Abstract
Esophageal localization of adenoid cystic carcinoma of minor salivary glands is rare; it may occur in the early to mid-sixties' age group and is more frequently encountered in men than women. In the majority of cases, it arises from subepithelial glands of the middle to lower third of the esophagus, a similar distribution as squamous cell carcinoma. Prognosis depends mostly on tumor staging and resectability, which represents the only chance of cure. Due to the extreme rarity of this condition, there is limited experience with systemic therapy for advanced disease. We report a case of a patient with metastatic primary esophageal adenoid cystic carcinoma progressing on platinum- and irinotecan-based regimens, who achieved an objective response with oxaliplatin-based chemotherapy in combination with cetuximab.
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