Octreotide improves biochemical, radiologic, and symptomatic indices of gastroenteropancreatic neoplasia in patients with multiple endocrine neoplasia type 1 (MEN-1)

Burgess, John; Greenaway, TM; Parameswaran, V.; Shepherd, J. J.

doi:10.1002/(sici)1097-0142(19991115)86:10<2154::aid-cncr39>3.0.co;2-w

Cited by 23 publications

(11 citation statements)

References 27 publications

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“…The treatment of hypergastrinaemia in MEN 1 is the subject of debate 7,21,22 . Unlike sporadic gastrinoma, in MEN 1 this disease is rarely cured by surgery and may follow an indolent, albeit progressive and malignant course.…”

Section: Step 3 – Manage Tumour Syndromes Identified By Screeningmentioning

confidence: 99%

How should the patient with multiple endocrine neoplasia type 1 (MEN 1) be followed?

Burgess

2009

Clinical Endocrinology

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SummaryMultiple endocrine neoplasia type 1 (MEN 1) is a complex multisystem disease manifesting a diverse range of primary and secondary metabolic and neoplastic disorders. It is possible to improve patient prognosis by early disease detection and treatment. Regular biochemical and radiological screening for parathyroid, gastroenteropancreatic, pituitary, intrathorasic and adrenal lesions forms the basis of surveillance. The likelihood of adverse sequelea such as renal and bone disease resulting from hyperparathyroidism, severe peptic ulceration and gastric carcinoidosis secondary to hypergastrinaemia can be ameliorated by early detection and management.

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Section: Step 3 – Manage Tumour Syndromes Identified By Screeningmentioning

confidence: 99%

How should the patient with multiple endocrine neoplasia type 1 (MEN 1) be followed?

Burgess

2009

Clinical Endocrinology

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“…Omeprazole-induced hypergastrinaemia in man is inhibited by octreotide (Meijer et al 1993), and the hypergastrinaemia in patients with multiple endocrine neoplasia type 1 is also reduced (Burgess et al 1999).…”

Section: Discussionmentioning

confidence: 98%

“…Somatostatin analogues are successfully used in symptomatic treatment of neuroendocrine gastroenteropancreatic tumours (Burgess et al 1999). The presence of somatostatin receptors in these tumours represents a basis for both treatment and imaging (Scherubl et al 1993).…”

Section: Introductionmentioning

confidence: 99%

Spontaneous enterochromaffin-like cell carcinomas in cotton rats (Sigmodon hispidus) are prevented by a somatostatin analogue.

Fossmark

Martinsen²,

Torp³

et al. 2004

Endocr Relat Cancer

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Among inbred female cotton rats (Sigmodon hispidus) 25-50% of the animals develop spontaneous gastric carcinomas; the corresponding figure for male cotton rats is approximately 1%. Animals with carcinomas have hypergastrinaemia and gastric hypo-anacidity and the tumours are derived from enterochromaffin-like (ECL) cells. The mechanism behind the hypo-anacidity is unknown. Carcinomas are found in all female cotton rats with hypergastrinaemia lasting more than 4 months and this represents an excellent animal model for studying gastric carcinogenesis. In this study, the somatostatin analogue octreotide was given to female cotton rats to prevent carcinoma development caused by hypergastrinaemia. Twelve female cotton rats were given monthly injections of long-acting octreotide (5 mg i.m.) for 6 months. A control group of 20 animals was not given injections. Of the 20 control animals, 13 developed hypergastrinaemia and histologically invasive carcinomas or dysplasia. Of the 12 animals in the octreotide group, five developed hypergastrinaemia. None of these five animals developed histological cancer ðP < 0:05Þ; whereas three had dysplasia. However, octreotide did not affect plasma gastrin concentration or antral gastrin mRNA abundance significantly. Dysplasia of the oxyntic mucosa in hypergastrinaemic animals was accompanied by a marked increase in chromogranin A-immunoreactive cells and cells positive for Sevier-Munger staining. The malignant tissue also contained groups of cells with Sevier-Munger staining. In conclusion, octreotide prevented ECL cell carcinomas in hypergastrinaemic cotton rats without lowering the gastrin concentration.

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“…For example octreotide is a safe and effective adjunct to surgical strategies for the management of GEP neoplasia in hypergastrinemic MEN-1 patients [25]. Despite that surgery should be offered when NETs are resectable and there is curative intent (or when debulking offers palliation) [26].…”

Section: Discussionmentioning

confidence: 99%

Multiple endocrine neoplasia type 1: a case report and review of the literature

et al. 2014

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AbstractMultiple endocrine neoplasia syndrome, type 1 (MEN1) is an underdiagnosed autosomal dominant inherited cancer predisposition syndrome with inter- and intrafamilial variability without a known genotype-phenotype correlation. Disease is caused by mutations in the MEN1 gene located on chromosome 11, but other genes (CDKN1B, AIP) and mechanisms might be involved too. We performed retrospective case series study of MEN1 syndrome patient and his family and present our experience of management of genetically confirmed 9 MEN1 syndrome large family members from Lithuania with novel MEN1 gene mutation (MEN1 exon 6 — c. 879delT (p.Pro293Profs*76)) and delineate its clinical phenotype. At present the diagnosis of MEN1 syndrome must be established by direct mutation testing. MEN1 syndrome patients, their relatives and patients suspected of MEN1 are eligible for mutation testing. Patients with MEN1 have a shorter life expectancy than the general population. MEN1 patients and mutation carriers should be subjected to periodic screening in order to detect manifestations in an early stage. Early genetic diagnosis and subsequent periodic screening is associated with less morbidity and mortality at follow-up. Our study confirmed the absence of genotype-phenotype correlation and showed high intrafamilial clinical expression variability of the MEN1 syndrome.

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Octreotide improves biochemical, radiologic, and symptomatic indices of gastroenteropancreatic neoplasia in patients with multiple endocrine neoplasia type 1 (MEN-1)

Cited by 23 publications

References 27 publications

How should the patient with multiple endocrine neoplasia type 1 (MEN 1) be followed?

How should the patient with multiple endocrine neoplasia type 1 (MEN 1) be followed?

Spontaneous enterochromaffin-like cell carcinomas in cotton rats (Sigmodon hispidus) are prevented by a somatostatin analogue.

Multiple endocrine neoplasia type 1: a case report and review of the literature

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