Zollinger - Ellison Syndrome.

Alternative Names:

Z - E Syndrome; gastrinoma

DEFINITION:

A gastrin-secreting tumour of the pancreas that causes ulceration of the upper gastrointestinal tract.

Causes, incidence, and risk factor:

With this condition, many people have symptoms of peptic ulcer disease or possibly pancreatitis, but fail to respond to the usual treatment. The tumour can be located in the duodenum, stomach, spleen, and lymph nodes in addition to the pancreas. In one-half to two-thirds of the people, the tumours are malignant, and spread slowly to the liver or the lymph nodes. What causes people with Z-E syndrome to develop tumours is unknown, but the cause may be an abnormal tumour supressor gene, and about 25% of people with gastrinoma may also have a genetic syndrome called multiple endocrine neoplasia type 1 (MEN 1). The disease is most common in people between 20 - 60 years old; it is very rare in children. The overall incidence is 6 out of 100,000 people.

SYMPTOMS:

  • peptic ulcer pain.

  • vomiting acidic fluid/blood.

  • diarrhoea.

  • fatigue.

  • weakness.

  • weight loss.

Signs and Tests:

  • The gastrin level is increased.

  • A secretin stimulation test for pancreas activity is positive.

  • A Calcium infusion test is positive.

  • Possible increased amounts of fat in the stools (steatorrhea).

  • Because pancreatic lipase inactivated by acidity, bile salts not absorbed

  • Abdominal CT scan shows duodenal mass (aggregation of cells) or pancreatic tumour.

The disease may also alter results of the stomach acid test.

TREATMENT:

H-2 receptor agonists such as cimetidine, ranitidine, famotidine, nizatidine, lansoprazole and omeprazole reduce gastric acid secretion which aids ulcer healing and provides pain relief. Surgery to treat peptic ulcers or to remove tumours in the pancreas or duodenum are other treatment options. Total gastrectomy is sometimes recommended for people who fail to respond to medical treatment.

Expectations (prognosis):

Complete surgical removal of the gastrinoma with cure has been achieved in only 25% of those treated.

Complications:

  • Metastasis of the tumour

  • Failure to locate the tumour during surgery.

 

Extracts from: http://thriveonline.oxygen.com/medical/library/article/000325.html
(with additions related to a known case.)

 

Other sites:

http://www.middk.nih.gov/health/digest/summary/zolling/zolling.htm
http://www.rarediseases.org

MEDICATIONS:

Common brand names (mainly USA):
cimetidine (Tagamet, Acibilin, Acinil, Antil, Asaurex, Beamat, Biomag, Brumetidina, Cidine, Cigamet, Cimal, Cimebec, Cimeldine, Cimetag, Cimetase, Cimetegal, Cimetegal, Cimetidina, Cimetiget, Cimetil, Cimetum, Cimewet, Cimulcer, Cinulcus, Citius, Ciwidine, Convenal, Corsamet, Defense, Dyspamet, Edalene, Eureceptor, Fremet, Gastril, Gastrobitan, Gawer,Gerucim, Haldin, Histodil, Lenamet, Muxina,Neutronorm, Novocimetine, Nulcer, Palia, Paoweian, Peptol, Procimeti, Proctospre, Sanmetidin, Siamidine, Silmet, Simalgen, Somakon, Stogamet, Stomet, Tagagel, Tagamed, Tagamin, Tametin, Tobymet, Tratol, Ulcedine, Ulcedon, Ulcemet, Ulcerfen, Ulcimet, Ulcimfan, Ulpax, Ulset, Valmagen, Weisdin, Wergen, Ulsikur.)
famotidine – oral (Pepcid, Brolin, Facid, Famocid, Famodil, Famonit, Fanox, Ganor, Gaster, Gastrion, Motiax, Pepdul, Tamin, Topcid.)
lansoprazole Zoton.
nizatidine (Axid, Calmaxid, Cronizat, Distaxid, Gastrax, Naxidine, Nizaxid, Zanizal.)
omeprazole (Losec, Zoltan)
ranitdine Zantac, Acidex, Actoral, Atural, Axoban, Azantac, Coralen, Dantin, Gastrial, Gastrosedol, Histac, Istomar, Logast, Lydin, Mauran, Nodol, Quantor, Radinat, Ranaid, Randin, Raniben, Ranidil, Ranidine, Ranin, Raniogas, Raniplex, Ranisen, Ranital, Ranitiget, Rantin, Raticine, Rosimol, Sostril, Taural, Ul-pep, Ulcex, Ulsal, Vizerul, Zantadin, Zantic, Anistal.)


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