Neuroendokrine Tumoren des Verdauungstrakts - Daten des deutschen NET-Registers
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Neuroendokrine Tumoren des Verdauungstrakts - Daten des deutschen NET-Registers. / Begum, N; Maasberg, S; Plöckinger, U; Anlauf, M; Rinke, A; Pöpperl, G; Lehnert, H; Izbicki, J R; Krausch, M; Vashist, Y K; Raffel, A; Bürk, C G; Hoffmann, J; Goretzki, P; Pape, U F; Weitere Vertreter des deutschen NET-Registers.
In: ZBL CHIR, Vol. 139, No. 3, 06.2014, p. 276-83.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Neuroendokrine Tumoren des Verdauungstrakts - Daten des deutschen NET-Registers
AU - Begum, N
AU - Maasberg, S
AU - Plöckinger, U
AU - Anlauf, M
AU - Rinke, A
AU - Pöpperl, G
AU - Lehnert, H
AU - Izbicki, J R
AU - Krausch, M
AU - Vashist, Y K
AU - Raffel, A
AU - Bürk, C G
AU - Hoffmann, J
AU - Goretzki, P
AU - Pape, U F
AU - Weitere Vertreter des deutschen NET-Registers
PY - 2014/6
Y1 - 2014/6
N2 - BACKGROUND: Neuroendocrine tumours (NET) are rare and heterogeneous neoplasia. To obtain valid data on epidemiology, diagnostics, therapy, prognosis and risk factors is the aim of the German NET registry.PATIENTS AND METHODS: Data from 2009 histologically proven NET were collected from 35 NET centres between 1999 and 2010. Data collection has been performed prospectively since 2004. Results: Median follow-up was 34.5 months and median age at diagnosis 56.4 years. Primary tumour localisations were pancreas (34.2%), midgut (5.8%), stomach (6.5%), bowel (6.9%), duodenum (4.8%) and neuroendocrine CUP (12.6%). Synchronous metastases were seen in 46% and second malignancies in 12%. From 860 patients, 402 (46.7%) had functional tumours with the following hormone excess syndromes: carcinoid syndrome (19.1%; n = 164), persistent hyperinsulinaemic hypoglycaemia (17.7%; n = 152), Zollinger- Ellison syndrome (7.1%; n = 61), glucagonoma (0.7%; n = 15), Verner-Morrison syndrome (0.4%; n = 8) and somatostatinoma syndrome(0.1%; n = 2). Surgical therapy was performed in 78%, therapy with somatostatin receptor analogues(SSA) in 28%, peptide radioreceptor therapy (PRRT) in 19%, chemotherapy in 18% and interferon therapy in 6.5%. Only surgery was done in 47%, whereas 53% received a second therapy. General mortality rate during follow-up was 14.9%. The tumour-specific survival rates for 2, 5 and 10 years were 94, 85 and 70%. The 5-year survival is dependent on the surgical or non-surgical therapy (82 versus 61%, p < 0.001) and also on the primary tumour site (90/30% for midgut, 85/65% for pancreas, p < 0.001). Grading (G1, G2, G3) based on proliferation index Ki-67 recommended by the ENETS guidelines and WHO classification is highly correlated to the 5-year survival rate (88, 82, 33%, p < 0.001).CONCLUSION: The German NET registry provides valid multicentric data on NET in Germany. Surgical therapy is the most frequent and important therapy with good clinical outcome. In non-resectable, metastatic tumours, systemic therapies are common. Continuation and evaluation of the new WHO and TNM classifications for NET and their therapies will be a future focus of the registry.
AB - BACKGROUND: Neuroendocrine tumours (NET) are rare and heterogeneous neoplasia. To obtain valid data on epidemiology, diagnostics, therapy, prognosis and risk factors is the aim of the German NET registry.PATIENTS AND METHODS: Data from 2009 histologically proven NET were collected from 35 NET centres between 1999 and 2010. Data collection has been performed prospectively since 2004. Results: Median follow-up was 34.5 months and median age at diagnosis 56.4 years. Primary tumour localisations were pancreas (34.2%), midgut (5.8%), stomach (6.5%), bowel (6.9%), duodenum (4.8%) and neuroendocrine CUP (12.6%). Synchronous metastases were seen in 46% and second malignancies in 12%. From 860 patients, 402 (46.7%) had functional tumours with the following hormone excess syndromes: carcinoid syndrome (19.1%; n = 164), persistent hyperinsulinaemic hypoglycaemia (17.7%; n = 152), Zollinger- Ellison syndrome (7.1%; n = 61), glucagonoma (0.7%; n = 15), Verner-Morrison syndrome (0.4%; n = 8) and somatostatinoma syndrome(0.1%; n = 2). Surgical therapy was performed in 78%, therapy with somatostatin receptor analogues(SSA) in 28%, peptide radioreceptor therapy (PRRT) in 19%, chemotherapy in 18% and interferon therapy in 6.5%. Only surgery was done in 47%, whereas 53% received a second therapy. General mortality rate during follow-up was 14.9%. The tumour-specific survival rates for 2, 5 and 10 years were 94, 85 and 70%. The 5-year survival is dependent on the surgical or non-surgical therapy (82 versus 61%, p < 0.001) and also on the primary tumour site (90/30% for midgut, 85/65% for pancreas, p < 0.001). Grading (G1, G2, G3) based on proliferation index Ki-67 recommended by the ENETS guidelines and WHO classification is highly correlated to the 5-year survival rate (88, 82, 33%, p < 0.001).CONCLUSION: The German NET registry provides valid multicentric data on NET in Germany. Surgical therapy is the most frequent and important therapy with good clinical outcome. In non-resectable, metastatic tumours, systemic therapies are common. Continuation and evaluation of the new WHO and TNM classifications for NET and their therapies will be a future focus of the registry.
KW - Adolescent
KW - Adult
KW - Aged
KW - Aged, 80 and over
KW - Combined Modality Therapy
KW - Digestive System Neoplasms
KW - Female
KW - Follow-Up Studies
KW - Hormones, Ectopic
KW - Humans
KW - Male
KW - Middle Aged
KW - Neoplasm Grading
KW - Neuroendocrine Tumors
KW - Prognosis
KW - Registries
KW - Syndrome
KW - Young Adult
U2 - 10.1055/s-0032-1315199
DO - 10.1055/s-0032-1315199
M3 - SCORING: Zeitschriftenaufsatz
C2 - 23042103
VL - 139
SP - 276
EP - 283
JO - ZBL CHIR
JF - ZBL CHIR
SN - 0044-409X
IS - 3
ER -
