Local and systemic treatment in small cell carcinoma of the esophagus

Primary small cell carcinoma of the esophagus is a rare and aggressive disease. We report on our experience with two patients having a small cell cancer of the esophagus, being treated with photodynamic therapy combined with irradiation and induction-chemotherapy as well as a review of literature. Both patients were admitted with severe dysphagia, weight loss and a Karnovsky performance status of 90. Diagnostic work-up revealed tumor-stenosis in the proximal third in one and in the distal third in the other case. Clinical staging showed T4N2M0 and T3N2M0, pure small cell carcinoma. Due to dysphagia and lymph node enlargement, local and systemic therapy were considered as first-line treatment. Restaging after three cycles of induction-chemotherapy revealed partial response in both cases. Esophagectomy as a second-line treatment was considered. However, in the preoperative period, one patient developed motorical aphasia. The CT-scan of the brain showed multiple brain metastases. External beam irradiation and further chemotherapy was initiated. The patient died 12 months after admission. The other patient revealed anatomical inoperability at the staging laparoscopy. External beam irradiation and a second session of PDT was performed. The patient is still alive, 12 months after his first admission. The biological behavior of this aggressive disease and metastases in about 50% of patients at admission, as well as significant dysphagia makes combined systemic and local treatment necessary. Nevertheless, after reviewing the literature, esophagectomy and adjuvant chemotherapy may have an advantage pertaining to survival time when anatomical and functional operability is given.