Published online Jun 10, 2016. doi: 10.5306/wjco.v7.i3.308
Peer-review started: February 2, 2016
First decision: March 21, 2016
Revised: March 28, 2016
Accepted: April 14, 2016
Article in press: April 18, 2016
Published online: June 10, 2016
Processing time: 121 Days and 21.2 Hours
AIM: To study clinical outcomes and management of lymph nodes extrapulmonary small cell carcinoma (LNEPSCC).
METHODS: 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.
RESULTS: 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.
CONCLUSION: 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.
Core tip: Extrapulmonary small cell cancer confined to lymph nodes (LNEPSCC) is extremely rare. A systematic literature review yielded 3 index and 14 previous case descriptions. Chemotherapy or surgery was most common treatments given with complete response achieved in 70% of the cases. Surgical resection with curative intent is feasible in those with limited disease. Prognosis of LNEPSCC is better than that of small cell lung cancer in general.