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. 2013 Mar 22;20(2):187-96.
doi: 10.1530/ERC-12-0340. Print 2013 Apr.

Clinical presentation, recurrence, and survival in patients with neuroendocrine tumors: results from a prospective institutional database

Monica Ter-Minassian  1 , Jennifer A Chan, Susanne M Hooshmand, Lauren K Brais, Anastassia Daskalova, Rachel Heafield, Laurie Buchanan, Zhi Rong Qian, Charles S Fuchs, Xihong Lin, David C Christiani, Matthew H Kulke
Affiliations

Clinical presentation, recurrence, and survival in patients with neuroendocrine tumors: results from a prospective institutional database

Monica Ter-Minassian et al. Endocr Relat Cancer. .

Abstract

The rarity of neuroendocrine tumors (NET) has contributed to a paucity of large epidemiologic studies of patients with this condition. We characterized presenting symptoms and clinical outcomes in a prospective database of over 900 patients with NET. We used data from patient questionnaires and the medical record to characterize presenting symptoms, disease-free survival (DFS), and overall survival (OS). The majority of patients in this database had gastroenteropancreatic NET. The median duration of patient-reported symptoms before diagnosis was 3.4 months; 19.5% reported durations from 1 to 5 years, 2.5% from 5 to 10 years, and 2% >10 years. The median DFS among patients with resected small bowel NET or pancreatic NET (panNET) was 5.8 and 4.1 years respectively. After correcting for left truncation bias, the median OS was 7.9 years for advanced small bowel NET and 3.9 years for advanced panNET. Chromogranin A (CGA) above twice the upper limit of normal was associated with shorter survival times (hazard ratios 2.8 (1.9, 4.0) P<0.001) in patients with metastatic disease, regardless of tumor subtype. Our data suggest that while most NET patients are diagnosed soon after symptom onset, prolonged symptom duration before diagnosis is a prominent feature of this disease. Though limited to observations from a large referral center, our observations confirm the prognostic value of CGA and suggest that median survival durations may be shorter than that reported in other institutional databases.

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Conflict of interest statement

Declaration of Interest

The authors declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported. MH Kulke has served as a consultant to Pfizer, Novartis and Ipsen. CS Fuchs has served as a consultant to Amgen, Sanofi-Aventis, Pfizer, Genentech, Roche and Bayer.

Figures

Figure 1
Figure 1
Figures 1(A–C): Patient-reported symptoms at time of diagnosis Figure 1A. Patient-reported symptoms, entire cohort (N=731) Figure 1B. Symptoms reported by patients presenting with localized disease (N=323) Figure 1C. Symptoms reported by patients with metastatic disease (N=408) Figure 1D. Patient-reported time from initial onset of symptoms to diagnosis (N=393)
Figure 1
Figure 1
Figures 1(A–C): Patient-reported symptoms at time of diagnosis Figure 1A. Patient-reported symptoms, entire cohort (N=731) Figure 1B. Symptoms reported by patients presenting with localized disease (N=323) Figure 1C. Symptoms reported by patients with metastatic disease (N=408) Figure 1D. Patient-reported time from initial onset of symptoms to diagnosis (N=393)
Figure 1
Figure 1
Figures 1(A–C): Patient-reported symptoms at time of diagnosis Figure 1A. Patient-reported symptoms, entire cohort (N=731) Figure 1B. Symptoms reported by patients presenting with localized disease (N=323) Figure 1C. Symptoms reported by patients with metastatic disease (N=408) Figure 1D. Patient-reported time from initial onset of symptoms to diagnosis (N=393)
Figure 1
Figure 1
Figures 1(A–C): Patient-reported symptoms at time of diagnosis Figure 1A. Patient-reported symptoms, entire cohort (N=731) Figure 1B. Symptoms reported by patients presenting with localized disease (N=323) Figure 1C. Symptoms reported by patients with metastatic disease (N=408) Figure 1D. Patient-reported time from initial onset of symptoms to diagnosis (N=393)
Figure 2
Figure 2
Figure 2A. Disease-free survival following resection according to tumor site of origin Figure 2B. Overall survival for patients with metastatic disease according to tumor subtype (analysis limited to patients diagnosed within 1 year of study)
Figure 2
Figure 2
Figure 2A. Disease-free survival following resection according to tumor site of origin Figure 2B. Overall survival for patients with metastatic disease according to tumor subtype (analysis limited to patients diagnosed within 1 year of study)
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