Extrapulmonary small cell carcinoma localized in lymph nodes: Is it a different clinical entity?

Background. Extrapulmonary small cell carcinomas (EPSCC) can clinically progress differently depending on the primary site of disease involvement. This review is focused on patients with small cell carcinoma (SmCC) exclusively localized in a lymph node or in multiple lymph nodes without any evidence of a primary tumor in any other organ. Methods. We searched the period 1980 to 2007 in the PubMed database and idendified 11 publications in the English language presenting at least one case of SmCC. In total 28 individual patients were included in the present study. They were scrutinized in terms of epidemiology, clinical presentation, staging, pathology, etiology, treatment and prognosis. Results. Characteristics such as age, gender and smoking were similar to those seen in other EPSCCs. Median survival was not reached (42+, range, 9.1 to 100 months). The survival rate was found to be 79% at 3 years. Seventy-seven percent of the patients had limited stage disease. These patients completely responded to surgical therapy, chemotherapy, radiotherapy or to a combination of these treatments. Seventy-one percent of the patients with limited stage SmCC localized in lymph nodes were recurrence-free during the study periods. Discussion. Our review patient group with SmCC localized in lymph nodes exhibited an excellent clinical behavior and survival results when compared to other patients with pulmonary and non-pulmonary SmCCs. SmCCs localized in lymph nodes may be a separate clinical entity.