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Management Conference. A WOMAN WITH EPIGASTRIC PAIN, VOMITING Raika Jamali M.D. Gastroenterologist and hepatologist Tehran University of Medical Sciences. A 62 year old woman with epigastric pain , post prandial vomiting and weight loss from 2 months ago.
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Management Conference A WOMAN WITH EPIGASTRIC PAIN, VOMITING Raika Jamali M.D. Gastroenterologist and hepatologist Tehran University of Medical Sciences
A 62 year old woman with epigastricpain, post prandial vomiting and weight loss from 2 months ago. • The epigastric pain is constant with episodes of colicky pain after meals followed by vomiting. • No radiation and no response to PPI is reported for epigastric pain. • Weight loss is about 8 Kg in the past 2 months.
There was a history of cholecystectomy and choledochodeudenostomy due to cholecystitis and cholelithiasis In 83.12.27. • The patient had epigastric pain from one year before surgery which was aggravated in the past 2 months before admission in 83.12.27. • Epigastric pain aggravated by eating but no vomiting was reported.
The patient had an episode of acute abdominal pain in 84.2.30 that lead to laparotomy for evaluation of acute abdomen. • The surgical report was: Serosanginous fluid in abdomen & pelvic. Adhesions from previous surgery and edematous pancreas but no mass was seen in the pancreas. • The patient discharged with the diagnosis of pancreatitis.
The epigastric pain persisted and did not respond to PPI so endoscopy performed : • GERD grade A + Hiatal hernia + pan gastritis + mild duodenitis • PPI continued
There was an episode of colicky abdominal pain which resulted in third laparotomy for evaluation of acute abdomen (85.2.29). • The surgical diagnosis was pancreatitis.
After 3 laparatomies the patient referred to gastroentrologist for evaluation of persistent epigastric pain, vomiting and weight loss in 85.6.25.
OB = negative • AST=26 • ALT=36 • ALP=612*
UGI endoscopy: Duodenitis • Colonoscopy: normal • Sonography: Multiple hypoechoic lesions in liver. Enlargment of pacreatic head. CT scan recommended.
What is the next diagnostic step ? Small intestine follow through for evaluation of partial obstructoin and GI blood loss?
Endoscopy Report: Esophagus:Crico-pharyngeus , upper third and middle third were normal. Medium-sized Hiatal hernia was found. There was a esophagitis in lower third. ____________________________Stomach:Fundus, body, incisura, antrum and pre-pyloric area were normal. ____________________________Duodenum:Bulb was normal. There was choledocoduodenostomy. Also there was a mass lesion at begining of D3 with partial obstruction . The scope was not passed through the mass. ____________________________
The pathology report was: Poorly differentiated adenocarcinoma
Prevalence of small bowel tumor • 1.1 - 2.4 % of GI malignancies • Approximately 2/3 small intestine tumors are malignant. • Adenocarcinoma is the most common small bowel malignancy with incidence of 3.9 cases per year. • Mean age at the time of diagnosis is between 50-60 years.
Distribution • Deudenum(55%) • Jejunum (18%) • Ileum (13%) • Not specified in terms of location (14%)
Histology: • Adenocarcinoma from mucosal glands(35-50%) • Carcinoid from argantaffin cells(20-40%) • Lymphoma (14%) • Leiomyosarcoma from smooth muscle • Neurofibroma from neurons • Angiosarcoma from endothelial cells • GIST from mesenchymal cells
Adenocarcinomahistologic classification • Approximately 50% of tumours will be moderately differentiated while • 15% will be well differentiated, • 33.9% will be poorly differentiated and 1.5% will be anaplastic.
Adenocarcinoma • Risk factors: • diets high in protein & animal fat. • Two fold increase in consumers of meat once a week. • Smoked foods eaten one to three per month with odds ratio of 1.7:1 . • Bile acids: synergic effect of bile acids and germ line APC mutation to foster the high predilection of duodenal polyps and adenocarcinoma in FAP.
Clinical Risk Factors • SBAs are reported to occur more frequently in patients with a history of CD, celiac disease, • and hereditary gastrointestinal cancers syndrome such as familial adenomatous polyposis (FAP), HNPCC, and Peutz-Jeghers syndrome (PJS).
Pathogenesis and Risk Factors of Small Bowel Adenocarcinoma: A Colorectal Cancer Sibling? • Thierry Delaunoit, M.D. • The American Journal of GastroenterologyVolume 100 Issue 3 Page 703 - March 2005
Why Are Duodenal SBAs more Frequent • Bile acids seem to promote the development of intestinal cancer in animals studies . • High fat and low fiber diets are often associated with bile acid excess, as well as increased risk of SBAs . • Distribution of proximal SI neoplasms in patients with FAP is also suggestive of a role played by bile acids in adenoma and adenocarcinoma development, since patients with FAP have been shown to have relatively higher total and unconjugated bile acids concentrations compared to the general population .
The capability of bile acids to produce DNA adducts in FAP patients seems pH dependent. • Scates and colleagues studied the role of an acid environment on the development of DNA adducts in patients with FAP and compared those results to a control group. • Bile acid from FAP patients produced higher levels of DNA adducts at pH 4–5 than at pH 6–8.
Clinical features • No specific sign or symptom • Cramping periumbilical pain, vomiting and distention ( GI obstruction) • Constant pain, ( back pain suggest spread to retroperitoneum, bleeding into the tumor, invasion of ganglia, ischemia and serosal involvement )
GI bleeding is the second most frequent sign ( massive GIB with sarcoma) • Weight loss • Intestinal perforation ( frequent with lymphoma and sarcoma) • Jaundice and pancreatitis ( periampulary tumor) • Cachexia, ascites, hepatomegaly
Diagnosis • UGI Endoscopy • Small bowel follow through • Enteroclysis ( small bowel enema): with greater accuracy • Ct scan: for detecting extramural disease • Small bowel enteroscopy: in cases with GIB • Intra operative enteroscopy • Video capsule enteroscopy: in cases with GIB
Barium studies • The most sensitive investigation for assessing mucosal and intraluminal abnormalities beyond the ligament of Treitz is a barium contrast study . • Enteroclysis has been suggested as a more useful investigation than a follow-through examination for diagnosing jejunal and ileal neoplasms. It is a relatively simple and rapid (< 1 h) investigation .
CT scan • Extra-mucosal spread, lymphadenopathy and distant metastases can all be detected . • Neoplastic disease is suspected when small bowel thickness exceeds 1.5 cm (normal: 4 mm). • The accuracy of CT in detecting small bowel tumours is approximately 47%. • There is a high sensitivity but low specificity for the detection of lymphadenopathy.
Push enteroscopy • Push enteroscopy as an alternative is not practical in most cases. It takes up to 8 h to perform, may not visualize the entire small bowel and up to 50–70% of the mucosa of the bowel examined is not seen properly.
MRI • Magnetic resonance (MR) enteroclysis is a single investigation with no irradiation of the patient. • It separately enhances the small bowel wall and lumen as well as giving images of the mesentery, surrounding structures and rest of the abdominal cavity.
Zhan J, et al. Gastrointestinal Division of Internal Medicine, Second Hospital, Sun Yat-Sen University, Guangzhou 510120, Guangdong Province, China.World J Gastroenterol. 2004 Sep 1;10(17):2585-7. Clinical analysis of primary small intestinal disease: A report of 309 cases
The major clinical symptoms included • abdominal pain (71%), • abdominal mass (14%), • vomiting (10%), • melena (10%), • and fever (9%). • Duodenum was the most common part involved in small intestine. • Double-contrast enteroclysis was still the simplest and the most available examination method in diagnosis of primary small intestinal disease.
What is the best management ? • Chemotherapy • Palliative surgery • combination
Treatment • In the first or second portion of duodenum usually are treated by pancreaticoduodenectomy. • Segmental resection is sufficient for patients with tumors arising from the third and forth portion of duodenum. • Even with large tumors and positive lymph nodes, surgeons resect the lesion for symptomatic relief.
Adenocarcinoma of the small bowel • REVIEW ARTICLE, Robert R. Hutchins, Ahmed Bani Hani, Pipin Kojodjojo, Robyn Ho and Steven J. Snooks • Australian and New Zealand Journal of SurgeryVolume 71 Issue 7 Page 428 - July 2001
Tx : Primary tumour not evaluated T0 : No pathological evidence of tumour Tis: In situ cancer T1 : Invades lamina propria or submucosa T2 : Invades muscularis propria T3 : Invades < 2 cm beyond serosaor non-peritonealized perimusculartissue (mesentery or retroperitoneum) T4 : Perforates visceral peritoneumor invades adjacent structure > 2 cm TNM Staging system
N0 : No regional nodes • N1 : Lymph node metastases • Mx : Metastases not evaluated • M0 : No metastases • M1 : Distant metastases
AJCC staging system • Stage 0 Tis N0 M0 • Stage 1 T1or2 N0 M0 • Stage 2 T3or4 N0 M0 • Stage 3 Any T N1 M0 • Stage 4 AnyT AnyN M1
Frequency of staging • Stage 0 is seen in 2.7% of patients, • stage I is seen in 12% of patients, • stage II is seen in 27% of patients, • stage III is seen in 26% of patients • stage IV is seen in 32.3% of patients.
Treatment • The mainstay of treatment of smallbowel cancer is surgical resection. • This may be curative or palliative and the type of procedure depends on the site of origin and stage of the tumor.
Curative surgery • Jejunal and ileal tumours are resected en bloc with draining regional lymph nodes in a manner similar to colorectal tumours. • The margin of tumor, is required to be at least macroscopically and microscopically clear .