RESUMO
OBJECTIVE: The aim of this study was to review cases of extra-pulmonary small cell carcinoma (EPSCC), including their clinical manifestations and treatment outcomes. METHODS: We retrospectively reviewed the medical records and pathological reports of patients who were diagnosed with EPSCC from 1998 to 2005. RESULTS: Overall 21 EPSCC patients were eligible for this study. The most common primary sites were the gastrointestinal organs and the nasal cavity. Eleven patients (52.3%) had limited disease (LD) and 10 patients (47.7%) had extensive disease (ED). Nine patients underwent radical surgery alone, four received only radical radiation and two received only palliative chemotherapy. Two patients received adjuvant radiation or chemotherapy following surgical resection and one received a combination of all three treatment modalities. Three patients declined specific treatment and were treated with best supportive care. The median overall survival in the ED group was only 3 months (range 1-16 months), compared to 30 months (range 20-61 months) for LD. EPSCC of pancreas demonstrated a favorable clinical outcome with treatment, whereas primary EPSCC of the liver, esophagus and rectum had an aggressive natural history and a poor response to treatment. CONCLUSION: Our report suggests that EPSCC may have a different biology from that of pulmonary small cell carcinoma. When detected at an early stage, EPSCC may have an excellent prognosis with treatment. Additional studies involving more patients with EPSCC are warranted to further define the optimal roles of each treatment modality.
