BACKGROUND: Primary intestinal-like adenocarcinoma of the major salivary glands has not previously been reported. METHODS: The clinical and radiological findings of 2 patients with primary submandibular and sublingual tumors are presented. Histopathologic and immunohistochemical examinations of tumor sections were performed. Metastatic workup for distant occult primary was carried out. RESULTS: The light-optic and the immunomarkers revealed intestinal-like adenocarcinoma consistent with upper respiratory tract origin. Metastatic workup of both patients was negative for primary gastro-intestinal primary. CONCLUSION: Primary intestinal-like adenocarcinoma can develop in major salivary glands and should be considered in the differential diagnosis.
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