Extrapulmonary small cell carcinoma of lymph node: Pooled analysis of all reported cases.

Abstract

To study clinical outcomes and management of lymph nodes extrapulmonary small cell carcinoma (LNEPSCC). Herein, we perform a systematic search of published literature in the PubMed and EMBASE databases for studies describing LNEPSCC. For uniformity of reporting, LNEPSCC was staged as limited if it involved either single lymph node station or if surgery with curative intent had been undertaken. The disease was staged extensive if it involved two or more lymph node regions. The systematic literature review yielded eight descriptions (n = 14) involving cervical, submandibular and inguinal lymph nodes. Eleven (64.7%) patients had limited disease (LD) and six (35.3%) had extensive disease (ED) at presentation. Chemotherapy (n = 6, 35.3%) or surgery (n = 4, 23.5%) were the most common form of treatment given to these patients. Complete response was achieved in 12 (70.6%) of the patients. Median (interquartile range) progression free survival and overall survival was 15 (7-42) mo and 22 (12.75-42) mo respectively. Of the three illustrative cases, two patients each had ED at presentation and achieved complete remission with platinum based combination chemotherapy. LNEPSCC is a rare disease with less than 15 reported cases in world literature. Surgical resection with curative intent is feasible in those with LD while platinum based combination chemoradiation is associated with favorable outcomes in patients with ED. Prognosis of LNEPSCC is better than that of small cell lung cancer in general.