APUDomas:

- term given to an embryologically related group of tumours of endocrine organs.

- APUD: amine precursor uptake and decarboxylation.
- APUD cells share properties of both neural and endocrine tissues i.e. NEUROENDOCRINE
CELLS.
- Tumors of this system: APUDomas.
- Tumors of these cells secrete the hormone of the tissue of origin.
- The freq. of the production of a given hormone appears to correlate with the degree of
embryological relationship of the cancer of origin to other tissues I the APUD system.
- The APUDomas of the GIT include:
1. carcinoid
2. insulinoma
3. MEN I
4. gastrinoma
5. VIPoma ( Verner Morriso syndrome / WDHA

CARCINOID TUMORS:

Epidemiology:
- most common site -> appendix followed by small bowel and rectum / rectosigmoid jxn
- no sex predilection.
- Age groups: 50 – 60yrs non-appendiceal origin
Mean age of 36yrs: appendiceal carcinoids.

Pathology:
- carcinoids may arise in organs derived from foregut; midgut and hindgut.
- Rarely diagnosed preoperatively.
- Usually is a small neoplasm arising in the submucosa.
- As the tumor enlarges it usually leaves the underlying mucosa intact.
- Growth is exceedingly slow.
- Criteria for malignancy:
1. evidence of spread into the serosal layer
2. size >2cm
3. mets to lymph nodes
4. mets to distant sites
- histochemical staining reactions of tumors vary depending on where they are found:
1. foregut: argyrophillic +; argentaffin –
2. midgut: argentaffin +
3. hindgut: argyrophillic - ; argentaffin –

-biochemically:
1. foregut: secrete 5-HTP into serum and urine
2. midgut: serotonin – serum ; 5-hydroxyindoleacetic acid in urine
3. hindgut – rarely secrete 5-HTP or serotonin
CARCINOID OF APPENDIX:

- predominantly asymptomatic.
- Majority located in the tip.
- Some degree of invasion of the wall – 80%.
- Rx: betwn. 1 – 2cm: if can be totally resected by appendectomy should be done
Otherwise R hemicolectomy.
- >2cm: R hemicolectomy.

CARCINOID OF SMALL BOWEL:

- pain is an occasional presenting symptom.

- Other clinical manifestations may be duodenal or common bile duct obstruction.
- Duodenal carcinoids form about 5%; usually small odules that can be excised by wedge
excision.
- Carcinoids of the rest of the bowel: 25-30%
- They are characteristically multiple; are asymptomatic ( when they are not tend to cause bowel
obstruction)
- Early radiologic diagnosis is difficult since most of these tumors are located in the submucosa.
- The perineoplastic fibrous reaction may lead to twisting and kinking of the bowel.
- The treatment of carcinoids of the small bowel is primarily surgical: simple resection of the
involved segment with anastomosis.

INSULINOMAS:

- are the commonest pancreatic APUDomas and the most freq cause of organic hyperinsulinism.
- 95% of insulinomas are benig & 90% are single.
- Diagnosic hallmark of the syndrom is the whipple triad namely symptoms of hypoglycemia ,
low blood glucose level and reliefe of symptoms after IVI administration of glucose .
- As soon as a patient is identified , care must be taken to prevent severe hypoglycemia .
- Plain abdominal x-ray ad ultrasound studies are really helpful.
- CT and MRI locate 50 – 60% of tumors .
- Arteriography also plays an important role .
- Treatment for insulinoma is surgical : the whole abdomin should be explored & a careful search
for liver mets and regional lymph nodes .
- Tumors in the head of the pancreas that appear benign should be removed locally by dissection.
- Those in the body or tail best treated by distal pancreatectomy .
- The generalized islet cell involvment in patients with MEA I suggest that total pancreatectomy is
the treatment of choice in this syndrom.
 GASTRINOMA:

- first described in 1955 by Zollinger & Ellison

- the hormone involved is gastrin.
- Comprises virulent ulcer diathesis, massive gastric acid hypersecretion & an islet tumor of the
pancreas.
- 75% sporadic , 25% associated MEN I syndrome .
- abdominal pain is the chief complaint & neartly 2/3 of patient have diarrhoea
- duodenal ulcers are more common but jejunal ulceration has been documented.
- ZES must be excl in all patients with intractable peptic ulcer, severe oesophagitis or persistent
secretory diarrhoea..
- Diagnosis depends on the presece of hypergastrinaemia in the face of increased secretion of
gastric acid.
- CT , MRI and arteriography have partial success.; more useful with the larger tumors.
- In terms of treatment, the use of PPI’s has rendered total gastrectomy unnecessary.
- The role of surgery still remains unclear.

VIPOMA:

- usually arising from pancreatic islets that secrete VIP.

- It is a syndrome of profound watery diarrhoea, hypokalaemia and achlorhydia.
- The diagnostic triad is secretory diarrhoea, high circulating levels of VIP and a pancreatic tumor.
- Localization is achieved with enhanced CT, MRI or arteriography .
- As soon as diagnosis is made treatment with octreotide should be used to control fluid loss.
- Most VIPomas can be excised by distal pancreatectomy; the adrenals and retroperitoneal tissues
should be carefully examined.
- Chemotherapy in non-resectable tumors is of minimal benefit.

GLUCAGONOMA:

- it is a tumor of the islet alpha cells.

- Clinical pic is that of a characteristic skin rash, DM, anaemia, wt loss and high circulating levels
of glucagon
- The diagnosis is made by the high glucagon levels and enhanced CT that picks up the pancreatic
tumor.
- Pre-op admin of TPN and pre-op heparin is indicated for these patients.
-
- Treatment is surgical excision of the tumor and chemotherapy has proved disappointing.