Benign Neoplasm of the Small Intestine

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Background

Benign tumors of the small bowel are rare clinical entities that often remain asymptomatic throughout life.[1, 2, 3, 4] Despite comprising 75% of the length and 90% of the surface area of the GI tract, the small bowel harbors relatively few primary neoplasms and fewer than 2% of GI malignancies.[5]

Benign small bowel tumors may develop as a single lesion or as multiple lesions of several subtypes. Subtypes include hyperplastic polyps, adenomas, GI stromal tumors, lipomas, hemangiomas, and those associated with Peutz-Jeghers syndrome.[6]

These tumors are generally characterized by slow growth and delayed clinical presentation. They often remain inherently asymptomatic, only to be discovered incidentally at autopsy.

The tumors may be found throughout the duodenum, jejunum, and ileum (in order of increasing frequency). Tumors may be single, multiple, or widespread, that is, as part of a polyposis syndrome. Three growth patterns have been identified, as follows: (1) intraluminal, (2) infiltrative, and (3) serosal. Intraluminal lesions are most often associated with the development of secondary bowel obstruction and intussusception, while serosal lesions are linked to small bowel volvulus.

Several factors have been suggested to explain both the scarcity of small bowel lesions and the infrequency of their malignant transformation.[7] First, rapid intestinal transit through the small bowel limits contact time to the small bowel mucosa. Second, greater fluidity of small bowel chyme may dilute luminal irritants. Third, alkaline pH may play a role, as may the low bacterial colony counts of the small bowel. Finally, higher levels of benzyl peroxidase (thought to detoxify potential carcinogens) have been detected in the small bowel.

Together, with increased levels of immunoglobulin A and widespread gut lymphoid tissue, these factors may impede the growth and development of tumors and their malignant transformation.

Epidemiology

Race

No racial or ethnic predisposition has been discovered.

Sex

Several series have noted a slight predominance in males compared to females.

Age

Benign small bowel lesions have been documented in persons of all age groups, although the mean age of presentation reportedly is between the fifth and sixth decades of life.

History

Clinically, benign small bowel lesions are characterized by a lack of identifying symptoms. A review of published reports reveals that multiple findings can occur sporadically; no hallmark presentation has been described. Possible signs and symptoms are as follows:[1, 2, 3, 4]

The interval from symptom onset to diagnosis reportedly ranges from less than a month to more than a year, with a mean duration of symptoms of 6 months. Despite their frequently unobtrusive nature, benign small bowel tumors may manifest primarily as secondary complications of their growth. Note the following:

Benign small bowel tumors may develop as a single lesion or as multiple lesions of several subtypes. The types of tumors include the following:

Physical

The physical examination is usually unrevealing, except in the case of larger tumors (>6 cm), which occasionally manifest as a palpable abdominal mass. Abdominal pain may occasionally be elicited upon palpation of the lesion. Most patients exhibit no distinct physical findings upon examination.

Laboratory Studies

Results from routine laboratory testing do not reveal abnormalities in most patients with small bowel tumors. Microcytic anemia may be observed in conjunction with vascular or ulcerated bleeding lesions. Electrolyte abnormalities are not commonly identified in patients with small bowel tumors.

Imaging Studies

Findings from plain films of the abdomen are frequently normal. Larger lesions may demonstrate signs of complete or partial small bowel obstruction (eg, dilated small bowel, air-fluid levels, volvulus).

Barium contrast studies (eg, upper GI series, small bowel enteroclysis) are the most frequently used diagnostic tools.

Images from upper GI series may demonstrate the lesion in up to 29% of cases. The radiographic appearance on upper GI series includes irregular mucosal surfaces, extraluminal, barium-filled cavities (showing central lesion necrosis), and dumbbell-shaped lesions (indicating intraluminal and extraluminal growth).

In reports, CT scan images of the abdomen demonstrate up to 27% of benign small bowel tumors. Gut stromal tumors larger than 2 cm are frequently imaged successfully using a CT scan. Accurate size, evidence of ulceration, and lesion necrosis are often detected.

Ultrasound images of the abdomen may demonstrate larger tumors (>4 cm) and can help differentiate intraluminal, intramural, and extraluminal growth patterns.

Barium enema helps identify distal ileal lesions with successful reflux of contrast through the ileocecal valve.[26]

Procedures

Upper endoscopy/intraoperative enteroscopy

Upper endoscopy has been used successfully in the detection of proximal benign small bowel lesions in 12-30% of reported cases.

For more distal small bowel lesions, intraoperative enteroscopy is an effective technique that allows simultaneous palpation and visualization of the small bowel in its entirety to increase the possibility of lesion identification.[27]

Endoscopy allows concomitant biopsy of intraluminal lesions. Polypectomy may also be performed for small lesions.

Gut stromal tumors and lipomas frequently cannot be removed via endoscopy because of their deep intramural location and the subsequent elevated risk of bowel perforation during attempted removal. In addition, some authorities caution against endoscopic lesion biopsy because of the increased risk of shedding cells, which could lead to nests of local tumor recurrence. Note the image below.


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Benign neoplasm of the small intestine. Intraoperative view of an ependymal small bowel stromal tumor. Notice the narrow lesion stalk and high degree ....

Capsule endoscopy

The newer modality of capsule endoscopy has been successfully used to detect small bowel lesions that have previously remained undiagnosed by other methods.[20, 28, 29] Both color video images and transit time values can be analyzed for regional mucosal abnormalities.

Solid benign tumors, such as leiomyomas, as well as vascular lesions (eg, angiodysplasia, varices), have been identified in patients through the use of capsule endoscopy.

Arteriography

Selective arteriography may be used to aid in the diagnosis of possible vascular lesions and potential embolization of active bleeding. Both subserosal tumors and hemangiomas may be identified by characteristic tumor blush visualized on arteriograms. Additional clues include multiple feeding arteries, irregular draining veins, and venous pooling around the lesion.

Arteriography may assist in differentiating malignant lesions from benign lesions. Benign tumors frequently receive arterial supply from either the gastroduodenal artery or the superior mesenteric artery. Malignant lesions often demonstrate aberrant arterial inflow from renal arteries, lumbar arteries, or both. Note the image below.


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Benign neoplasm of the small intestine. Arteriogram demonstrating small bowel gut stromal tumor, indicated by round tumor blush in lower right corner ....

Medical Care

Strict medical management currently has no role in benign small bowel tumors. Patients with suggestive abdominal pain, particularly if associated with evidence of anemia or intermittent obstruction, should be referred for surgical evaluation and management. Asymptomatic evidence of small bowel mass, stricture, or intermittent obstruction discovered on incidental radiographs should be referred for surgical evaluation.[30]

Surgical Care

Surgical excision of small bowel tumors remains the recommended therapy. Exploratory laparotomy with excision of the lesion provides the safest and most direct method for lesion identification and treatment.

Tumors discovered incidentally at laparotomy should be removed to prevent future symptom development and secondary complications.

Both segmental resection and enterotomy/polypectomy have been used for lesion removal. If the pathology cannot be established at the time of resection, full segmental resection with adequate margins is recommended. Note the image below.


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Benign neoplasm of the small intestine. Cross section of a gross ependymal small bowel stromal tumor after removal. Mixed stromal elements and a high ....

Literature confirms an excellent prognosis for tumors resected prior to tumor perforation or onset of massive GI hemorrhage.

Consultations

Consultations with either gastroenterology or general surgery may be helpful when abdominal pain of unknown origin or suspicion of small bowel pathology exists so that the patient may benefit from full endoscopy, capsule endoscopy, or intraoperative push enteroscopy.[31]

Diet

No special dietary recommendations are applicable.

Activity

Activity is generally as tolerated for the patient. If the patient requires operative exploration for a benign small bowel tumor, the postoperative activity regimen should follow the recommendation of the operative surgeon.

Complications

Surgical complications for small bowel tumor resection should be minimal and limited to the technical aspects of the operation. Intraoperative bleeding, wound infections, anastomotic leak, and failure to localize the tumor at the time of operation have all been reported.

Postoperative complications include ileus, incisional hernia, and delayed small bowel obstruction from adhesions. Given the limited amount of bowel commonly resected for these rare tumors, short-bowel syndrome is not typically a concern.

Author

Shawn M Terry, MD, FACS, Clinical Assistant Professor of Surgery, Penn State University College of Medicine; Consulting Staff, Department of Trauma and Emergency General Surgery, Community Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

Thomas Santora, MD, Professor and Vice-Chair for Clinical Affairs, Department of Surgery, Temple University Hospital, Temple University School of Medicine

Disclosure: Nothing to disclose.

Specialty Editors

Francisco Talavera, PharmD, PhD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Amy L Friedman, MD, Professor of Surgery, Director of Transplantation, State University of New York Upstate Medical University College of Medicine, Syracuse

Disclosure: Nothing to disclose.

Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy

Disclosure: Nothing to disclose.

Chief Editor

John Geibel, MD, DSc, MA, Vice Chair and Professor, Department of Surgery, Section of Gastrointestinal Medicine, and Department of Cellular and Molecular Physiology, Yale University School of Medicine; Director, Surgical Research, Department of Surgery, Yale-New Haven Hospital

Disclosure: AMGEN Royalty Consulting; Ardelyx Ownership interest Board membership

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Benign neoplasm of the small intestine. Intraoperative view of an ependymal small bowel stromal tumor. Notice the narrow lesion stalk and high degree of vascularity.

Benign neoplasm of the small intestine. Arteriogram demonstrating small bowel gut stromal tumor, indicated by round tumor blush in lower right corner of the image.

Benign neoplasm of the small intestine. Cross section of a gross ependymal small bowel stromal tumor after removal. Mixed stromal elements and a high degree of cellularity are apparent.

Benign neoplasm of the small intestine. Arteriogram demonstrating small bowel gut stromal tumor, indicated by round tumor blush in lower right corner of the image.

Benign neoplasm of the small intestine. Intraoperative view of an ependymal small bowel stromal tumor. Notice the narrow lesion stalk and high degree of vascularity.

Benign neoplasm of the small intestine. Cross section of a gross ependymal small bowel stromal tumor after removal. Mixed stromal elements and a high degree of cellularity are apparent.