An esophageal tumor was found during evaluation of severe weight loss and dysphagia in a 65-year-old man. Endoscopic appearance and histology are shown. The treatment is

A. esophagogastrectomy.
B. external beam radiotherapy.
C. immunotherapy.
D. multidrug chemotherapy.
E. neoadjuvant therapy and esophagogastrectomy.

Answer D

Small cell carcinoma of the esophagus is an uncommon tumor, representing less than 1% of esophageal tumors. Patients with small cell carcinoma of the esophagus usually present with significant dysphagia and severe weight loss. Men in their sixth or seventh decades of life with a heavy smoking history are typically afflicted with this lethal esophageal neoplasm. There are many incidental case reports, which are typically anecdotal. However, a recently published meta-analysis is illustrative. Approximately two-thirds of these neoplasms are pure small cell carcinomas, while a third have a mixed histology including adenocarcinoma or squamous cell carcinoma. Approximately half of the tumors reported were limited stage tumors. Univariable analysis showed improved survival in patients less than sixty years in age, patients with tumor less than 5 cm, and in those patients who received systemic chemotherapy. As with small cell carcinoma of the lung, multi-drug chemotherapy is the mainstay of therapy.

Casas F, Ferrer F, Farrus B, Casals J, Biete A. Primary small cell carcinoma of the esophagus: A review of the literature with emphasis on therapy and prognosis. Cancer 1997;80:1366-72.
BACKGROUND. Few studies of patients with esophageal small cell carcinoma (SCC) have been conducted. Choice of treatment remains controversial. METHODS. The authors analyzed data on 199 evaluable esophageal SCC patients, selected from among 230 patients found in the literature, and a data extraction form that recorded 11 features was completed. To allow for the evaluation of prognostic factors that influenced survival, the patients were grouped according to limited stage (LS), which was defined as disease confined to the esophagus, or extensive stage (ES), which was defined as disease that had spread beyond locoregional boundaries. Univariate and multivariate analyses were performed. Treatment was categorized as either local or local with systemic; for the ES cases, the categories were defined as treatment versus no treatment. RESULTS. The tumor site was described in 178 cases (89%). Mean tumor size was 6.1. Pure SCC was found in 137 cases (68.8%), whereas 62 cases (31.2%) showed mixed SCC; 93 (46.7%) were LS, whereas 95 (47.7%) were ES. In 11 cases (5.5%), the stage was not determined. There was a significant difference in survival between patients with LS and those with ES (P < 0.0001). The median survival was 8 months for patients with LS and 3 months for those with ES. Univariate analysis of LS showed 3 significant prognostic factors: age (for patients age #60 years, the median survival was 11 months, whereas for those age >60 years, the median survival was 6 months), tumor size (for those with tumors #5 cm, the median survival was 12 months, whereas for those with tumors >5 cm, the median survival was 4 months), and type of treatment (with local plus systemic treatment, the median survival was 20 months, whereas with local it was 5 months). In multivariate analysis, tumor size (P = 0.007) and type of treatment (P < 0.001) were shown to be independent predictive variables. CONCLUSIONS. Esophageal SCC is an aggressive type of tumor. This study shows that there are significant differences between LS and ES and that in LS, both tumor size and type of treatment are possible prognostic factors.