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Insulinomatosis: a multicentric insulinoma disease that frequently causes early recurrent hyperinsulinemic hypoglycemia.
Am J Surg Pathol. 2009 Mar; 33(3):339-46.AJ

Abstract

BACKGROUND

Multicentric insulinoma disease was characterized with regard to its histopathology, multiple endocrine neoplasia type 1 (MEN1) status, precursor lesions, and the risk of hyperinsulinemic hypoglycemia recurrence.

METHODS

Fourteen patients with multicentric insulinoma disease were compared with 267 patients with sporadic and familial insulinomas. The tumors were classified according to the World Health Organization (WHO) criteria. The MEN1 status was defined clinically and by germline mutation analysis. Detection of the MEN1 gene locus was performed using fluorescence in situ hybridization. The surgical interventions and the duration of disease-free survival were recorded.

RESULTS

Fourteen patients (5%) without evidence of MEN1 showed 53 macrotumors and 285 microtumors expressing exclusively insulin. In addition, they had small proliferative insulin-expressing monohormonal endocrine cell clusters (IMECCs). No allelic loss of the MEN1 locus was detected in 64 tumors. All but one patient had benign disease. Recurrent hypoglycemia occurred in 6/14 patients (11 recurrences; mean time to relapse 8.4 y). Thirteen patients with MEN1 (4.6%) showed 41 insulinomas and 133 tumors expressing islet hormones other than insulin. IMECCs were not detected. Allelic loss of the MEN1 locus was found in 17/19 insulinomas. Recurrent hypoglycemia occurred in 4/13 patients (4 recurrences; mean time to relapse 14.5 y). Solitary insulinomas were found in 254/281 patients (90.4%). IMECCs were absent. There was no recurrent hypoglycemia in 84 patients with benign insulinomas.

CONCLUSIONS

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.

Authors+Show Affiliations

Department of Pathology, University of Kiel, Kiel, Germany.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

19011561

Citation

Anlauf, Martin, et al. "Insulinomatosis: a Multicentric Insulinoma Disease That Frequently Causes Early Recurrent Hyperinsulinemic Hypoglycemia." The American Journal of Surgical Pathology, vol. 33, no. 3, 2009, pp. 339-46.
Anlauf M, Bauersfeld J, Raffel A, et al. Insulinomatosis: a multicentric insulinoma disease that frequently causes early recurrent hyperinsulinemic hypoglycemia. Am J Surg Pathol. 2009;33(3):339-46.
Anlauf, M., Bauersfeld, J., Raffel, A., Koch, C. A., Henopp, T., Alkatout, I., Schmitt, A., Weber, A., Kruse, M. L., Braunstein, S., Kaserer, K., Brauckhoff, M., Dralle, H., Moch, H., Heitz, P. U., Komminoth, P., Knoefel, W. T., Perren, A., & Klöppel, G. (2009). Insulinomatosis: a multicentric insulinoma disease that frequently causes early recurrent hyperinsulinemic hypoglycemia. The American Journal of Surgical Pathology, 33(3), 339-46. https://doi.org/10.1097/PAS.0b013e3181874eca
Anlauf M, et al. Insulinomatosis: a Multicentric Insulinoma Disease That Frequently Causes Early Recurrent Hyperinsulinemic Hypoglycemia. Am J Surg Pathol. 2009;33(3):339-46. PubMed PMID: 19011561.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Insulinomatosis: a multicentric insulinoma disease that frequently causes early recurrent hyperinsulinemic hypoglycemia. AU - Anlauf,Martin, AU - Bauersfeld,Juliane, AU - Raffel,Andreas, AU - Koch,Christian A, AU - Henopp,Tobias, AU - Alkatout,Ibrahim, AU - Schmitt,Anja, AU - Weber,Achim, AU - Kruse,Marie L, AU - Braunstein,Stefan, AU - Kaserer,Klaus, AU - Brauckhoff,Michael, AU - Dralle,Henning, AU - Moch,Holger, AU - Heitz,Philipp U, AU - Komminoth,Paul, AU - Knoefel,Wolfram T, AU - Perren,Aurel, AU - Klöppel,Günter, PY - 2008/11/18/pubmed PY - 2009/3/26/medline PY - 2008/11/18/entrez SP - 339 EP - 46 JF - The American journal of surgical pathology JO - Am J Surg Pathol VL - 33 IS - 3 N2 - BACKGROUND: Multicentric insulinoma disease was characterized with regard to its histopathology, multiple endocrine neoplasia type 1 (MEN1) status, precursor lesions, and the risk of hyperinsulinemic hypoglycemia recurrence. METHODS: Fourteen patients with multicentric insulinoma disease were compared with 267 patients with sporadic and familial insulinomas. The tumors were classified according to the World Health Organization (WHO) criteria. The MEN1 status was defined clinically and by germline mutation analysis. Detection of the MEN1 gene locus was performed using fluorescence in situ hybridization. The surgical interventions and the duration of disease-free survival were recorded. RESULTS: Fourteen patients (5%) without evidence of MEN1 showed 53 macrotumors and 285 microtumors expressing exclusively insulin. In addition, they had small proliferative insulin-expressing monohormonal endocrine cell clusters (IMECCs). No allelic loss of the MEN1 locus was detected in 64 tumors. All but one patient had benign disease. Recurrent hypoglycemia occurred in 6/14 patients (11 recurrences; mean time to relapse 8.4 y). Thirteen patients with MEN1 (4.6%) showed 41 insulinomas and 133 tumors expressing islet hormones other than insulin. IMECCs were not detected. Allelic loss of the MEN1 locus was found in 17/19 insulinomas. Recurrent hypoglycemia occurred in 4/13 patients (4 recurrences; mean time to relapse 14.5 y). Solitary insulinomas were found in 254/281 patients (90.4%). IMECCs were absent. There was no recurrent hypoglycemia in 84 patients with benign insulinomas. CONCLUSIONS: 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. SN - 1532-0979 UR - https://www.unboundmedicine.com/medline/citation/19011561/Insulinomatosis:_a_multicentric_insulinoma_disease_that_frequently_causes_early_recurrent_hyperinsulinemic_hypoglycemia_ L2 - https://doi.org/10.1097/PAS.0b013e3181874eca DB - PRIME DP - Unbound Medicine ER -