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Non-functional Neuroendocrine Carcinoma of the Pancreas: Incidence, Tumor Biology, and Outcomes in 2,158 Patients

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Journal of Gastrointestinal Surgery Aims and scope

Abstract

Objective

Pancreatic neuroendocrine cancer is a rare, indolent malignancy with no effective systemic therapy currently available. This population-based analysis evaluated the hypothesis that long-term survival benefit is greater with aggressive, rather than limited, surgical therapy.

Methods

Non-functional pancreatic neuroendocrine carcinoma (NF-pNEC) cases diagnosed from 1973 to 2004 were retrieved from the SEER database.

Results

A total of 2,158 patients with NF-pNEC were identified, representing 2% of all pancreatic malignancies. The annual incidence increased from 1.4 to 3.0 per million during the study period. On average, tumors measured 59 ± 35 mm (median 50), and age at diagnosis was 59 ± 15 years; 29% of patients were younger than 50. Nodal (44%) and systemic metastases (60%) were common. Overall the 5-, 10-, and 20-year survival rates were 33%, 17%, and 10%, respectively. Removal of the primary tumor significantly prolonged survival in the entire cohort (median 1.2 vs. 8.4 years; p < 0.001) and among those with metastases (median 1.0 vs. 4.8 years; p < 0.001). No survival difference was seen between enucleation and resection of the primary tumor (median 10.2 versus 9.2 years, p = 0.456).

Conclusion

This study suggests that surgical therapy improves survival among patients with localized, as well as metastatic, NF-pNEC. Enucleation may be oncologically equivalent to resection.

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References

  1. Pape UF, Bohmig M, Berndt U, Tiling N, Wiedenmann B, Plockinger U. Survival and clinical outcome of patients with neuroendocrine tumors of the gastroenteropancreatic tract in a german referral center. Ann N Y Acad Sci 2004;1014:222–233.

    Article  PubMed  Google Scholar 

  2. Modlin IM, Lye KD, Kidd M. A 5-decade analysis of 13,715 carcinoid tumors. Cancer 2003;97:934–959.

    Article  PubMed  Google Scholar 

  3. Rindi G, Kloppel G, Alhman H, Caplin M, Couvelard A, de Herder WW, Erikssson B, Falchetti A, Falconi M, Komminoth P, Korner M, Lopes JM, McNicol AM, Nilsson O, Perren A, Scarpa A, Scoazec JY, Wiedenmann B. TNM staging of foregut (neuro)endocrine tumors: a consensus proposal including a grading system. Virchows Arch 2006;449:395–401.

    Article  CAS  PubMed  Google Scholar 

  4. Panzuto F, Nasoni S, Falconi M, Corleto VD, Capurso G, Cassetta S, Di Fonzo M, Tornatore V, Milione M, Angeletti S, Cattaruzza MS, Ziparo V, Bordi C, Pederzoli P, Delle Fave G. Prognostic factors and survival in endocrine tumor patients: comparison between gastrointestinal and pancreatic localization. Endocr Relat Cancer 2005;12:1083–1092.

    Article  PubMed  Google Scholar 

  5. Klőppel G, Perren A, Heitz PU. The gastroenteropancreatic neuroendocrine cell system and its tumors: the WHO classification. Ann N Y Acad Sci 2004;1014:13–27.

    Article  PubMed  Google Scholar 

  6. Ferrone CR, Tang LH, Tomlinson J, Gonen M, Hochwald SN, Brennan MF, Klimstra DS, Allen PJ. Determining prognosis in patients with pancreatic endocrine neoplasms: can the WHO classification system be simplified? J Clin Oncol 2007;25:5609–5615.

    Article  PubMed  Google Scholar 

  7. NCI. Surveillance, Epidemiology, and End Results (SEER) Program Limited-Use Data (1973–2004), National Cancer Institute, DCCPS, Surveillance Research Program, Cancer Statistics Branch, based on the November 2006 submission. In. April 2007 ed; 2007.

  8. Yu F, Venzon DJ, Serrano J, Goebel SU, Doppman JL, Gibril F, Jensen RT. Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome. J Clin Oncol 1999;17:615–630.

    CAS  PubMed  Google Scholar 

  9. Phan GQ, Yeo CJ, Hruban RH, Littemoe KD, Pitt HA, Cameron JL. Surgical experience with pancreatic and peripancreatic neuroendocrine tumors: review of 125 patients. J Gastrointest Surg 1998;2:473–482.

    Article  CAS  PubMed  Google Scholar 

  10. Schindl M, Kaczirek K, Kaserer K, Niederle B. Is the new classification of neuroendocrine pancreatic tumors of clinical help? World J Surg 2000;24:1312–1318.

    Article  CAS  PubMed  Google Scholar 

  11. Thompson GB, van Heerden JA, Grant CS, Carney JA, Ilstrup DM. Islet cell carcinomas of the pancreas: a twenty-year experience. Surgery 1988;104:1011–1017.

    CAS  PubMed  Google Scholar 

  12. Kent RB 3rd, van Heerden JA, Weiland LH. Nonfunctioning islet cell tumors. Ann Surg 1981;193:185–190.

    Article  PubMed  Google Scholar 

  13. Bilimoria KY, Tomlinson JS, Merkow RP, Stewart AK, Ko CY, Talamonti MS, Bentrem DJ. Clinicopathologic features and treatment trends of pancreatic neuroendocrine tumors: analysis of 9,821 patients. J Gastrointest Surg 2007;11:1460–1467. discussion 7–9.

    Article  PubMed  Google Scholar 

  14. Bettini R, Boninsegna L, Mantovani W, Capelli P, Bassi C, Pederzoli P, Delle Fave GF, Panzuto F, Scarpa A, Falconi M. Prognostic factors at diagnosis and value of WHO classification in a mono-institutional series of 180 non-functioning pancreatic endocrine tumours. Ann Oncol 2008;19:903–908.

    Article  CAS  PubMed  Google Scholar 

  15. Fitzgerald T, Hickner Z, Schmitz M, Seah A, Kort E. Increasing incidence of nonfunctional neuroendocrine tumors of the pancreas. In: ASCO Gastrointestinal Cancers Symposium Orlando, Florida, 2007.

  16. Bloomston M, Muscarella P, Shah MH, Frankel WL, Al-Saif O, Martin EW, Ellison EC. Cytoreduction results in high perioperative mortality and decreased survival in patients undergoing pancreatectomy for neuroendocrine tumors of the pancreas. J Gastrointest Surg 2006;10:1361–1370.

    Article  PubMed  Google Scholar 

  17. Gullo L, Migliori M, Falconi M, Pederzoli P, Bettini R, Casadei R, Delle Fave G, Corleto VD, Ceccarelli C, Santini D, Tomassetti P. Nonfunctioning pancreatic endocrine tumors: a multicenter clinical study. Am J Gastroenterol 2003;98:2435–2439.

    Article  PubMed  Google Scholar 

  18. Solorzano CC, Lee JE, Pisters PW, Vauthey JN, Ayers GD, Jean ME, Gagel RF, Ajani JA, Wolff RA, Evans DB. Nonfunctioning islet cell carcinoma of the pancreas: survival results in a contemporary series of 163 patients. Surgery 2001;130:1078–1085.

    Article  CAS  PubMed  Google Scholar 

  19. Bilimoria KY, Bentrem DJ, Merkow RP, Tomlinson JS, Stewart AK, Ko CY, Talamonti MS. Application of the pancreatic adenocarcinoma staging system to pancreatic neuroendocrine tumors. J Am Coll Surg 2007;205:558–563.

    Article  PubMed  Google Scholar 

  20. Hellman P, Lundstrom T, Ohrvall U, Eriksson B, Skogseid B, Oberg K, Tiensuu Janson E, Akerstrom G. Effect of surgery on the outcome of midgut carcinoid disease with lymph node and liver metastases. World J Surg 2002;26:991–997.

    Article  PubMed  Google Scholar 

  21. Chamberlain RS, Canes D, Brown KT, Saltz L, Jarnagin W, Fong Y, Blumgart LH. Hepatic neuroendocrine metastases: does intervention alter outcomes? J Am Coll Surg 2000;190:432–445.

    Article  CAS  PubMed  Google Scholar 

  22. House MG, Cameron JL, Lillemoe KD, Schulick RD, Choti MA, Hansel DE, Hruban RH, Maitra A, Yeo CJ. Differences in survival for patients with resectable versus unresectable metastases from pancreatic islet cell cancer. J Gastrointest Surg 2006;10:138–145.

    Article  PubMed  Google Scholar 

  23. Musunuru S, Chen H, Rajpal S, Stephani N, McDermott JC, Holen K, Rikkers LF, Weber SM. Metastatic neuroendocrine hepatic tumors: resection improves survival. Arch Surg 2006;141:1000–1004. discussion 5.

    Article  PubMed  Google Scholar 

  24. Norton JA, Warren RS, Kelly MG, Zuraek MB, Jensen RT. Aggressive surgery for metastatic liver neuroendocrine tumors. Surgery 2003;134:1057–1063. discussion 63–65.

    Article  PubMed  Google Scholar 

  25. Touzios JG, Kiely JM, Pitt SC, Rilling WS, Quebbeman EJ, Wilson SD, Pitt HA. Neuroendocrine hepatic metastases: does aggressive management improve survival? Ann Surg 2005;241:776–783. discussion 83–85.

    Article  PubMed  Google Scholar 

  26. Givi B, Pommier SJ, Thompson AK, Diggs BS, Pommier RF. Operative resection of primary carcinoid neoplasms in patients with liver metastases yields significantly better survival. Surgery 2006;140:891–897. discussion 7–8.

    Article  PubMed  Google Scholar 

  27. Falconi M, Mantovani W, Crippa S, Mascetta G, Salvia R, Pederzoli P. Pancreatic insufficiency after different resections for benign tumours. Br J Surg 2008;95:85–91.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of Surgical Oncology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA

    Jan Franko, Linwah Yip, Elizabeth Genovese & A. James Moser

  2. Department of Biostatistics, University of Pittsburgh, Pittsburgh, PA, USA

    Wentao Feng

  3. Department of Surgery, University of Pittsburgh Medical Center, 497 Scaife Hall, Pittsburgh, PA, 15261, USA

    A. James Moser

Authors
  1. Jan Franko
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  2. Wentao Feng
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  3. Linwah Yip
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  4. Elizabeth Genovese
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  5. A. James Moser
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Corresponding author

Correspondence to A. James Moser.

Additional information

Supported by the John F. Fortney Charitable Pancreatic Cancer Research Foundation.

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Franko, J., Feng, W., Yip, L. et al. Non-functional Neuroendocrine Carcinoma of the Pancreas: Incidence, Tumor Biology, and Outcomes in 2,158 Patients. J Gastrointest Surg 14, 541–548 (2010). https://doi.org/10.1007/s11605-009-1115-0

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  • DOI: https://doi.org/10.1007/s11605-009-1115-0

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