Epidemiology and Retrospective Analysis in Extrapulmonary Neuroendocrine Carcinoma

Objectives: We conducted a retrospective analysis of patients with extrapulmonary neuroendocrine carcinomas (EPNECs) to explore the distribution and overall outcomes by different regimens and their primary sites. Setting: We reviewed the outcomes of one of the largest data sets of patients with extrapulmonary small cell carcinomas (EPSCCs) identified at Allegheny General Hospital located in Pittsburgh, Pennsylvania, USA. Participants: Patients diagnosed with grade 3 EPNECs were retrospectively identified. Primary endpoint and epidemiology: Overall survival (OS) with different treatment regimens was the primary endpoint. Also, epidemiological factors such as risk factors, race, family history of cancer, and associated comorbidities were recorded. Results: OS was 16 months in seven patients who received cisplatin/etoposide chemotherapy and 8.5 months in seven patients with carboplatin/etoposide chemotherapy. The commonest primary site was the gastrointestinal tract (GIT). Smoking history association was observed to be 50%. Merkel cell carcinoma (MCC) patients had significantly better OS. Simultaneously, an extensive form of disease pattern was also noticed in 94.4% of the patients. Significantly, neutropenic sepsis was observed in 71.4% of the patients who were treated with cisplatin/etoposide combination. Conclusion: EPNECs demonstrated a low response rate to chemotherapy and high rates of distant metastases. Conclusively, brain metastases were rare.