Nesidioblastosis - a Rare Cause of Hypoglycaemia in Adults

Abstract

A case of suspected clinically hormonally active insulinoma in a 48-year-old woman is presented. Despite the lack of features, which might correspond to the insulinoma in radiological examinations, the patient was qualified for a distal subtotal pancreatectomy and then, due to persistent hyperinsulinism, for total pancreatectomy. The insulinoma was found neither in a palpable examination of the pancreas nor in the intraoperative ultrasonic examination. In a histopathological examination supplemented with immunohistochemical tests, nesidioblastosis - a rare cause of hypoglycaemia in adults - was diagnosed.

Key words

Nesidioblastosis - hypoglycaemia - hyperinsulinaemia - pancreatectomy - pancreas

References

• 1 Gotz W, Schucht C, Roth J, Theuring F, Herken R. Endocrine pancreatic tumors in MSV-SV40 large T transgenic mice.  Am J Pathol. 1993;  142 1493-1503
• 2 Jerkins T W, Sacks H S, O'Dorisio T M, Tuttle S, Solomon S S. Medullary carcinoma of the thyroid, pancreatic nesidioblastosis and microadenosis, and pancreatic polypeptide hypersecretion: a new association and clinical and hormonal responses to long-acting somatostatin analog SMS 201 - 995.  J Clin Endocrinol Metab. 1987;  64 1313-1319
• 3 Kaczirek K, Soleiman A, Schindl M, Passler C, Scheuba C, Prager G, Kaserer K, Niederle B. Nesidioblastosis in adults: a challenging cause of organic hyperinsulinism.  Eur J Clin Invest. 2003;  33 488-492
• 4 Kim Y W, Park Y K, Park J H, Lee S M, Lee J, Ko S W, Yang M H. Islet cell hyperplasia of the pancreas presenting as hyperinsulinemic hypoglycemia in an adult.  Yonsei Med J. 2000;  41 426-429
• 5 Kon Y C, Loh K C, Chew S P, Wong D, Yap W M, Lee Y S, Low C H. Hypoglycaemia from islet cell hyperplasia and nesidioblastosis in a patient with type 2 diabetes mellitus - a case report.  Ann Acad Med Singapore. 2000;  29 682-687
• 6 Le Bodic M F, Heymann M F, Lecomte M, Berger N, Berger F, Louvel A, De Micco C, Patey M, De Mascarel A, Burtin F, Saint-Andre J P. Immunohistochemical study of 100 pancreatic tumors in 28 patients with multiple endocrine neoplasia, type I.  Am J Surg Pathol. 1996;  20 1378-1384
• 7 Marks V, Teale J D. Tumours producing hypoglycaemia.  Endocr Relat Cancer. 1998;  5 111-129
• 8 Martignoni M E, Kated H, Stiegler M, Buchler M W, Friess H, Zimmermann A, Schirp U, Nitzsche E U. Nesidioblastosis with glucagon-reactive islet cell hyperplasia: a case report.  Pancreas. 2003;  26 402-405
• 9 Milner R D. Nesidioblastosis unravelled.  Arch Dis Child. 1996;  74 369-372
• 10 Seki S, Ikenoue T, Murakami N, Hokanishi H, Kikuura T, Tokunaga M, Akiyama H, Hayakawa K. Ectopic nesidioblastosis.  Acta Paediatr Jpn. 1990;  32 308-310
• 11 Service F J, Natt N, Thompson G B, Grant C S, van Heerden J A, Andrews J C, Lorenz E, Terzic A, Lloyd R V. Noninsulinoma pancreatogenous hypoglycemia: a novel syndrome of hyperinsulinemic hypoglycemia in adults independent of mutations in Kir6.2 and SUR1 genes.  J Clin Endocrinol Metab. 1999;  84 1582-1589
• 12 Stone D L, Hamosh A, Phillips J A, McKusick V A, Smith M. Nesidioblastosis of pancreas. John Hopkins University online knowledgebase: Online Mendelian Inheritance in Man. 2002: 256450
• 13 Tomaszewska R, Nowak W, Rudnicka-Sosin L, Stachura J. Nesidioblastosis in an adult man - case report.  Pol J Pathol. 1999;  50 43-46
• 14 Ueda Y, Kurihara K, Kondoh T, Okanoue T, Chiba T. Islet-cell hyperplasia causing hyperinsulinemic hypoglycemia in an adult.  J Gastroenterol. 1998;  33 125-128
• 15 van der Wal B C, de Krijger R R, de Herder W W, Kwekkeboom D J, van der Ham F, Bonjer H J, van Eijck C H. Adult hyperinsulinemic hypoglycemia not caused by an insulinoma: a report of two cases.  Virchows Arch. 2000;  436 481-486
• 16 Woo D, Scopes J W, Polak J M. Idiopathic hypoglycaemia in sibs with morphological evidence of nesidioblastosis of the pancreas.  Arch Dis Child. 1976;  51 528-531
• 17 Yeh S P, Wang J S, Wu H, Yu M S, Hsueh E J, Wang Y C. Nesidioblastosis, myelodysplastic syndrome and nodular diabetic glomerulosclerosis in an elderly nondiabetic woman: an autopsy report.  Diabet Med. 1999;  16 437-441
• 18 Zumkeller W. Nesidioblastosis.  Endocr Relat Cancer. 1999;  6 421-428