elements were positive for S-100, NF, NSE, and glial fibrillary acidic protein (GFAP), and the ganglion-like cells were strongly positive only for NSE and synaptophysin. On the other hand, there were no pituitary cells, such as prolactin-positive or adrenocorticotropic hormone (ACTH)-positive cells in the tumor tissue. Detailed histopathological examination suggested that the tumor might be a ganglioneuroma arising from the trigeminal ganglion. This report provides additional histopathological evidence of peripheral nerve neoplasms in mice.
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