Congenital hyperinsulinism: intraoperative biopsy interpretation can direct the extent of pancreatectomy

The American Journal of Surgical Pathology
Mariko SuchiEduardo Ruchelli

Abstract

Most cases of congenital hyperinsulinism (HI) manifest as either a diffuse or focal form. Diffuse HI is characterized by the presence of enlarged islet cell nuclei, defined as those occupying an area 3 times larger than the surrounding nuclei, throughout the pancreas, and usually requires near total pancreatectomy. Focal HI contains, within an otherwise normal pancreas with islet cell nuclei of normal size, a focus of adenomatous hyperplasia characterized by endocrine cell overgrowth occupying more than 40% of a given area. This form of HI is amenable to partial pancreatectomy. The current study assesses whether intraoperative frozen section evaluation can distinguish the 2 forms and guide the extent of pancreatectomy. By frozen section analysis, diffuse HI is diagnosed when enlarged islet cell nuclei are present in random intraoperative biopsies from the head, body, and tail of the pancreas. Focal HI is suggested when random biopsies contain no large islet cell nuclei, prompting a further search for a focal lesion. Fifty-two HI patients who underwent pancreatectomy from October 1, 1998 to September 30, 2002 were reviewed. On permanent sections, 18 were classified as diffuse HI, 30 had focal HI, and 4 could not be categorized a...Continue Reading

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Citations

Dec 17, 2005·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Mariko SuchiCharles A Stanley
May 29, 2014·Pediatrics International : Official Journal of the Japan Pediatric Society·Tohru YorifujiHironori Nishibori
Aug 1, 2014·Annals of Pediatric Endocrinology & Metabolism·Tohru Yorifuji
Jun 28, 2020·Journal of Veterinary Internal Medicine·Simon CookChris Scudder
Mar 24, 2007·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Ristan M GreerAndrew M Cotterill
Dec 25, 2007·Clinical Chemistry·Andrew A PalladinoCharles A Stanley
Dec 21, 2006·Nature Clinical Practice. Endocrinology & Metabolism·Diva D De León, Charles A Stanley
May 21, 2010·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Silvana M LovisoloMaria Cláudia N Zerbini
May 3, 2012·Endocrine Practice : Official Journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists·Mary E PattersonJennifer K Yee
Oct 3, 2019·The Journal of Pathology. Clinical Research·Jayne Al HoughtonSarah E Flanagan
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