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Small bowel tumors. Dr. TAMERM. ABD ELRHMAN. Risk factors. Red meat, Ingestion of smoked or cured foods. Crohn’s dsease . Hereditary nonpolyposis colorectal cancer . Familial adenomatous polyposis (FAD) – 100% to develop duodenal CA. Peutz-Jeghers syndrome. Symptoms.
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Small bowel tumors Dr. TAMERM. ABD ELRHMAN
Risk factors • Red meat, Ingestion of smoked or cured foods. • Crohn’sdsease. • Hereditary nonpolyposis colorectal cancer. • Familial adenomatouspolyposis (FAD) – 100% to develop duodenal CA. • Peutz-Jegherssyndrome.
Symptoms • Most are asymptomatic. • Symptoms: • Vague abdominal pain(with N/V, diarrhea). • Bleeding. • Obstruction (intussuception, circumferencial growth, kinking of the bowel, intramural growth).
Diagnosis • For most are asymptomatic it is rarely diagnosed preoperatively. • Serological examination: • Serum 5-hydroxyindole acetic acid (HIAA) for carcinoid. • Radiological examination: • Enteroclysis (test of choice – 90% sensitivity). • CT scan. • Angiography --> bleeding lesions. • Endoscopy.
Benign tumors Adenomas: (most common benign neoplasm – duodenum): tubular, villous, tubulovillous. Fibromas, lipomas, hemangiomas, lymphangiomas, neurofibromas.
Peutz-Jeghers Syndrome • Inherited syndrome of: • Mucocutaneousmelatonic pigmentation (face, buccal mucosa, palm, sole, peri-anal area). • Gastrointestinal polyp(jejunum and ileum are most frequent part followed by colon, rectum and stomach). • Symptoms: • colicky abd.pain (due to intermittent intussuception). • Hemorrhage. • Treatment: • Segmental resection of the bowel causing obstruction or bleeding. • Cure impossible due to widespread intestinal involvement
Malignant neoplasm: • Adenocarcinoma: • Most common CA of small bowel. • Most common in duodenum and proximal jejunum. • Half involve the ampulla of Vater. • Carcinoid: • Appendix (46%) > Ileum (28%) > Rectum (17%) • Aggressive behavior than the appendicealcarcinoid. • appendix – 3% metastasize; Ileum – 35% metastasize • Appendix – solitary; Ileum – 30% multiple.
carcinoid syndrome • 25-50% with carcinoid tumor with liver metastasis develops carcinoid syndrome. • Secretes serotonin, bradykinin and substance P, (which undergo complete metabolism inliver, so symptoms of carcinoid syndrome is rare in absences of liver metastasis). • Diarrhea • Flushing • Hypotension • tachycardia • fibrosis of endocardium and valves of the right heart.
Malignant neoplasm: • Lymphomas: • Most common intestinal neoplasm in children under 10y/o. • Most common presentation: obstruction, Perforation. • Criteria of primary lymphomas of the small bowel: • Absence of peripheral lymphadenopathy. • No evidence of mediastinal LN enlargement. • Normal WBC count. • At operation, bowel lesion must predominate the only nodes are associated with the bowel lesion. • Absence of disease in the liver and spleen.
Malignant neoplasm: • GISTs: (gastrointestinal stromal tumors) • Most common mesenchymal tumors arising in the small bowel. • 70% arises from the stomach followed by the small bowel. • Associated with overt hemorrhage. • Has its expression of the receptor tyrosine kinaseKIT (CD117).
Treatment: • Benign lesions: • All symptomatic benign tumors should be surgically resected or removed endoscopically. • Duodenal adenomas with FAP should undergo panareaticoduodenectomy (it has 100% degenerate to CA).
Treatment: • Malignant lesions: • Adenocarcinoma: • Wide local resection with it’s mesentery to achieve regional lymphadenectomy. • Chemotherapy has no proven efficacy in the adjuvant or palliative treatment. • Small intestinal lymphoma: • For localized: segmental resection with adjacent mesentery. • If with diffused involvement: -->chemotherapy is primary therapy.
Treatment: • Carcinoid: • Segmental intestinal resection & regional lymphadenectomy. • 30% are multiple, hence entire small bowel should be examined prior to surgery. • If with metastatic lesions---> debulking. • Chemotherapy: ---> 30-50% response ( Doxorubicin, 5-fluorouracil. Streptozocin). • Octreotide: - most effective for management of symptoms of carcinoidsyndrome.
Treatment: • Small-intestine GISTs: • Segmental resection. • If was preoperatively diagnosed, lymphadenectomy not be done,as rarely associated with LN metastases. • Resistant to conventional chemotherapy. • IMATINIB (Gleevec): tyrosine kinase inhibitor • Clinical trial show 80% of pt with unresectable lesions or metastasis showed clinical benefits, 50 – 60% showed evidence of reduction in tumor volume.