Insulinomatosis

Anlauf, Martin; Bauersfeld, Juliane; Raffel, Andreas; Koch, Christian A.; Henopp, Tobias; Alkatout, İbrahim; Schmitt, Anja; Weber, Achim; Kruse, Marie Luise; Braunstein, Stefan; Kaserer, Klaus; Brauckhoff, Michael; Dralle, Henning; Moch, Holger; Heitz, Philipp U.; Komminoth, Paul; Knoefel, Wolfram Trudo; Perren, Aurel; Klöppel, Günter

doi:10.1097/pas.0b013e3181874eca

Cited by 74 publications

(59 citation statements)

References 18 publications

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“…Cytology may be helpful, particularly in metastatic disease. In patients without MEN1 but with multiple insulinomas or multiple recurrences, insulinomatosis should be suspected [140]. …”

Section: Histopathology and Genetics Of Functional P-nets [581517mentioning

confidence: 99%

ENETS Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Neoplasms: Functional Pancreatic Endocrine Tumor Syndromes

Jensen¹,

et al. 2012

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“…Cytology may be helpful, particularly in metastatic disease. In patients without MEN1 but with multiple insulinomas or multiple recurrences, insulinomatosis should be suspected [140]. …”

Section: Histopathology and Genetics Of Functional P-nets [581517mentioning

confidence: 99%

ENETS Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Neoplasms: Functional Pancreatic Endocrine Tumor Syndromes

Jensen¹,

et al. 2012

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“…NIPHS in adulthood was called “nesidioblastosis” in the past and is characterized, as in childhood, by an increase in the number and volume of beta cells. Three to five percent of all causes of hyperinsulinaemia are related to NIPHS diagnosed in adulthood [3-5]. Hyperinsulinaemia is a heterogeneous disorder that can be caused by various defects in the regulation of insulin secretion by the pancreatic β-cells [6].…”

Section: Postprandial Hypoglycaemiamentioning

confidence: 99%

Hypoglycaemia related to inherited metabolic diseases in adults

Douillard

Mention

Dobbelaere

et al. 2012

Orphanet Journal of Rare Diseases

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In non-diabetic adult patients, hypoglycaemia may be related to drugs, critical illness, cortisol or glucagon insufficiency, non-islet cell tumour, insulinoma, or it may be surreptitious. Nevertheless, some hypoglycaemic episodes remain unexplained, and inborn errors of metabolism (IEM) should be considered, particularly in cases of multisystemic involvement. In children, IEM are considered a differential diagnosis in cases of hypoglycaemia. In adulthood, IEM-related hypoglycaemia can persist in a previously diagnosed childhood disease. Hypoglycaemia may sometimes be a presenting sign of the IEM. Short stature, hepatomegaly, hypogonadism, dysmorphia or muscular symptoms are signs suggestive of IEM-related hypoglycaemia. In both adults and children, hypoglycaemia can be clinically classified according to its timing. Postprandial hypoglycaemia can be an indicator of either endogenous hyperinsulinism linked to non-insulinoma pancreatogenic hypoglycaemia syndrome (NIPHS, unknown incidence in adults) or very rarely, inherited fructose intolerance. Glucokinase-activating mutations (one family) are the only genetic disorder responsible for NIPH in adults that has been clearly identified so far. Exercise-induced hyperinsulinism is linked to an activating mutation of the monocarboxylate transporter 1 (one family). Fasting hypoglycaemia may be caused by IEM that were already diagnosed in childhood and persist into adulthood: glycogen storage disease (GSD) type I, III, 0, VI and IX; glucose transporter 2 deficiency; fatty acid oxidation; ketogenesis disorders; and gluconeogenesis disorders. Fasting hypoglycaemia in adulthood can also be a rare presenting sign of an IEM, especially in GSD type III, fatty acid oxidation [medium-chain acyl-CoA dehydrogenase (MCAD), ketogenesis disorders (3-hydroxy-3-methyl-glutaryl-CoA (HMG-CoA) lyase deficiency, and gluconeogenesis disorders (fructose-1,6-biphosphatase deficiency)].

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“…However, insulinomas associated with MEN-1 syndrome (affecting approximately 4–10% of MEN-1-syndrome patients) develop earlier and are frequently multicentric [1,18,21–23]. The risk of recurrence is also greater among patients with MEN-1 syndrome (21% at 10 and 20 years) than in those without the syndrome (0–5% at 10 years and 0–7% at 20 years) [1,24,25]. Owing to these characteristics of MEN-1 syndrome-associated insulinomas, simple enucleation and local resections are less likely to be curative.…”

Section: Sporadic Versus Men-1 Syndrome Tumorsmentioning

confidence: 99%

Insulinoma: Pathophysiology, Localization and Management

2010

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Insulinoma is a rare neuroendocrine tumor that causes oversecretion of insulin and, as a result, patients present with symptoms of hypoglycemia. Fortunately, insulinomas are usually benign and solitary, and surgical cure rates are highly favorable. Most of these tumors occur sporadically, but they can also be associated with multiple endocrine neoplasia type-1 syndrome. The diagnosis is confirmed by a supervised fast, and early detection is important. Several preoperative and intraoperative techniques with various success rates have been employed in order to localize the lesion. When technically feasible, tumor enucleation is the procedure of choice; however, a more formal resection may be necessary for certain tumors. In the age of laparoscopy, the role of laparoscopic surgery in the management of insulinomas is continuing to attract attention. This review will discuss the historical background, pathogenesis, diagnosis, localization and management of insulinomas.

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Insulinomatosis

Abstract: Insulinomatosis is characterized by the synchronous and metachronous occurrence of insulinomas, multiple insulinoma precursor lesions, and rare development of metastases, but common recurrent hypoglycemia. This disease differs from solitary sporadic and MEN1-associated insulinomas.

Cited by 74 publications

References 18 publications

ENETS Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Neoplasms: Functional Pancreatic Endocrine Tumor Syndromes

ENETS Consensus Guidelines for the Management of Patients with Digestive Neuroendocrine Neoplasms: Functional Pancreatic Endocrine Tumor Syndromes

Hypoglycaemia related to inherited metabolic diseases in adults

Insulinoma: Pathophysiology, Localization and Management

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