Hamartomatous polyps of the colon: ganglioneuromatous, stromal, and lipomatous.

Archives of Pathology & Laboratory Medicine
Owen T M Chan, Parviz Haghighi

Abstract

Intestinal ganglioneuromas comprise benign, hamartomatous polyps characterized by an overgrowth of nerve ganglion cells, nerve fibers, and supporting cells in the gastrointestinal tract. This polyposis has been divided into 3 subgroups, each with a different degree of ganglioneuroma formation: polypoid ganglioneuroma, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. The ganglioneuromatous polyposis subgroup is not known to coexist with systemic disorders that often have an associated intestinal polyposis, such as multiple endocrine neoplasia type IIb, neurofibromatosis type I, and Cowden syndrome. We report a case of ganglioneuromatous polyposis plus cutaneous lipomatosis in a 41-year-old man with no established systemic disease. However, he possessed unique anatomic findings in addition to his ganglioneuromatosis, suggesting that the ganglioneuromatosis-lipomatosis in our patient may represent an unrecognized syndrome. This case report and brief review of the literature provide an overview of intestinal ganglioneuromatosis in relation to the hereditary polyposis syndromes and describe the individual ganglioneuromatosis subgroups.

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Citations

May 1, 2008·Diagnostic Pathology·Isabel Maria MendezAndres Sanchez
Jan 1, 2011·BMJ Case Reports·Zubair AHmadAsim Qureshi
May 12, 2016·Hereditary Cancer in Clinical Practice·Steffen PistoriusHans K Schackert
Oct 17, 2017·Case Reports in Gastroenterology·Emmanuel OforiMadhavi Reddy
Apr 1, 2021·Italian Journal of Pediatrics·Angela MauroGiovanni Di Nardo
Feb 8, 2020·International Journal of Surgical Pathology·Hirotsugu HashimotoTeppei Morikawa
Mar 22, 2013·International Journal of Surgical Pathology·Sharmeen MansoorHani El-Fanek

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