Zollinger-Ellison Syndrome

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Classification according to ICD-10
E16.4 Abnormal gastrin secretion
- Zollinger-Ellison syndrome
ICD-10 online (WHO version 2019)

The Zollinger-Ellison syndrome is the paraneoplastic appearance, on the formation of the hormone gastrin based by so-called gastrinomas. This hormone stimulates the stomach to produce more hydrochloric acid . The disease is named after the first person to describe it, the American surgeons Robert Milton Zollinger (1903–1992) and Edwin Homer Ellison (1918–1970).

root cause

Zollinger-Ellison Syndrome is based on rare tumors in the pancreas (75%) or in the upper portion of the small intestine (20%), which are malignant in 60% of cases. These tumors produce the hormone gastrin and are therefore also called gastrinomas. Gastrinomas are classified as neuroendocrine tumors (NET), they can occur individually or as multiple tumors. About half to two-thirds of the individual gastrinomas are malignant ( malignant ) tumors in liver and lymph nodes can absiedeln. Almost 25% of patients with gastrinomas have multiple tumors as part of multiple endocrine neoplasia (MEN) type I : the three "P" pituitary (English for hypophysis ), pancreas (pancreas), parathyroid ( parathyroid gland ).

The gastrin produced by a gastrinoma causes overproduction of stomach acid . Gastrin acts on the parietal / parietal cells in the stomach and causes them to release more hydrogen ions into the stomach lumen ( proton pump ). The increase in acidity contributes to the development of gastric ulcers (ulcera ventriculi) and duodenal ulcer (ulcera duodeni).

Symptoms

Multiple ulcers of the duodenum

diagnosis

The increased gastrin level (hypergastrinaemia) is particularly important for the diagnosis. Values ​​over 1,000 ng / l are almost conclusive. In contrast to other diseases, in which the gastrin level can also be increased, in Zollinger-Ellison syndrome there is an often 100 percent increase in gastrin level after provocation with secretin . Neuroendocrine tumors typically produce chromogranin , which is detected in serum.

Therapy-resistant, often atypically localized ulcers can also be used to confirm the diagnosis.

The localization diagnosis is carried about with octreotide - scintigraphy , positron emission tomography , MRI , CT and endoluminal ultrasound .

therapy

Proton pump inhibitors can reduce acid secretion. If possible, the tumors should be surgically removed. Curatively , however, this is only possible in the absence of metastases . The chemotherapy has been shown to be very effective. The hormone production can be inhibited by somatostatin .

Metastatic neuroendocrine tumors can be treated with a palliative approach with 90 Y - DOTATOC or 177 Lu -DOTATOC.

Occurrence

This disease is extremely rare and only occurs in about five to ten cases per year and per million people. It can occur at any age, but predominantly in 30 to 60 year olds.

literature

  • SJ Konturek et al .: Case presentation of gastrinoma combined with gastric carcinoid with the longest survival record - Zollinger-Ellison syndrome: pathophysiology, diagnosis and therapy. In: Med Sci Monit. 2002, Volume 8, pp. CS43-59, PMID 12070442 .
  • EC Ellison et al: Forty-year appraisal of gastrinoma. Back to the future. In: Ann Surg . 1995, Vol. 222, pp. 511-521, Discussion 521-4, PMID 7574931 .
  • DK Andersen et al .: Current diagnosis and management of Zollinger-Ellison syndrome. In: Ann Surg. 1989, Volume 210, pp. 685-703, PMID 2686566 .
  • JW Harmon et al: Removal of gastrinomas for control of Zollinger-Ellison syndrome. In: Ann Surg. 1984, Vol. 200, pp. 396-404, PMID 6148919 .
  • S. Bonfils et al .: Results of surgical management in 92 consecutive patients with Zollinger-Ellison syndrome. In: Ann Surg. 1981, Volume 194, pp. 692-697, PMID 7030236 .
  • CW Deveney et al: The Zollinger-Ellison syndrome - 23 years later. In: Ann Surg. 1978, Volume 188, pp. 384-393, PMID 686901 .
  • EH Ellison et al .: THE ZOLLINGER-ELLISON SYNDROME: RE-APPRAISAL AND EVALUATION OF 260 REGISTERED CASES. In: Ann Surg. 1964, Vol. 160, pp. 512-530, PMID 14206854 .
  • CA Godellas et al. Gastrinoma: State of the Art. In: Cancer Control . 1997, Volume 4, pp. 30-34, PMID 10763001 .
  • M. Peracchi et al: Plasma chromogranin A in patients with sporadic gastro-entero-pancreatic neuroendocrine tumors or multiple endocrine neoplasia type 1. In: Eur J Endocrinol. 2003, Vol. 148, pp. 39-43, PMID 12534356 .
  • D. Granberg et al .: Clinical symptoms, hormone profiles, treatment, and prognosis in patients with gastric carcinoids. In: Good . 1998, Vol. 43, pp. 223-228, PMID 10189848 .
  • G. Cadiot et al .: Usefulness of somatostatin receptor scintigraphy in the management of patients with Zollinger-Ellison syndrome. Groupe de Recherche et d'Etude du Syndrome de Zollinger-Ellison (GRESZE). In: Good. 1997, Vol. 41, pp. 107-114, PMID 9274481 .
  • JM Temperley et al .: Zollinger-Ellison syndrome and hyperparathyroidism. In: Proc R Soc Med. 1971, Vol. 64, p. 1004, PMID 4329791 .
  • W. Shi et al.: Localization of neuroendocrine tumors with [111In] DTPA-octreotide scintigraphy (Octreoscan): a comparative study with CT and MR imaging. In: QJM . 1998, Vol. 91, pp. 295-301, PMID 9666953 .
  • LF Yee et al: Neuroendocrine disorders of the gut. In: West J Med. 1995, Vol 163, pp 454-462, PMID 8533409 .
  • P. Tomassetti et al: Treatment of Zollinger-Ellison syndrome. In: World J Gastroenterol. 2005, Vol. 11, pp. 5423-5432, PMID 16222731 .

Individual evidence

  1. ^ RM Zollinger, EH Ellison: Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas. In: Ann Surg. Volume 142, 1955, pp. 709-728. PMID 13259432 .
  2. ↑ M. Hofmann, T. Krause: Therapy with receptor-affine peptides. In: T. Kuwert, F. Grünwald, U. Haberkorn, T. Krause: Nuclear medicine . Stuttgart / New York 2008, ISBN 978-3-13-118504-4 .

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