Circulating tumor markers in patients with neuroendocrine tumors-a clinical perspective

Neuroendocrine tumors (NETs) are uncommon malignancies with a very diverse presentation and prognosis. Patients with NETs frequently have circulating tumor biomarkers that may aid in the diagnosis and help with prognostication. The most commonly used and best studied marker is chromogranin A, which appears to reflect the tumor burden and is useful at the time of diagnosis, and to monitor for recurrence after resection as well as to assess response to systemic therapy. Despite being the best studied marker, chromogranin A has significant limitations. Multiple other biomarkers are in use, but most have not been studied well and need further validation before being recommended for clinical practice. We review both established and novel circulating biomarkers, and highlight some of the limitations of tumor marker use in patients with NETs.