Parathyroid Hormone-Related Peptide (PTHrP) Secretion by Gastroenteropancreatic Neuroendocrine Tumors (GEP-NETs): Clinical Features, Diagnosis, Management, and Follow-Up

Context: Only a small number of case reports has been published on patients with PTHrP-hypersecreting metastatic gastroenteropancreatic (GEP) neuroendocrine tumors (NETs). Objective: The objective of this study was to evaluate the clinical, biochemical, and radiological features, management, and treatment outcome of patients with PTHrP-hypersecreting GEP-NETs. Design: Retrospective case series. Setting: Tertiary referral hospital. Main Outcome Measures: Clinical, biochemical, and radiological features were measured, as well as response to therapy and survival. Patients: Ten patients with PTHrP-secreting GEP-NETs (nine pancreatic and one unknown primary) with a median age of 50.4 years (range, 38.3–61.1) were studied. Multiple endocrine neoplasia type 1 patients were excluded. Results: The median follow-up was 57.2 months (range, 11.6–204.5 mo). Median overall survival was 86.0 months. In total, 51 different treatment interventions and combinations were applied. In seven of the 10 patients, somatostatin an...