Skip Navigation LinksHome > December 2011 - Volume 53 - Issue 6 > Hamartomatous Small Intestinal Polyp From Tuberous Sclerosis
Text sizing:
A
A
A
Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e318225f56e
Image of the Month

Hamartomatous Small Intestinal Polyp From Tuberous Sclerosis

Goldberg, Eric

Free Access
Article Outline
Collapse Box

Author Information

Division of Gastroenterology and Hepatology, University of Maryland School of Medicine, Baltimore, MD

Address correspondence and reprint requests to Eric Goldberg, MD, Division of Gastroenterology and Hepatology, University of Maryland School of Medicine, 22 S Greene St, N3W62, Baltimore, MD 21201 (e-mail: egoldber@medicine.umaryland.edu).

The author provides consulting services for Olympus America Inc, in the field of single-balloon enteroscopy.

Submissions for the Image of the Month should include high-quality TIF endoscopic images of unusual or informative findings. In addition, 1 or 2 other associated photographs, such as radiological or pathological images, can be submitted. A brief description of no more than 200 words should accompany the images. Submissions are to be made online at www.jpgn.org, and will undergo peer review by members of the NASPGHAN Endoscopy and Procedures Committee, as well as by the Journal.

A 22-year-old woman presented with intermittent left lower quadrant abdominal pain for 6 months. She was diagnosed as having had tuberous sclerosis (TS) during childhood based upon characteristic skin findings and recurrent seizures.

An abdominal computed tomography scan and esophagogastroduodenoscopy were unremarkable. Colonoscopy revealed 2 diminutive rectal polyps. Pathology revealed smooth muscle proliferation consistent with hamartomatous polyps. Capsule endoscopy revealed a large pedunculated multilobular polyp in the mid-jejunum (Fig. 1). A single-balloon enteroscopy identified a 3-cm pedunculated polyp (Figs. 2 and 3) in the jejunum, which was removed with a hot snare. Pathology revealed smooth muscle and vascular proliferation. Stains for angiomyolipoma (HMB-45) were negative and the polyp was determined to be a hamartoma (Fig. 4). The patient has remained asymptomatic postpolypectomy.

Figure 1
Figure 1
Image Tools
Figure 2
Figure 2
Image Tools
Figure 3
Figure 3
Image Tools
Figure 4
Figure 4
Image Tools

The most common gastrointestinal manifestation of TS is hamartomatous polyps of the rectosigmoid (1,2). In 1 report, 11 of 15 patients had hamartomatous rectal polyps (3). In another report, 5 of 12 patients had hamartomas (4). Although symptomatic angiomyolipomas of the small intestine have been reported in TS, there are no case reports documenting symptomatic small-bowel involvement by nonangiomyolipoma hamartomas (5–8). This is the first report of a symptomatic, partially obstructing, nonangiomyolipoma hamartoma of the small intestine from TS.

Back to Top | Article Outline

REFERENCES

1. Orlova KA, Crino PB. The tuberous sclerosis complex. Ann N Y Acad Sci 2010; 1184:87–105.

2. Gould SR. Hamartomatous rectal polyps are common in tuberous sclerosis. Ann N Y Acad Sci 1991; 615:71–80.

3. Gould SR, Stewart JB, Temple LN. Rectal polyposis in tuberous sclerosis. J Ment Defic Res 1990; 34:465–473.

4. Devroede G, Lemieux B, Masse S, et al. Colonic hamartomas in tuberous sclerosis. Gastroenterology 1988; 94:182–188.

5. Umeoka S, Koyama T, Miki Y, et al. Pictorial review of tuberous sclerosis in various organs. Radiographics 2008; 28:e32.

6. Kim B, Kim Y, Kim W. Hamartomatous gastric polyposis in a patient with tuberous sclerosis. J Korean Med Sci 2000; 15:467–470.

7. Lee CH, Kim JH, Yang DH, et al. Ileal angiomyolipoma manifested by small intestinal intussusception. World J Gastroenterol 2009; 15:1398–1400.

8. De Padua M, Gupta N, Broor SL, et al. Duodenal angiomyolipoma: a case report. Indian J Pathol Microbiol 2007; 50:568–569.

Copyright 2011 by ESPGHAN and NASPGHAN

Login

Article Tools

Images

Share

Connect With Us

 

 

Twitter

twitter.com/JPGNonline

 

Visit JPGN.org on your smartphone. Scan this code (QR reader app required) with your phone and be taken directly to the site.