Extrapulmonary Neuroendocrine Carcinomas: Current Management and Future Perspectives.

Area of Science:

• Oncology
• Pathology

Background:

• Neuroendocrine carcinomas (NECs) are aggressive neoplasms, with extrapulmonary sites (EP-NECs) being rare.
• Gastroenteropancreatic (GEP) and genitourinary (GU) tracts are common sites for EP-NECs.
• Limited evidence exists for EP-NEC treatment, necessitating a multidisciplinary approach.

Purpose of the Study:

• To provide an overview of EP-NEC pathology.
• To review current treatment options for EP-NECs.
• To highlight unmet needs in EP-NEC management.

Main Methods:

• Literature review of pathology and treatment of EP-NECs.
• Analysis of current standard of care (platinum-based chemotherapy).
• Exploration of ongoing clinical trials for novel therapies.

Main Results:

• Platinum-based chemotherapy is the current standard for EP-NECs, similar to small-cell lung cancer (SCLC).
• Targeted therapies, immune checkpoint inhibitors (ICIs), and radionuclide therapy are under investigation.
• EP-NECs represent rare 'orphan diseases' with limited evidence-based literature.

Conclusions:

• A multidisciplinary approach is crucial for managing EP-NEC patients.
• Emerging therapies show promise for breakthrough treatment options.
• Further research is needed to address the unmet needs in EP-NEC care.