Background. Small cell carcinoma of the esophagus is regarded as havin
g a poor prognosis with frequent systemic dissemination. Methods. A re
view of the records and histologic sections of 11 patients with primar
y small cell carcinoma of the esophagus seen in 11 years was undertake
n. They were analyzed and compared with the more common squamous cell
carcinomas and adenocarcinomas. Results. Small cell carcinoma of the e
sophagus constituted 1% of all esophageal tumors and was mainly locate
d at the middle and lower thirds (90%) of the esophagus. Primary treat
ment consisted of tumor resection in five patients (46%), chemotherapy
and radiotherapy in two (18%); surgical bypass in one (9%), radiother
apy after exploratory laparotomy in one (9%), intubation in one (9%),
and no active intervention in one (9%). Two of the five resected tumor
s were Stage IIB disease, and three were Stage III disease. Five of th
e six patients in the non-resection group had distant metastases at pr
esentation (45% of all patients). The median survival of patients who
had chemotherapy (three of whom also had radiotherapy) was 16.7 months
(range, 2.8-72 months) and was 2.2 months (range, 4 days to 9.1 month
s) for those with no chemotherapy. The overall median survival was 3.1
months for all patients. The prognosis was not significantly differen
t from those with squamous cell carcinomas or adenocarcinomas. Conclus
ions. Small cell carcinoma of the esophagus should be regarded as a sy
stemic disease, and multimodality treatment, including chemotherapy, s
hould be used. Surgery may be offered in selected patients to manage l
ocal disease as part of a chemotherapy-based treatment program.