Liver metastases of endocrine tumour associated with multiple endocrine neoplasia type 1: a sustained response to interferon therapy or a peculiar benign course?
The authors describe the case of a 51-year-old male with Zollinger-Ellison syndrome manifested by epigastralgia, nausea, vomiting, hypergastrinemia and multiple endocrine neoplasia type 1. History included a Billroth II procedure for a perforated duodenal ulcer. Multiple metastatic liver lesions were found that were gastrin-negative and chromogranin-positive. Endoscopy revealed a large ulcerated gastro-jejuno-colonic fistula which was surgically repaired. Pre- and postoperative imaging studies, including the highly sensitive somatostatin-receptor scintigraphic scan using In-pentetreotide, have consistently failed to disclose other tumors. Recent reports indicate that most Zollinger-Ellison syndrome-associated gastrinomas are small, easily overlooked lesions located in the proximal duodenum rather than in the pancreas as formerly believed. In the present patient therapy with omeprazole and alpha-interferon has produced complete remission of the Zollinger-Ellison syndrome and a stabilization of tumor growth has occurred during the last 7 years, allowing the patient to live a normal life. This peculiar response to therapy is discussed.
Grieco
A
A
Istituto di Medicina Interna e Geriatria, Policlinico Universitario A. Gemelli, Università Cattolica del S. Cuore, Roma, Italy.
Bianco
A
A
Alfei
B
B
Rufini
V
V
Vecchio
F M
FM
Marcoccia
S
S
Gasbarrini
G
G
D006801:Humans; D016898:Interferon-alpha / Q000627:therapeutic use*; D008113:Liver Neoplasms / Q000556:secondary* / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000628:therapy*; D009853:Omeprazole / Q000627:therapeutic use; D015043:Zollinger-Ellison Syndrome / Q000628:therapy*
D016898:Interferon-alpha; D009853:Omeprazole