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01-11-2007 | Original Article

Surgical treatment of persistent hyperinsulinemic hypoglycemia (PHH) (insulinoma and nesidioblastosis)

Authors: Daniel Casanova, Manuel G. Polavieja, Angel Naranjo, Fernando Pardo, Fernando Rotellar, Francisco Gonzalez, Cristina Luzuriaga, Sara Regaño, Julio Freijanes

Published in: Langenbeck's Archives of Surgery | Issue 6/2007

Abstract

Background

The persistent hyperinsulinemic hypoglycemia is characterized by clinical symptoms that occur when the blood glucose levels drop below the normal range. Two pathological situations cause this clinical problem: The presence of a tumor of the pancreas secreting excessive amounts of insulin, known as insulinoma, and congenital beta-cell hyperplasia in the pancreas in children and noninsulinoma pancreatogenic hypoglycemia syndrome in adults.

Patients and methods

Clinical characteristic and surgical outcome of a group of 20 patients operated on for this hypoglycemic syndrome; 18 for insulinoma and two for nesidioblastosis in children was studied.

Results

eight of the insulinomas were in the head of the pancreas, two in the body, and the remaining eight in the tail. The surgical technique was enucleation in nine cases, local resection in one case because of suspicious malignancy, and distal pancreatectomy in eight cases. Both children with nesidioblastosis underwent 85% pancreatectomy with splenic preservation. There was no mortality in the study, but three patients developed a low-volume pancreatic fistula after head enucleation.

Conclusions

Negative results in diagnostic localization together with the small size of the insulinoma represent a poor combination for successful surgery. The intraoperative ultrasonography is the method of choice for the identification of the tumor, as it is able to identify nonpalpable lesions.

Service FJ, McMahon MM, O’Brien PC, Ballard DJ (1991) Functioning insulinoma: incidence, recurrence, and long-term survival of patients. A 60-year study. Mayo Clin Proc 66:711–719PubMed

Kaczirek K, Soleiman A, Schindl M, Passler C, Scheuba C, Prager G (2003) Nesidioblastosis in adults: a challenging cause of organic hyperinsulinism. Eur J Clin Invest 33:488–492PubMedCrossRef

Starke A, Saddig C, Kirch B, Tschahargane C, Goretzki P (2006) Islet hyperplasia in adults: challenge to preoperatively diagnose non-insulinoma pancreatogenic hypoglycemia syndrome. World J Surg 30(5):670–679PubMedCrossRef

Van Heerden JA, Thomson GB (1993) Islet tumours of the pancreas. In: Trede M, Carter DC (eds) Surgery of the páncreas (49). Churchill Livingstone, Edinburgh, pp 545–561

Tucker ON, Crotty PL, Conlon KC (2006) The management of insulinoma. Br J Surg 93(3):264–275 (Mar)PubMedCrossRef

Whipple AO, Franz VK (1935) Adenoma of islet cells with hyperinsulinism. A review. Ann Surg 101:1299–1355PubMedCrossRef

Perry RR, Vinik AI (1995) Clinical review: diagnosis and management of functioning islet cell tumors. J Clin Endocrinol Metab 80:2273–2278PubMedCrossRef

Kaczirek K, Ba-Ssalamah A, Schima W, Niederle B (2004) The importance of preoperative localisation procedures in organic hyperinsulinism. Experience in 67 patients. Wien Klin Wochenschr 116(11–12):373–378 (Jun 30)PubMedCrossRef

Bottger TC, Junginger T (1993) Is preoperative radiographic localization of islet cell tumors in patients with insulinoma necessary? World J Surg 17:427–432PubMedCrossRef

10.

Boukhman MP, Karam JM, Shaver J, Siperstein AE, DeLorimier AA, Clark OH (1999) Localization of insulinomas. Arch Surg 134(8):818–822 (Aug)PubMedCrossRef

11.

Angelini L, Bezzi M, Tucci G, Lirici MM, Candiani F, Rubaltelli L, Tremolada C, Tamburrano G, Fegiz G (1987) The ultrasonic detection of insulinomas during surgical exploration of the pancreas. World J Surg 11(5):642–647PubMedCrossRef

12.

Klotter HG, Ruckert K, Kummerle F, Rothmund M (1987) The uses of intraoperative sonography in endocrine tumors of the pancreas. World J Surg 11:635–641PubMedCrossRef

13.

Wayne JD, Tanaka R, Kaplan EL (1997) Insulinomas. In: Clark OH, Duh Q-Y (eds) Textbook of endocrine surgery. Saunders, Philadelphia

14.

Machi J, Sigel B (1993) Intraoperative ultrasonography. In: Trede M, Carter DC (eds) Surgery of the pancreas. Churchill Livingstone, Edinburgh

15.

Norton JA, Sigel B, Baker AR (1985) Localization of an occult insulinoma by intraoperative ultrasound. Surgery 97:381–384PubMed

16.

Zeiger MA, Shawker TH, Norton JA (1993) Uses of intraoperative ultrasonography to localize Islet cell tumors. World J Surg 17:448–454PubMedCrossRef

17.

Orlefors H, Sundin A, Ahlstrom H, Bjurling P, Bergstrom M, Lilja A (1998) Positron emission tomography with 5-hydroxytryptophan in neuroendocrine tumors. J Clin Oncol 16:2534–2541PubMed

18.

Ahlstrom H, Eriksson B, Bergstrom M, Bjurling P, Langstrom B, Oberg K (1995) Pancreatic neuroendocrine tumors: diagnosis with PET. Radiology 195:333–337PubMed

19.

Hirshberg B, Cochran C, Skarulis MC, Libutti SK, Alexander HR, Wood BJ, Chang R, Kleiner DE, Gorden P (2005) Malignant insulinoma: spectrum of unusual clinical features. Cancer 104(2):264–272 (Jul 15)PubMedCrossRef

20.

Brentjens R, Saltz L (2001) Islet cell tumors of the pancreas: the medical oncologist’s perspective. Surg Clin North Am 81:527–542PubMedCrossRef

21.

Power N, Reznek RH (2002) Imaging pancreatic islet cell tumours. Imaging 14:147–159

22.

Ueno N, Tomiyama T, Tano S, Wada S, Aizawa T, Kimura K (1996) Utility of endoscopic ultrasonography with color Doppler function for the diagnosis of islet cell tumour. Am J Gastroenterol 91:772–776PubMed

23.

Rosch T, Lightdale CJ, Botet JF, Boyce GA, Sivak MV, Yasuda K (1992) Localization of pancreatic neuroendocrine tumours by endoscopic ultrasonography. N Engl J Med 326:1721–1726PubMedCrossRef

24.

Schumacher B, Lubke HJ, Frieling T, Strohmeyer G, Starke AAR (1996) Prospective study on the detection of insulinomas by endoscopic ultrasonography. Endoscopy 28:273–276PubMed

25.

Ichikawa T, Peterson MS, Federle MP (2000) Islet cell tumor of the pancreas: biphasic CT versus MR imaging in tumor detection. Radiology 216:163–171PubMed

26.

Fidler JL, Fletcher JG, Reading, Andrews JC, Thompson GB, Grant CS, Service FJ (2003) Preoperative detection of pancreatic insulinomas on multiphasic helical CT. AJR Am J Roentgenol 181:775–780PubMed

27.

Brown CK, Bartlett DL, Doppman JL, Gorden P, Libutti SK, Fraker DL, Shawker TH, Skarulis MC, Alexander HR (1997) Intraarterial calcium stimulation and intraoperative ultrasonography in the localization and resection of insulinomas. Surgery 122(6):1189–1193 (Dec)PubMedCrossRef

28.

Chavan A, Kirchhoff TD, Brabant G, Scheumann GFW, Wagner S, Galanksi M (2000) Role of the intra-arterial calcium stimulation test in the preoperative localisation of insulinomas. Eur Radiol 10:1582–1586PubMedCrossRef

29.

Chayvialle J-A (1996) A comparison of imaging techniques for the localisation of gastro-enteropancreatic neuroendocrine tumours. Digestion 57:54–56PubMedCrossRef

30.

Carneiro DM, Levi JU, Irvin GL (2002) Rapid insulin assay for intraoperative confirmation of complete resection of insulinomas. Surgery 132(6):937–942 (Dec)PubMedCrossRef

31.

O’Riordain DS, O’Brien T, van Heerden JA, Service FJ, Grant CS (1994) Surgical management of insulinoma associated with multiple endocrine neoplasia type I. World J Surg 18:488–493PubMedCrossRef

32.

Jaroszewski DE, Schlinkert RT, Thompson GB, Schlinkert DK (2004) Laparoscopic localization and resection of insulinomas. Arch Surg 139:270–274PubMedCrossRef

33.

Berends FJ, Cuesta MA, Kazemier G, van Eijck CH, de Herder WW, van Muiswinkel JM, Bruining HA, Bonjer HJ (2000) Laparoscopic detection and resection of insulinomas. Surgery 128(3):386–391 (Sep)PubMedCrossRef

34.

Lo CY, Lo CM, Fan ST (2000) Role of laparoscopic ultrasonography in intraoperative localization of pancreatic insulinoma. Surg Endosc 14(12):1131–1135 (Dec)PubMedCrossRef

35.

Pedrazzoli S, Pasquali C, Alfano D’Andrea A (1994) Surgical treatment of insulinoma. Br J Surg 81(5):672–676 (May)PubMedCrossRef

36.

Chen X, Cai WY, Yang WP, Li HW (2002) Pancreatic insulinomas: diagnosis and surgical treatment of 74 patients. Hepatobiliary Pancreat Dis Int 1(3):458–461 (Aug)PubMed

37.

Hirshberg B, Libutti SK, Alexander HR, Bartlett DL, Cochran C, Livi A, Chang R, Shawker T, Skarulis MC, Gorden P (2002) Blind distal pancreatectomy for occult insulinoma, an inadvisable procedure. J Am Coll Surg 194(6):761–764 (Jun)PubMedCrossRef

38.

Grant CS (2005) Insulinoma. Best Pract Res Clin Gastroenterol 19(5):783–798 (Oct)PubMedCrossRef

39.

Soga J, Yakuwa Y, Osaka M (1998) Insulinoma/hypoglycemic syndrome: a statistical evaluation of 1085 reported cases of a Japanese series. J Exp Clin Cancer Res 17:379–388PubMed

40.

Fernandez-Cruz L, Saenz A, Astudillo E, Martinez I, Hoyos S, Pantoja JP (2002) Outcome of laparoscopic pancreatic surgery: endocrine and nonendocrine tumors. World J Surg 26:1057–1065PubMedCrossRef

41.

Mabrut JY, Fernandez-Cruz L, Azagra JS, Bassi C, Delvaux G, Weerts J, Fabre JM, Boulez J, Baulieux J, Peix JL, Gigot JF, Pancreatic Section (HBPS) of the Royal Belgian Society of Surgery, Belgian Group for Endoscopic Surgery (BGES), Club Coelio (2005) Laparoscopic pancreatic resection: results of a multicenter European study of 127 patients. Surgery 137(6):597–605 (Jun)PubMedCrossRef

42.

Sarmiento JM, Que FG, Grant CS, Thompson GB, Farnell MB, Nagorney DM (2002) Concurrent resections of pancreatic islet cell cancers with synchronous hepatic metastases: outcomes of an aggressive approach. Surgery 132:976–982PubMedCrossRef

Title: Surgical treatment of persistent hyperinsulinemic hypoglycemia (PHH) (insulinoma and nesidioblastosis)
Authors: Daniel Casanova
Manuel G. Polavieja
Angel Naranjo
Fernando Pardo
Fernando Rotellar
Francisco Gonzalez
Cristina Luzuriaga
Sara Regaño
Julio Freijanes
Publication date: 01-11-2007
Publisher: Springer-Verlag
Published in: Langenbeck's Archives of Surgery / Issue 6/2007
Print ISSN: 1435-2443
Electronic ISSN: 1435-2451
DOI: https://doi.org/10.1007/s00423-007-0158-5

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Surgical treatment of persistent hyperinsulinemic hypoglycemia (PHH) (insulinoma and nesidioblastosis)

Abstract

Background

Patients and methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Patients and methods

Results

Conclusions

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