Purpose: Extrapulmonary small cell carcinoma (ESCC) has been a difficult disease to manage since it was first described > 80 years ago. Definitive treatment recommendations are lacking, and the treatment strategies commonly utilized are extrapolated from our experience with pulmonary small cell carcinoma. A better understanding of this entity will improve our collective management. By pooling our collective knowledge, we will hopefully be able to draw some meaningful conclusions about this disease.
Methods: The University of Kansas tumor registry was reviewed from 1990-2013. Forty-two potential cases of ESCC were identified and the charts reviewed. Of these, 35 cases met the inclusion and exclusion criteria for review. Information gathered included age, gender, smoking status, weight loss, metastatic disease related data, stage, ECOG performance status (PS), treatment received, and survival data.
Results: Patients were evaluated with a variety of primary locations of disease including gastrointestinal (GI) tract, genitourinary (GU) tract, pelvic organs, head and neck, and unknown primary. Several sites of metastatic disease were noted, with 57% and 43% of patients meeting criteria for limited disease and extensive disease, respectively. Chemotherapy, surgery, and radiation were used in several different regimens, with small-cell lung cancer-type regimens incorporating a platinum and etoposide being the most common. Kaplan-Meier survival estimates were used to identify possible prognostic variables including stage, primary site, number of treatment modalities received, use of chemotherapy in limited stage disease, and ECOG PS.
Conclusions: In our review of 35 patients, GI (29%) and GU (31%) tract tumors were the most common primary sites of disease with a male to female predominance and 57% of patients presenting with limited disease. The majority of patients treated with chemotherapy received a platinum/etoposide doublet (74%), and almost half received radiation (43%). Statistically significant prognostic factors included stage, ECOG PS, site of primary disease, use of chemotherapy, and number of treatment modalities used. Additional clinical trials are needed to better understand this rare disorder to further our knowledge of the optimum management approach.
