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Tumours of the Small Intestine

1. BENIGN NEOPLASMS

Gastro-intestinal stromal tumours (GIST)

  • Most common benign tumour
  • Unlike most other GIT tumours GISTs are non-epithelial
  • Arise from intestinal cell of Cajal
  • Show c-kit mutation
  • Express the CD117 and CD34
  • Mostly found in the stomach, jejunum, ileum (rare in the duodenum)
  • Grow intramurally – can cause obstruction
  • Can outgrow blood supply
  • Treatment is via surgical resection

Adenomas

  • Benign tumour of glandular origin
  • 3 types – true, villous, Brunner gland adenomas
    • True and villous type are premalignant
  • Most common in ileum, jejunum, duodenum
  • Commonly present with bleeding and obstruction

Types

  • Brunner gland adenoma
    • Benign hyperplastic tumour in duodenum. No malignant potential
    • Bleeding is the usual presentation
    • Treatment – endoscopic resection
  • Villous adenoma
    • Most common in periampullary region – presents as obstructive jaundice and upper abdominal pain
    • High malignant potential – especially with adenomas >3cm
    • Treatment – ERCP to relieve obstruction; panreaticoduodenectomy
  • True adenomas
    • Usually occurs as a single adeoma
    • Treatment – endoscopic excision or pancreaticoduodenectomy depending on invasiveness
  • Familial adenomas
    • Associated with familial adenomatous polyposis (FAP) – 5% risk of adenocarcinoma
    • It is diffuse throughout the duodenum
    • Screening endoscopy is done every year
    • Treatment
      • Endoscopic/open polypectomy – for benign lesions
      • Pancreaticoduodenectomy – for high grade lesions or carcinoma in situ

Lipomas

  • Benign tumour of fatty tissue
  • Most common in ileum – located intramurally in submucosa
  • Mostly seen in elderly men
  • Can cause intussusception, obstruction or bleeding
  • No malignant potential

Peutz-Jegher’s syndrome

  • Autosomal dominant condition, characterised by benign hamartomatous polyps in the GIT and hyperpigmented macules on the lips and oral mucosa
    • Hyperpigmented lesions can also occur on forearms, palms, soles, digits
  • Clinical features – colicky abdominal pain; intussusception, bleeding, anaemia
  • Increased risk of developing cancer of the small intestine, stomach, pancreas, ovary, lung, uterus and breast
  • Treatment – small resection for removal

Hemangioma

  • Usually occur as multiple benign tumours
  • They are abnormal submucosal proliferative vessels – most common in the jejunum
  • Presents as intestinal bleeding
  • Treatment – endoscopic sclerotherapy; angiographic embolisation; resection of bowel segment

2. MALIGNANT TUMOURS

Adenocarcinoma

  • Most common in the duodenum and jejunum
  • Risk factors – FAP, adenomas, Crohn’s disease, Peutz-Jegher’s syndrome
  • Spreads to lymph nodes, liver and peritoneal cavity
  • Clinical features – colicky abdominal pain, nausea and vomiting, weight los, occult bleeding, obstruction, jaundice
  • Treatment – surgical resection
    • Pancreaticoduodenectomy for periampullary/duodenal tumours
    • Radical resection for ileal/jejunal tumours
    • Right hemicolectomy for terminal ileum tumours
    • Poor prognosis for carcinoma patients who previously had Crohn’s Disease

Carcinoid tumours

  • Arise from entero-chromaffin cells of crypts of Leiberkuhn
  • Commonly occur in the appendix, ileum and rectum
    • Extra-intestinal sites – pancreas, biliary tract, bronchus
  • 75% of cases are asymptomatic and are found incidentally
  • Treatment – surgical resection

3 types

  • Foregut tumours – bronchial, stomach, biliary tree
    • Secrete low levels of serotonin, but can secrete ACTH
  • Midgut – jejunum, ileum, appendix, right colon
    • Secrete high level of serotonin
  • Hindgut – left colon, rectum
    • Secrete somatostatin

Clinical features

  • Can be asymptomatic or can present with abdominal pain, intestinal obstruction, diarrhoea
  • Carcinoid syndrome – occurs after the carcinoid tumour has metastasised
    • Secretion of serotonin, prostaglandins, histamine, kinins – causes flushing, diarrhoea, cyanosis, wheezing, palpitations
    • Characteristically exacerbated by alcohol

Non-Hodgkin’s Lymphoma

  • GIT is the most common extra-nodal site of NHL MALT, centrocytic, Mediterranean, Burkit type, T-cell
  • B cell type is the most common; however, T cell type has worse prognosis
  • Clinical features
    • Acute – obstruction, perforation; can lead to peritonitis
    • Chronic – malaise, weight loss, diarrhoea, abdominal mass
  • Treatment – resection followed by adjuvant chemotherapy

Gastro-intestinal stromal tumour (GIST)

  • Most are benign but can become malignant
    • Large tumours and tumours with high mitotic index have a higher malignant potential
  • Clinical features – bleeding (melena), abdominal pain, weakness, weight loss, obstruction or perforation
  • Treatment – don’t respond well to radio/chemotherapy, so treatment is excision
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