Neuroendocrine prostate carcinoma-understanding, diagnosing, treating

Neuroendocrine prostate carcinoma (NEPC) is an aggressive variant of prostate cancer characterized by a high proliferation rate, expression of neuroendocrine markers in tissue and serum, androgen-independent tumor growth, and a metastatic pattern that is unusual for prostate cancer. In < 1% of cases, NEPC is already present at diagnosis; most NEPC develop during the course of treatment as a result of a transdifferentiation process. This multifactorial process is determined by various genetic and epigenetic alterations. Escalating treatment to target the androgen receptor signal transduction pathway appears to play a key role. To date, there is no generally recognized treatment standard. In first-line therapy, platinum-based chemotherapy is usually used in combination with etoposide or possibly a taxane. Immunotherapeutic approaches and targeted treatment strategies are currently undergoing clinical development. Due to the still limited evidence, patients should be included in clinical trials and recorded in registries.