Epidemiology and Retrospective Analysis in Extrapulmonary Neuroendocrine Carcinoma.

ABSTRACT

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.