• Remo Accorona, Diego Barbieri, Davide Farina, Davide Lombardi, Mario Bussi, and Piero Nicolai.
• Unit of Otorhinolaryngology - Head and Neck Surgery, University of Brescia, Brescia - Italy.
• Tumori. 2017 Nov 15; 103 (Suppl. 1): e73-e77.

BackgroundCarcinoma ex-pleomorphic adenoma of the parapharyngeal space is an exceedingly rare entity.MethodsThe clinical records of two patients with primary carcinoma ex-pleomorphic adenoma of the parapharyngeal space surgically treated at two different centers were retrospectively analyzed.ResultsDespite ultrasound-guided fine-needle aspiration cytology and magnetic resonance imaging, the correct diagnosis was preoperatively missed in both cases. Excision was obtained through a transcervical-transparotid approach in 1 case and a minimally-invasive endoscopic-assisted transcervical approach in the other. Definitive histology was consistent with intracapsular carcinoma ex-pleomorphic adenoma. Adjuvant radiotherapy was performed in the case with macroscopic malignant degeneration; both patients are alive and free from disease 36 months after surgery.ConclusionsCarcinoma ex-pleomorphic adenoma of the parapharyngeal space is a rare disease, and definitive diagnosis may be challenging. Surgery is the mainstay of treatment, but adjuvant radiotherapy also may be required in the presence of specific risk factors.

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