What Do We Need to Know About Colonic Polypoid Ganglioneuroma? A Case Report and A Comprehensive Review

Abdelfatah, Mohamed M.; Sangah, George; Harvin, Glenn

doi:10.1007/s12029-016-9892-7

Cited by 3 publications

(6 citation statements)

References 31 publications

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“…Polypoid ganglioneuroma has been reported to have a good prognosis with no malignant potential. 7 However, some studies have reported that ganglioneuromatous polyposis and diffuse ganglioneuromatosis might be accompanied by adenomatous polyps or colorectal cancer, although the incidence is rare. The first case of cecal adenocarcinoma surrounded by diffuse ganglioneuromatosis in a 54-year-old male patient was reported by Snover et al 8 in 1981.…”

Section: Discussionmentioning

confidence: 99%

Development of colon cancer in a patient with longstanding colonic diffuse ganglioneuromatosis: a case report

Oh¹,

Hong

Noh

et al. 2022

Clin Endosc

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Colonic diffuse ganglioneuromatosis is an extremely rare disease in which multiple tumors derived from the ganglion cells, nerve fibers, and supporting cells are distributed in the colon. It is generally considered to be a benign neoplastic condition and is occasionally associated with rare hereditary conditions such as neurofibromatosis type I or multiple endocrine neoplasia type 2B. Here, we report a case of a patient in whom colon cancer developed 12 years after the initial diagnosis of colonic diffuse ganglioneuromatosis, which suggests a possible association between colonic diffuse ganglioneuromatosis and colorectal cancer.

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Section: Discussionmentioning

confidence: 99%

Development of colon cancer in a patient with longstanding colonic diffuse ganglioneuromatosis: a case report

Oh¹,

Hong

Noh

et al. 2022

Clin Endosc

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“…Gastrointestinal involvement is often incidentally recognized. Manifestations of obstruction, constipation, abdominal pain, diarrhea, and melena have been associated (1,3,4). An intestinal ganglioneuroma has also presented as the lead point in pediatric intussusception (2).…”

Section: Figurementioning

confidence: 99%

“…GP is defined by greater than 20 polypoid lesions. GP has been described in association with Cowden syndrome, neurofibromatosis 1, and other cutaneous stigmata (1–3). Diffuse intestinal ganglioneuromatosis (DIG) denotes intramural or transmural involvement, with proliferation of submucosal and myenteric nerve plexus elements.…”

Section: Figurementioning

confidence: 99%

“…Intestinal ganglioneuromas are classified into 3 histopathologic forms (1). Polypoid ganglioneuromas are the most common subtype, are single or few in number, and arise in mucosa and/or submucosa (1,3). GP is defined by greater than 20 polypoid lesions.…”

Section: Figurementioning

confidence: 99%

“…Diffuse intestinal ganglioneuromatosis (DIG) denotes intramural or transmural involvement, with proliferation of submucosal and myenteric nerve plexus elements. This has a closer association with multiple endocrine neoplasia type 2B and the rearranged during transfection proto-oncogene mutation (1,3). Genetic screening is a necessary part of the workup in GP, DIG, or in children with a single lesion and an index of suspicion (4,5).…”

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confidence: 99%

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Pediatric Hematochezia Caused by Intestinal Ganglioneuromatous Polyposis

Eldredge¹,

Manton²,

Abu‐Assi³

2022

Journal of Pediatric Gastroenterology &Amp; Nutrition

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This two-day-old term, female infant was born via uncomplicated vaginal delivery following an uneventful pregnancy. On the first day of life, the infant was noted to have pink, raised, perianal softtissue protuberances, larger while crying but spontaneously reducing when calm (Fig. 1).Anorectal disorders in children include neonatal etiologies, mostly anorectal malformations and infantile/childhood causes such as anal fissures and hemorrhoids (1). Our team suspected neonatal hemorrhoids, which can be manifested as an isolated clinical problem or can coexist with more complex anorectal anomalies (2,3).Neonatal hemorrhoids can be caused by compression of the abdominal vasculature from anatomic obstructions as can be seen with malignancy or embryologic abnormality. Similarly, impaired venous return from prolonged pressure on the newborn's abdominal cavity during delivery or portal hypertension from portal vein thrombosis following umbilical vein catheterization can result in neonatal hemorrhoids (4). A rectal polyp can masquerade as a hemorrhoid (5). Chronic liver disease is associated with hemorrhoids in up to 35% of children with portal hypertension; however, there are no reported cases of this etiology in neonates ( 6).An ultrasound of the abdomen and pelvis revealed normal hepatic and genitourinary anatomy (7). Doppler waveforms demonstrated hepatopedal flow in the portal vein. A newborn screen was normal.The protuberances self-resolved by 2 weeks of life. Since the infant was thriving, had normal imaging, and had hemorrhoid resolution, no serious underlying pathology was suspected. Neonatal hemorrhoids are a rare and often benign finding that warrants imaging to exclude underlying anatomic anomalies or portal hypertension and close follow-up.

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Colonic Ganglioneuroma: A Combined Single-Institution Experience and Review of the Literature of Forty-Three Patients

et al. 2023

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Ganglioneuromas (GNs) are rare, benign tumors composed of ganglion cells, nerve fibers, and glial cells. Three types of colonic GN lesions exist: polypoid GNs, ganglioneuromatous polyposis, and diffuse ganglioneuromatosis. Less than 100 cases of GN are documented in the literature. A 10-year retrospective search of the pathology database at our institution identified eight cases of colonic GNs. All cases were incidental. Seven of the eight cases presented with colonoscopy findings of small sessile polyps (ranging between 0.1 and 0.7 cm) treated with polypectomy, whereas one case showed a 4 cm partially circumferential and partially obstructing mass in the ascending colon, treated with right hemicolectomy. Almost two-thirds of the cases (5/8) demonstrated associated diverticulosis. All cases were positive for S100 protein and Synaptophysin via immunohistochemistry (IHC). No syndromic association was identified in any of the cases. We also conducted a comprehensive review using PubMed to identify cases of colonic GN reported in the literature. In total, 173 studies were retrieved, among which 36 articles met our inclusion criteria (35 patients and 3 cases on animals). We conclude that while most GNs are incidental and solitary small sessile lesions, many can be diffuse and associated with syndromes. In these cases, the tumor can result in bowel obstruction simulating adenocarcinoma.

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What Do We Need to Know About Colonic Polypoid Ganglioneuroma? A Case Report and A Comprehensive Review

Cited by 3 publications

References 31 publications

Development of colon cancer in a patient with longstanding colonic diffuse ganglioneuromatosis: a case report

Development of colon cancer in a patient with longstanding colonic diffuse ganglioneuromatosis: a case report

Pediatric Hematochezia Caused by Intestinal Ganglioneuromatous Polyposis

Colonic Ganglioneuroma: A Combined Single-Institution Experience and Review of the Literature of Forty-Three Patients

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