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Surgical experience with pancreatic and peripancreatic neuroendocrine tumors: Review of 125 patients

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Journal of Gastrointestinal Surgery Aims and scope

Abstract

Neuroendocrine tumors of the pancreas and peripancreatic area are rare entities with a wide spectrum of clinical presentation. This study retrospectively reviews the patients who underwent surgery for these tumors at The Johns Hopkins Hospital from 1949 to 1996, inclusive There were 125 patients (65 males and 60 females) whose mean age was 51 ± 1 years Fifty-eight patients (48%) had nonfunctional tumors, whereas 64 (52%) had functional tumors 35 (55%) insulinomas, 23 (36%) gastrmomas, three (5%) VIP-omas, two (3 %) glucagonomas, and one (1%) ACTHorna All patients with functional tumors presented with appropriate signs and symptoms of hormonal excess, 86% of patients with nonfunctional tumors presented with weight loss, abdominal pain, or jaundice Preoperaüve computed tomography (CT) correctly localized the tumor in 66 (76%) of 87 patients, angiography in 45 (58%) of 78 patients, and CT plus angiography in 54 (79%) of 68 patients Tumors were benign in 60 patients (48%), malignant m 65 patients (52%), and were located in the head, neck, or uncinate process of the pancreas in 54, body in 14, tail in 18, and duodenum in eight The most common operative procedures performed were 50 pancreaacoduodenectomies (40%), 39 distal pancreatectomies (31%), and 21 tumor enucleations (17%) Nine synchronous hepatic resections were performed for métastases Of the evaluable patients, 46 (43%) had postoperative complications, the most common of which were pancreatic fistula (16%), wound infection (15%), and delayed gastric emptying (8%) There were three in-hospital deaths (2 8%) With a mean follow-up of 55 ±6 months, there have been 30 additional deaths, 23 of which were related to disease progression The overall 2-, 5-, and 10-year actuarial survival rates were 82%, 65%, and 47%, respectively The 5-year survival for patients with functional tumors was 77% compared to 52% for those with nonfunctional tumors (P = 0 025), the 5-year survival for patients with benign tumors was 91% compared to 49% for those with malignant tumors (P = 0 0004) By univanate analysis the most powerful predictor of poor outcome for patients with malignant tumors (n = 60) was positive surgiad margins (P = 0 006) This single-institution experience documents low mortality and moderate morbidity for patients treated operatively for pancreatic and penpancreaac neuroendocrine tumors The most favorable outcomes are observed in patients with benign functional tumors and in those with completely resected malignant tumors

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References

  1. Nicholls AG Simple adenoma of the pancreas arising from an island of Langerhans JMed Res 1902,8 385–395

    CAS  Google Scholar 

  2. Wilder RM, Allan FN, Power MH, Robertson HE Carcinoma of the islands of the pancreas Hypennsulirism and hypoglycemia JAMA 1927,89 348–355

    CAS  Google Scholar 

  3. Howland G, Campbell WR, Maltby EJ, Robinson WL Dysmsuknism Convulsions and coma due to islet cell tumor of the pancreas, with operation and cure JAMA 1929,93 674–679

    Google Scholar 

  4. Whipple AO Pancreaacoduodenectomy for islet cell carcinoma A five-year follow-up Ann Surg 1945,121 847–852

    Article  PubMed  CAS  Google Scholar 

  5. Zolhnger RM, Ellison EH Primary peptic ulcerations of the jejunum associated with islet cell tumors of the pancreas Ann Surg 1955,142 709–728

    Article  Google Scholar 

  6. Priest WM, Alexander MK Islet-cell tumour of the pancreas with peptic ulceration, diarrhoea, and hypokalaemia Lancet 1957,2 1145–1147

    Article  Google Scholar 

  7. Verner JV, Morrison AB Islet cell tumor and a syndrome of refractory watery diarrhea and hypokalemia Am J Med 1958,25.374–380

    Article  PubMed  CAS  Google Scholar 

  8. Becker SW, Kahn D, Rothman S Cutaneous manifestations of internal malignant tumors Arch Dermatol Syphilol 1942,45 1069–1080

    Google Scholar 

  9. McGavran MH, Unger RH, Recant L, Polk HC, Kilo C, Levin ME A glucagon-secretmg alpha-cell carcinoma of the pancreas N Engl J Med 1966,274 1408–1413

    Article  PubMed  CAS  Google Scholar 

  10. Ganda OP, Weir GC, Soeldner JS, Legg MA, Chick WL, Patel YC, Ebeid AM, Gabbay KH, Reichlin S ‘Somatostatinoma’ A somatostatin-containing tumor of the endocrine pancreas N Engl J Med 1977,296 963–967

    Article  PubMed  CAS  Google Scholar 

  11. Krejs GJ, Orci L, Cordon JM, Ravazzola M, Davis GR, Raskin P, Collins SM, McCarthy DM, Baetens D, Rubenstein A, Aldor TAM, Unger RH Somatostatinoma syndrome Biochemical, morphologic and clinical features N Engl J Med 1979,301 285–292

    Article  PubMed  CAS  Google Scholar 

  12. Yeo CJ Neoplasms of the endocrine pancreas In Greenfield LJ, Mulholland MW, Oldam KT, Zelenock GB, Lillemoe KD, eds Surgery Scientific Principles and Practice, 2nd ed Philadelphia Lippincott-Raven, 1997, pp 918–929

    Google Scholar 

  13. Yeo CJ, Wang BH, Anthone GJ, Cameron JL Surgical experience with pancreatic islet-cell tumors Arch Surg 1993,128 1143–1148

    PubMed  CAS  Google Scholar 

  14. Udelsman R, Yeo CJ, Hruban RH, Pitt HA, Niederhuber JE, Coleman J, Cameron JL Pancreaticoduodenectomy for selected pancreatic endocrine tumors Surg Gynecol Obstet 1993,177 269–278

    PubMed  CAS  Google Scholar 

  15. Cameron JL, Pitt HA, Yeo CJ, Lillemoe KD, Kaufalan HS, Coleman J One hundred and forty-five consecutive pancreaticoduodenectomies without mortality Ann Surg 1993,217 430–438

    Article  PubMed  CAS  Google Scholar 

  16. Phan GQ, Yeo CJ, Cameron JL, Maher MM, Hruban RH, Udelsman R Pancreaticoduodenectomy for selected periampullary neuroendocrine tumors 50 patients Surgery 1997,122 989–997

    Article  PubMed  CAS  Google Scholar 

  17. Yeo CJ Alanagement of complications following pancreaticoduodenectomy Surg Clin North Am 1995,75 913–924

    PubMed  CAS  Google Scholar 

  18. Rienhoff WF Jr, Lewis D Surgical affections of the pancreas met with in The Johns Hopkins Hospital from 1889 to 1932, including a report of a case of an adenoma of the islands of Langerhans, and a case of pancreatohthiasis Bull Johns Hopkins Hosp 1934,54 386–429

    Google Scholar 

  19. Gordon TA, Burleyson GP, Tielsch JM, Cameron JL The effects of regionalizauon on cost and outcome for one general high-risk surgical procedure Ann Surg 1995,221 43–49

    Article  PubMed  CAS  Google Scholar 

  20. Stark DD, Moss AA, Goldberg HI, Deveney CW, Way L Computed tomography and nuclear magnetic resonance imaging of pancreatic islet cell tumors Surgery 1983,94 1024–1027

    PubMed  CAS  Google Scholar 

  21. Broughan TA, Leslie JD, SotoJM, Hermann RE Pancreatic islet cell tumors Surgery 1986,99 671–678

    PubMed  CAS  Google Scholar 

  22. Thompson GB, van Heerden JA, Grant CS, Carney JA, II-strup DM Islet cell carcinomas of the pancreas A twenty-year experience Surgery 1988,104 1011–1017

    PubMed  CAS  Google Scholar 

  23. Frucht H, Doppman JL, Norton JA, Miller DL, Dwyer AJ, Frank JA, Vînayek R, Maton PN, Jensen RT Gastrinomas Comparison of MR imaging with CT, angiography, and US Radiology 1989,171 713–717

    PubMed  CAS  Google Scholar 

  24. Wise SR, Johnson J, Sparks J, Carey LC, Ellison EC Gastrinoma The predictive value of preoperative localization Surgery 1989,106 1087–1093

    PubMed  CAS  Google Scholar 

  25. Fulton RE, Sheedy PF, Mcllrath DC, Ferns DO Preoperative angiographie localization of insulin-producing tumors of the pancreas AJR 1975,123 367–377

    CAS  Google Scholar 

  26. Rosch T, Lightdale CJ, Botet JF, Boyce GA, Sivak MVJr, Yasuda K, Heyder N, Palazzo L, Dancygier H, Schusdziarra V, Classen M Localization of pancreatic endocrine tumors by endoscopic ultrasonography N Engl J Med 1992,326 1721–1726

    Article  PubMed  CAS  Google Scholar 

  27. Muller MF, Meyenberger C, Bertschinger P, Schaer R, Marîncek B Pancreatic tumors Evaluation with endoscopie US, CT, and MR imaging Radiology 1994,190 745–751

    PubMed  CAS  Google Scholar 

  28. Vinik AI, Delbridge L, Moattari R, Cho K, Thompson N Transhepanc portal vein catherization for localization of in- sulinomas A ten-year experience Surgery 1991,109 1–11

    PubMed  CAS  Google Scholar 

  29. Lamberts SWJ, Bakker WH, Reubi JC, Krenning EP Somatostann-receptor imaging in the localization of endocrine tumors N EnglJ Med 1990,323 1246–1249

    Article  CAS  Google Scholar 

  30. van Eijck CH, Lamberts SW, Lemaire LC, Jeekel H, Bosnian FT, Reubi JC, Bruimng HA, Krenning EP The use of soinatostatin receptor scintigraphy in the differential diagnosis of pancreatic duct cancers and islet cell cancers Ann Surg 1996,224119–124

    Article  PubMed  Google Scholar 

  31. Wu H-S, Little AG Penoperative blood transfusions and cancer recurrence J Clin Oncol 1988,6 1348–1354

    PubMed  CAS  Google Scholar 

  32. Legaspi A, Brennan MF Management of islet carcinoma Surgery 1988,104 1018–1023

    PubMed  CAS  Google Scholar 

  33. Carty SE, Jensen RT, Norton JA Prospective study of aggressive resection of metastatic pancreatic endocrine tumors Surgery 1992,112 1024–1032

    PubMed  CAS  Google Scholar 

  34. Evans DB, Slabber JM, Lee JE, Cleary KR, Ajani JA, Gagel RF, Sellin RV, Fenoglio CJ, Merrell RC, Hickey RC Non-funcnoning islet cell carcinoma of the pancreas Surgery 1993,114 1175–1182

    PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Surgery, The Johns Hopkins Medical Institutions, Baltimore, Md

    Giao Q Phan MD, Charles J Yeo MD. (Professor), Keith D Littemoe MD, Henry A Pitt M D. & John L Cameron M D

  2. Department of Pathology, The Johns Hopkins Medical Institutions, Baltimore, Md

    Ralph H Hruban MD

Authors
  1. Giao Q Phan MD
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  2. Charles J Yeo MD.
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  3. Ralph H Hruban MD
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  4. Keith D Littemoe MD
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  5. Henry A Pitt M D.
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  6. John L Cameron M D
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Supported in part by a grant from the National Institutes of Health (R01-CA56130)

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Phan, G.Q., Yeo, C.J., Hruban, R.H. et al. Surgical experience with pancreatic and peripancreatic neuroendocrine tumors: Review of 125 patients. J Gastrointest Surg 2, 473–482 (1998). https://doi.org/10.1016/S1091-255X(98)80039-5

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  • DOI: https://doi.org/10.1016/S1091-255X(98)80039-5

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