title: Clinical presentation, recurrence, and survival in patients with neuroendocrine tumors: results from a prospective institutional database.
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.
authors: Department of Medical Oncology, Dana-Farber Cancer Institute, Dana 1250, 450 Brookline Avenue, Boston, MA 02215, USA. monica_ter-minassian1@dfci.harvard.edu
authors: Monica
authors: M
authors: Ter-Minassian
authors: Jennifer A
authors: JA
authors: Chan
authors: Susanne M
authors: SM
authors: Hooshmand
authors: Lauren K
authors: LK
authors: Brais
authors: Anastassia
authors: A
authors: Daskalova
authors: Rachel
authors: R
authors: Heafield
authors: Laurie
authors: L
authors: Buchanan
authors: Zhi Rong
authors: ZR
authors: Qian
authors: Charles S
authors: CS
authors: Fuchs
authors: Xihong
authors: X
authors: Lin
authors: David C
authors: DC
authors: Christiani
authors: Matthew H
authors: MH
authors: Kulke
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D053379:Chromogranin A / Q000097:blood; D016208:Databases, Factual; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000097:blood / Q000175:diagnosis* / Q000453:epidemiology; D011379:Prognosis; D012008:Recurrence; D055815:Young Adult
chemical_list: D053379:Chromogranin A