Registry of Neuroendocrine Tumors (NET) in Czech Republic After Three Years of Data Collection

Authors: P. Vítek 1;  J. Strenková 2;  E. Sedláčková 1;  J. Barkmanová 1;  J. Mužík 2;  O. Louthan 1
Authors‘ workplace: Kooperativní skupina pro neuroendokrinní nádory o. s., Praha2 Institut bio­statistiky a analýz, MU, Brno 1
Published in: Klin Onkol 2013; 26(4): 271-280
Category: Original Articles


Neuroendocrine tumors are traditionally considered to be “rare” diseases. On contrary, the prevalence of neuroendocrine tumors is high. Therefore, the diagnostics, treatment and follow-up of neuroendocrine tumors are subjected to an evolving interest. There are various specifics of neuroendocrine tumors requiring an appropriate feedback of each intervention i.e. data collection and central data evaluation. The “Cooperative Group for Neuroendocrine Tumors” (KSPNN) has been conducting a nationwide neuroendocrine tumors registry since June 2009. The first data summary after three years is aimed at evaluation of feasibility and data utility.

Material and Methods:
The anonymous data on diagnostics, therapy and follow up of patients with neuroendocrine tumors of any primary site are collected in the registry. The contribution is conditioned by morphologically proven diagnosis according to the current WHO 2010 classification, in earlier cases WHO 2000 classification. The registry is operated by the Institute of Biostatistics and Analyses, Masaryk University (Brno). The initial analysis includes data from June 2009 to October 2012.

Data of a substantial share of neuroendocrine tumor carriers have been collected –  742 subjects with a valid record, i.e. about 14% of presumed prevalence. Moreover, the registry covers nearly one fourth of incidence in the period 2009– 2011. The morphological diagnoses with the sign of nonspecific “neuroendocrine tumors” comprise the majority of records (75%); the most frequent is “carcinoid tumor neuroendocrine tumors”. This results in a clear requirement for more detailed specifications of morphology as well as separation of small‑cell (neuroendocrine) carcinoma posses­sing principal bio­logic differences to neuroendocrine tumors itself. There is an apparent polarity of recorded clinical stages. Both stage I and stage IV comprise 30% of the records. This result is presumably related to how the diagnosis is established, either early and incidentally in initial stage or late with a developed endocrine symptomatology, in advanced stage. There is an evident selection bias. The treatment data reflect current trends, dominance of surgical therapy including reasonable cytoreductive surgery, vast use of somatostatine analogues in advanced disease and persistent position of chemotherapy for high‑grade tumors. The distribution of treatment modalities in the records documents a certain adherence to international treatment standards (ENETS, ESMO, NCCN).

The dynamics of data contributions confirm feasibility of data collection in the registry. The registry reveals a clear requirement for more detailed analyses of bio­psies and more detailed disease morphology classification. In the near future, the registry is aimed to maintain the increasing volume of collected data and to cover the majority of neuroendocrine tumors incidence.

Key words:
neuroendocrine tumor –  carcinoid –  small cell neuroendocrine carcinoma –  registry –  classification WHO 2010 –  somatostatine –  incidence –  prevalence


1. Berge T, Linell F. Carcinoid tumors: Frequency in a defined population during a 12‑year period. Acta Pathol Microbio­l Scand A 1976; 84(4): 322– 330.

2. Lawrence B, Gustafsson BI, Chan A et al. The epidemiology of gastroenteropancreatic neuroendocrine tumors. Endocrinol Metab Clin North Am 2011; 40(1): 1– 18.

3. Ellis L, Shale MJ, Coleman MP. Carcinoid tumors of the gastrointestinal tract: trends in incidence in England since 1971. Am J Gastroenerol 2010; 105(12): 2563– 2569.

4. The NET Alliance [homepage on the Internet]. Prevalence of NET: More common than one might think, 2011. Available from:  http:/ / health‑ care‑professional/ prevalence‑ of‑ nets.jsp.

5. Balmadrid BL, Thomas CM, Coffman CJ et al. Factors associated with survival of veterans with gastrointestinal neuroendocrine tumors. J Cancer Epidemiol. In press 2012; 2012: 986708.

6. Hamilton SR, Aaltonen LA (eds). Pathology and genetics of tumors of the digestive system, World Health Organization Classification of Tumors, Pathology and Geetics of Tumors of the Digestive System. Lyon: IARC 2010.

7. Barkmanová J (ed.). Historie registru neuroendokrinních nádorů v České republice. Praha: Farmakon Press 2007: 5– 7.

8. Oberg K, Akerström G, Rindl G et al. Neuroendocrine gastroenteropancreatic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow‑up. Ann Oncol 2010; 21 (Suppl 5): v223– v227.

9. Oberg K, Hellman P, Kwekkeboom D et al. Neuroendocrine bronchial and thymic tumors: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow‑up. Ann Oncol 2010; 21 (Suppl 5): v220– v222.

10. Hauso O, Gustafsson BI, Kidd M et al. Neuroendocrine tumor epidemiology: contrasting Norway and North America. Cancer 2008; 113(10): 2655– 2664.

11. Moran CA, Suster S, Coppola D et al. Neuroendocrine carcinomas of the lung: a critical analysis. Am J Clin Pathol 2009; 131(2): 206– 221.

12. Gustafsson BI, Kidd M, Chan A et al. Bronchoplumonary neuroendocrine tumors. Cancer 2008; 113(1): 5– 21.

13. The US National Cancer Institute [homepage on the Internet]. Surveillance Epidemiology and End Results (SEER) data base, 1973– 2004. Available from: http:/ / 2007.

14. Fraenkel M, Kim M, Faggiano A et al. The increasing incidence of gastroenteropancreatic neuroendocrine tumors arend the world: a systematic review of the literature. 10th Annual Conference for the Diagnosis and Treatment of Neuroendocrine Tumor Disease, Barcelona 6.– 8. 3. 2013: 47.

15. ÚZIS ČR [internetová stránka]. Ústav zdravotnických informací a statistiky ČR. TNM klasifikace zhoubných nádorů. Česká republika 2011. Dostupné z: http:/ / katalog/ klasifikace/ tnm‑ klasifikace‑ zhoubnych‑ novotvaru.

16. Garcia‑ Carbonero R, Capdevilla J, Crespo‑ Herrero G et al.Incidence, patterns of care and prognostic factors for outcome of gastroenteropancreatic neuroendocrine tumors (GEP‑ NETs): results from the National Cancer Registry of Spain (RGETNE). Ann Oncol 2010; 21(9): 1794– 1803.

17. Feldman JM. Carcinoid tumors and the carcinoid syndrome. Curr Probl Surg 1989; 26(12): 835– 85.

18. Modlin IM, Kidd M, Latich I et al. Current status of gast­rointestinal carcinoids. Gastroenterology 2005; 128(6): 1717– 1751.

19. Bhattacharya S, Toumpanakis C, Burke M et al. Features of carcinoid heart disease identified by 2- and 3- dimensional echocardiography and cardiac MRI. Circ Cardiovasc Imaging 2010; 3(1): 103– 111.

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