Nasopharingeal polymorphous low-grade adenocarcinoma in a patient with nonfunctioning pituitary macroadenoma

Abstract

Nasopharingeal polymorphous low-grade adenocarcinoma in a patient with non-functioning pituitary macroadenoma. Background: Polymorphous low grade adenocarcinoma (PLGA) is a rare malignant neoplasm of the minor salivary glands occurring in the fifth and sixth decade with a 2:1 female predominance. The nasal cavity is involved in less than 1% of cases. The incidence of clinically recognisable pituitary adenoma is 15 cases/million/year. The prevalence of non-functioning pituitary adenoma (NFPA) is estimated to be 70-90 cases/million. Both types of adenoma represent 20-45% of pituitary tumours.

Case report: The report describes the first case of PLGA associated with NFPA, both incidentally diagnosed. Three months after the exeresis of the NFPA using a trans-sphenoidal approach, an endoscopic resection of the PLGA was performed. No recurrence was observed during a 13 months follow-up.

Conclusions: Complete surgical excision is the preferred management for PLGA. Radiotherapy has not been demonstrated to be effective in treating PLGA. The prognosis is good with a very low rate of metastasis and local recurrence.