Tumors of the Small Intestine Smallbowel tumors comprise
Tumors of the Small Intestine
Small-bowel tumors comprise <3% of gastrointestinal neoplasms. Because of their rarity, a correct diagnosis is often delayed
Small-bowel tumors should be considered in the differential diagnosis in the following situations recurrent, unexplained episodes of crampy abdominal pain intermittent bouts of intestinal obstruction, especially in the absence of IBD or prior abdominal surgery intussusception in the adult evidence of chronic intestinal bleeding in the presence of negative conventional contrast radiographs
A careful small-bowel barium study is the diagnostic procedure of choice the diagnostic accuracy may be improved by infusing barium through a nasogastric tube placed into the duodenum (enteroclysis
SMALL INTESTINE TUMORS Benign Epithelial Tumors Lymphoproliferative disorders Malignant Epithelial Tumors Brunner Benign • Pr. ADC Gland Intestinal • Metastasis. Lesions polypi • Carcinoid. • Adenomas • Hamartomas T cell Enteropathy associated T-cell lymphoma GIST • Benign • Malignant Mesenchymal Tumors B cell • Diffuse large cell lymphoma. • Small non cleaved cell lymphoma. • MALT cell lymphoma. • Mantle cell lymphoma. • Immuonoproliferative small cell disease Fatty Neural tumors • Lipoma • Liposarcoma Para Smooth Vasc. Ms gangl. tumors • Leioyomayosarcoma • Gut autonoic tumor • Schwannoma • Neurofibroma • Granular cell tumor • Haemangioma • Angiosarcoma • Lymphangioma • Kaposi sarcoma
Benign Tumors Adenoma Lipomas Leiomyoma Angioma others
Malignant Tumors Adenocarcinoma Lymphomas Carcinoid Tumors others Leiomyosarcomas
Brunner's gland adenomas not truly neoplastic a hypertrophy or hyperplasia of submucosal duodenal glands appear as small nodules in the duodenal mucosa that secrete a highly viscous alkaline mucus an incidental radiographic finding not associated with any specific clinical disorder
Polypoid Adenomas About 25% of benign small-bowel tumors Gardner's syndrome the sessile or papillary form of the tumor is sometimes associated with a coexisting carcinoma Hamartomatous polyps In Peutz-Jeghers (not adenoma) FAP
Angiomas they frequently cause intestinal bleeding. They may take the form of telangiectasia or hemangiomas Multiple intestinal telangiectasias occur in a nonhereditary form confined to the gastrointestinal tract or as part of the hereditary Osler-Rendu-Weber syndrome of isolated hemangiomas, most commonly in the jejunum
Adenoma in duodenum
A, Film from an enteroclysis demonstrating a smooth, submucosal lesion that was found to be lipoma (arrow). B, Surgical resection specimen of a lipoma from another patient who presented with intussusception and bleeding
CT scan of the lower abdomen demonstrates a soft tissue mass of fat density in the lumen of the terminal ileum (arrow). These characteristics are diagnostic for a lipoma
Small bowel follow through examination demonstrates a smooth, well-circumscribed mass arising from the wall of the terminal ileum. The appearance is consistent with a benign mesenchymal tumor, such as a lipoma or a carcinoid tumor
Small bowel follow through examination shows a polypoid eccentric mass arising from the wall of the terminal ileum (arrow).
While rare, small-bowel malignancies occur in patients with long-standing regional enteritis celiac sprue AIDS
Adenocarcinomas The most common primary cancers of the small bowel are adenocarcinomas, accounting for ~50% of malignant tumors most often in the distal duodenum and proximal jejunum, where they tend to ulcerate and cause hemorrhage or obstruction Radiologically, they may be confused with chronic duodenal ulcer disease or with Crohn's disease
Small bowel follow through examination demonstrates a circumferential apple- core lesion of the jejunum (arrow), producing distension of the proximal small bowel.
S. I. ADENOCARCINOMA
Film from a small bowel follow through demonstrating an “apple-core” appearance caused by a metastatic lesion to the small intestine from a scirrhous gastric cancer.
Upper gastrointestinal endoscopy shows a duodenal adenocarcinoma in the second portion of the duodenum in a patient who presented with heme positive stool. The mass occupied approximately 50 percent of the diameter of the duodenum. The thick erythematous folds in the upper half of the image distinguish the lesion from the pale, thin folds of the normal tissue in the lower half.
Contrast examination of the small intestine in a patient with partial small bowel obstruction and extensive extramucosal disease caused by metastatic lung cancer.
Small bowel follow through study shows multiple rounded, nodular filling defects in the wall of the small bowel (arrows). Multiple small bowel tumors may be seen in metastatic disease or in polyposis syndromes; the most common cause of small bowel metastases is melanoma.
• Histologic confirmation of lymphoma • Normal peripheral blood smear or on bone marrow aspiration and biopsy • No palpable adenopathy • No hepatosplenomegaly • No evidence of lymphoma is seen on: • Chest radiograph • CT scan primary intestinal lymphoma
Secondary lymphoma of the small bowel Involvement of the intestine by a lymphoid malignancy extending from involved retroperitoneal or mesenteric lymph nodes
Primary intestinal lymphoma ~20% of malignancies of the small bowel non-Hodgkin's lymphomas; they usually have a diffuse, large-cell histology and are of T cell origin involves the ileum, jejunum, and duodenum, in decreasing frequency Risk factors: celiac sprue , regional enteritis , depressed immune function due to congenital immunodeficiency syndromes, prior organ transplantation, autoimmune disorders, or AIDS.
Diagnosis of small-bowel lymphoma • suspected from the appearance on contrast radiographs of patterns such as infiltration and thickening of mucosal folds, mucosal nodules, areas of irregular ulceration, or stasis of contrast materia • Intestinal lymphoma can occasionally be diagnosed by peroral intestinal mucosal biopsy, but since the disease mainly involves the lamina propria, full-thickness surgical biopsies are usually required. • The diagnosis can be confirmed by surgical exploration and resection of involved segments
Luminal small bowel tumors may cause intussusception
Barium enema shows a large soft tissue mass in the cecum (arrows) caused by intussusception of a lymphoma arising in the terminal ileum
CT scan of the abdomen demonstrates a large mass in the lumen of a distended loop of small bowel. Note mesenteric fat in the center of this intraluminal mass (arrow).
Treatment of primary inestinal lymphoma Resection of the tumor constitutes the initial treatment modality postoperative short-term (three cycles) systemic treatment with combination chemotherapy The probability of sustained remission or cure is ~75% in patients with localized disease but is ~25% in individuals with unresectable lymphoma. radiation therapy following a total resection? In patients whose tumors are not resected, chemotherapy may lead to bowel perforation
Immunoproliferative small intestinal disease (IPSID), Mediterranean lymphoma, or -heavy chain disease diffusely involves the entire intestine B cell tumor presence in the blood and intestinal secretions of an abnormal Ig. A that contains a shortened heavy chain and is devoid of light chains chronic diarrhea and steatorrhea associated with vomiting and abdominal cramps; clubbing of the digits clinical course of exacerbations and remissions
IPSID-Treatment The use of oral antibiotics such as tetracycline appears to be beneficial in the early phases of the disorder, suggesting a possible infectious etiology. Combination chemotherapy has been administered during later stages of the disease, with variable results
Carcinoid tumors arise from argentaffin cells of the crypts of Lieberkühn and are found from the distal duodenum to the ascending colon, areas embryologically derived from the midgut. More than 50% of intestinal carcinoids are found in the distal ileum, with most congregating close to the ileocecal valve. Most intestinal carcinoids are asymptomatic and of low malignant potential, but invasion and metastases may occur, leading to the carcinoid syndrome
S. I. CARCINIOD
CT scan demonstrates a soft tissue mass containing coarse central calcifications (short arrow) in the right lower quadrant. This carcinoid tumor is producing a characteristic desmoplastic response with spiculation of the adjacent mesenteric fat (long arrow).
Leiomyosarcomas often are >5 cm in diameter and may be palpable on abdominal examination Bleeding, obstruction, and perforation are common Such tumors should be analyzed for the expression of mutant c -kit receptor (defining GIST
Capsule endoscopy view of an ulcerated mass in a patient who presented with gastrointestinal bleeding. Four ulcerated, bleeding masses were found throughout the small bowel; these were confirmed at surgery and found to be sarcomas
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