Solitary colonic polypoid ganglioneuroma
© Mendez et al; licensee BioMed Central Ltd. 2008
Received: 17 January 2008
Accepted: 29 April 2008
Published: 29 April 2008
This short report discusses a case of solitary colonic polypoid ganglioneuroma associated with melanosis coli in a woman with no systemic manifestations. To our knowledge this is the first ganglioneuroma reported in the literature in association with melanosis coli. The nature and significance of this event remains unclear, although this may be coincidental due to the laxative intake. Further investigation is necessary to clarify this point. The interest of this case lies moreover in the rarity of this entity and its endoscopic and histologic resemblance to sessile polyps frequent in the clinical practice.
The hereditary syndromes of the gastrointestinal tract are classified as adenomatous or hamartomatous . As a part of the hamartomatous polyposes, ganglioneuroma (GN) of the gastrointestinal tract are rare tumors composed of ganglion cells, nerve fibers, and supporting cells of the enteric nervous system [1, 2]. There are few reports in the literature. We present a case of polypoid GN and melanosis coli. To our knowledge this is the first case reported in literature with this association.
The diagnosis was solitary polypoid ganglioneuroma of the colon.
Shekita et al. categorized ganglioneuromas (GN) of the intestinal tract into three groups: polypoid GN, ganglioneuromatous polyposis and diffuse ganglioneuromatosis.
The GN of the gastrointestinal tract have been found in patients with familial adenomatosis coli, Cowden's disease, tuberous sclerosis, juvenile polyposis, von Recklinghausen's disease and multiple endocrine neoplasia type II . The series by Shekita indicate that a solitary polypoid GN is not a sign for increased risk of Recklinghausen's disease or Multiple endocrine neoplasia (MEN) type II, but it has been reported in a few cases of Cowden's disease, tuberous sclerosis, polyposis coli and juvenile polyposis. In contrast, diffuse GN appears to be a strong factor for extensive neurogenic disease. In our case ganglioneuromas occurred without evidence of systemic disease.
Polypoid GN are small, sessile or pedunculated polyps with a histologic resemblance to hyperplastic polyps, juvenile polyps or adenomas [1, 3]. In the intestinal tract, these lesions are solitary or few in number . In the cases by Shekita et al, most of them were located in the large intestine . Polypoid GN produces no characteristic symptoms and may be noted incidentally during endoscopy, surgery or autopsy. Abdominal pain, obstruction, constipation, ileus, appendicitis and weight loss have been listed as potential complications of the lesion, and all are related to their size and anatomical location .
The link between intestinal ganglioneuromatosis and malignancy is not well established, although some investigators have described the coexistence of intestinal ganglioneuromatosis and colorectal adenocarcinoma [5, 6].
To our knowledge this is the first ganglioneuroma reported in the literature in association with melanosis coli. The nature and significance of this event remains unclear although this may be coincidental due to the laxative intake. Further investigation is necessary to clarify this point. The interest of this case lies moreover in the rarity of this entity and its endoscopic and histologic resemblance to sessile polyps frequent in the clinical practice.
Multiple endocrine neoplasia.
We thank to the Library Service of the Hospital Costa del Sol for his help in the acquisition of data and funding of this work.
We have obtained consent for publication from the patient.
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