The polypoid ganglioneuroma associated with hyperplastic polyposis
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Ganglioneuroma (GN) of the gastrointestinal (GI) tract is rare tumor composed of ganglion cells, nerve fibers, and supporting cells of the enteric nervous system. Diffuse ganglioneuromatosis is associated with multiple endocrine neoplasia IIB (MEN IIB), neurofibromatosis type I (NF1, also known as von Recklinghausen disease), multiple cutaneous or GI tract neurofibromatosis, and neurogenic sarcoma of the GI tract [1]. However, usual presentation of GN is small mucosal polyp < 1 to 2 cm, or sometimes multiple polyps. Few cases of solitary polypoid GN were reported in the literature, and colonic mucosal GN associated with colonic polyps is a very unusual finding. We report here a case of polypoid GN associated with hyperplastic polyposis and discuss the important pathological features and clinical issues.
Abstract
Ganglioneuroma (GN) of the gastrointestinal (GI) tract is rare tumor composed of ganglion cells, nerve fibers, and supporting cells of the enteric nervous system. Diffuse ganglioneuromatosis is associated with multiple endocrine neoplasia IIB (MEN IIB), neurofibromatosis type I (NF1, also known as von Recklinghausen disease), multiple cutaneous or GI tract neurofibromatosis, and neurogenic sarcoma of the GI tract [1]. However, usual presentation of GN is small mucosal polyp < 1 to 2 cm, or sometimes multiple polyps. Few cases of solitary polypoid GN were reported in the literature, and colonic mucosal GN associated with colonic polyps is a very unusual finding. We report here a case of polypoid GN associated with hyperplastic polyposis and discuss the important pathological features and clinical issues.
참고문헌 (4)
- Shekitka KM, "Ganglioneuromas of the gastrointestinal tract. Relation to Von Recklinghausen disease and other multiple tumor syndromes", Am J Surg Pathol, 18, pp.250~257 (1994)
- Carney JA, "Alimentary-tract ganglioneuromatosis: a major component of the syndrome of multiple endocrine neoplasia, type 2b", N Engl J Med, 295, pp.1287~1291 (1976)
- Hochberg FH, "Gastrointestinal involvement in von Recklinghausen’s neurofibromatosis", Neurology, 24, pp.1144~1151 (1974)
- Weidner N, "Mucosal ganglioneuromatosis associated with multiple colonic polyps", Am J Surg Pathol, 8, pp.779~786 (1984)