Exp Clin Endocrinol Diabetes 2013; 121 - P57
DOI: 10.1055/s-0033-1336764

Clinical outcome of poorly differentiated (neuro)-endocrine carcinomas (NEC-G3) in a multi-center cohort from Germany

S Maasberg 1, S Klose 2, F Weber 3, C Metzner 4, D Hörsch 5, M Schott 6, MM Weber 7, C Auernhammer 8, UF Pape 1 P Goretzki 9, im Namen des Deutschen Registers für neuroendokrine Tumore (NET-Register)
  • 1Universitätsmedizin Charite, Med. Klinik m. Schwerpunkt Hepatologie und Gastroenterologie, Berlin, Germany
  • 2Otto von Guericke Universitätsklinikum, Klinik f. Endokrinologie und Stoffwechselkrankheiten, Magdeburg, Germany
  • 3Universitätsklinikum Essen, Klinik f. Allgemein-, Viszeral- und Transplantationschirurgie, Essen, Germany
  • 4Universitätsklinikum Heidelberg, Innere Klinik I, Endokrinologie und klinische Chemie, Heidelberg, Germany
  • 5Zentralklinik Bad Berka GmbH, Klinik f. Innere Medizin, Gastroenterologie und Endokrinologie, Bad Berka, Germany
  • 6Universitätsklinikum der Heinrich Heine Universität, Klinik f. Endokrinologie und Diabetologie, Düsseldorf, Germany
  • 7Universitätsmedizin Mainz, I. med. Klinik u. Poliklinik, Schwerpunkt Endokrinologie u. Stoffwechselerkrankungen, Mainz, Germany
  • 8Universitätsklinikum der Ludwig Maximilian Universität, med. Klinik u. Poliklinik II, München-Großhadern, Germany
  • 9Lukaskrankenhaus Neuss, chirurgische Klinik I, Neuss, Germany

Introduction: Poorly differentiated (neuro)-endocrine carcinomas (NEC-G3) comprise a small but highly malignant subgroup of neuroendocrine neoplasms (NEN). The overall long-term outcome is poor and treatment options are limited.

Aims and methods: The German-NET-registry retro- and prospectively collects data from histologically proven NEN throughout Germany with initial diagnosis since 1999. From this multicentric cohort all NEC-G3-pts were identified. Histopathological parameters and clinical data as well as information on overall and NET-specific survival were obtained and statistically analysed with regard to prognosis.

Results: Of 2358 cases registered 210 NEC-G3-cases (9%) were identified. 83 (39.5%) were female and the median age was 61 years at initial diagnosis (ID). The main primary tumour localisation was in the pancreas (30%), eosophagus (5.2%), stomach (7.1%), small intestine (4.8%), colon (7.6%) or unknown (24.8%). 9 hormone hypersecretion syndromes were documented (carcinoid: 5, insulinoma: 1, Zollinger-Ellison: 1, atypical carcinoid: 1). During a median observation period of 10 months 95 (45.2%) pts died and the 1-, 2-, and 5-year survival rates (YSR) were 64%, 50%, and 35%, which differed significantly from well differentiated neuroendocrine tumours (NET-G1/G2, p < 0.001). At ID 54 pts (26%) had no distant metastasis reported (limited disease, LD), while 156 pts (74%) presented with distant metastasis (extensive disease, ED). Outcome of LD-pts was significantly better than in ED-pts (p < 0.001). Surgery and chemotherapy were the main therapeutic options, especially as 1st-line therapy. Pts. with surgery had a better prognosis than pts without surgical therapy (median survival 35 vs. 12 months, p < 0.001).

Conclusion: NEC-G3 is a rare subgroup of NEN with highly malignant behaviour and poor overall survival especially when compared with well differentiated NET. Prognosis is significantly influenced by metastasis at ID and surgical therapeutic approach.