Insulinoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

Overview

Insulinoma can occur in association with inherited syndrome, such as multiple endocrine neoplasia type 1 von Hippel-Landau syndrome or sporadic.^[1]

Multiple endocrine neoplasia type 1 is an autosomal dominant disorder caused by alterations of the MEN1 gene located at chromosomal region 11q13.
Approximately 10% of insulinomas are multiple and 10% malignant.^[2]

On microscopic histopathological analysis characteristic findings of insulinoma are:

Solid or gyriform patterns, usually without glands
Endocrine cells in insulinoma are organised in nests and trabecular pattern scattered throughout a fibro-vascular stroma with well-localised fibrous septa separating it from normal tissue^[3]
Absence of exocrine tissue with the loss of normal pancreatic architecture

Histopathology of pancreatic endocrine tumor (insulinoma). H&E stain^[4]
Histopathology of pancreatic endocrine tumor (insulinoma)^[4]
Histopathology of pancreatic endocrine tumor (insulinoma). Chromogranin A immunostain^[4]
Histopathology of pancreatic endocrine tumor (insulinoma). Insulin immunostain^[4]

↑ Murray PD, McKenzie DT, Swain SL, Kagnoff MF (1987). "Interleukin 5 and interleukin 4 produced by Peyer's patch T cells selectively enhance immunoglobulin A expression". J Immunol. 139 (8): 2669–74. PMID 3498768.
↑ Insulinoma. Dr Yuranga Weerakkody and Dr Frank Gaillard et al. Radiopaedia 2015. http://radiopaedia.org/articles/insulinoma
↑ Padidela, R.; Fiest, M.; Arya, V.; Smith, V. V.; Ashworth, M.; Rampling, D.; Newbould, M.; Batra, G.; James, J.; Wright, N. B.; Dunne, M. J.; Clayton, P. E.; Banerjee, I.; Hussain, K. (2014). "Insulinoma in childhood: clinical, radiological, molecular and histological aspects of nine patients". European Journal of Endocrinology. 170 (5): 741–747. doi:10.1530/EJE-13-1008. ISSN 0804-4643.
↑ ^4.0 ^4.1 ^4.2 ^4.3 Neuroendocrine tumour of the pancreas. Libre Pathology. http://librepathology.org/wiki/index.php/Neuroendocrine_tumour_of_the_pancreas