Primary mucoepidermoid carcinoma of the esophagus: review of the literature

Mucoepidermoid carcinoma of the esophagus (MECE) is an unusual variant of esophageal cancer composed of a mixture of mucus-secreting adenocarcinoma and squamous cell carcinoma. Its pathological interpretation and nomenclature have altered, and even at the present time, two different theories to account for its histological origin can be considered. Previous reports have indicated that MECE shows aggressive biological behavior and has a poor prognosis. However, because of its rarity, the clinicopathological features have not been fully investigated. We collected 125 reported cases of MECE and analyzed their features. The median patient age was 62 years (range 40–92 years) and a male predominance was noted with a male:female ratio of 3.2:1. MECE preferentially occurs in the middle part of the thoracic esophagus. Lymph node metastasis was present in 53.8 % of advanced (T2–4) cases and 21.4 % of T1 cases. The outcome of MECE appears to have improved significantly during the past two decades (P < 0.01). However, survival period did not differ between patients undergoing surgery alone (n = 65) and those receiving additional chemo/radiotherapy (n = 28, P = 0.43). Cox multivariate regression analysis revealed that the presence of lymph node metastasis (P < 0.01) and male gender (P = 0.01) were independent factors negatively impacting survival.