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Primary thyroid-like low-grade papillary adenocarcinomas are extremely rare neoplasms that generally originate in the nasopharynx. It is important that clinicians recognize this disease, as these neoplasms are amenable to surgical resection and carry an excellent prognosis. We present a case with a pedunculated tumor in the roof of the nasopharynx. Complete tumor excision via endonasal approach and electrocauterization of the origin of mass was undergone. Postoperative histopathological examinations and immunohistochemical studies confirmed thyroid-like low-grade papillary adenocarcinoma of the nasopharynx. In addition, we include a review of the literature about this rare entity.

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