Extrapulmonary small cell carcinoma (EPSCC): 10 years' multi-disciplinary experience at Charité.

Background: Extrapulmonary small cell carcinoma (EPSCC) is a rare disease of highly proliferating neuroendocrine tumor cells. Therapy and outcome of EPSCC patients treated at our institution were retrospectively analyzed.

Patients And Methods: The records of all patients diagnosed with EPSCCs between 1998 and 2007 at the Charité Berlin were retrieved. Stage of disease, therapy, treatment response, time to progression and overall survival were assessed.

Results: Twenty-eight patients with EPSCC were identified. Primary tumor sites were genitourinary tract, head and neck, gastrointestinal tract, breast, and unknown primary. Fifteen patients had locoregional (LD) and 13 had distant metastatic disease (ED). Median survival was 43 and 12 months in LD and ED patients. Four out of seven LD patients receiving chemotherapy plus local treatment remained free of disease.

Conclusion: Prolonged survival can be achieved in patients with LD by multimodal therapy. Overall survival in patients with ED is comparable to patients with ED small cell lung cancer.