Endocrinology Fellow University of New Mexico Hospital Albuquerque, New Mexico, United States
Introduction : Pituitary neuroendocrine tumors (PitNET) typically present with either endocrine and/or neuro-ophthalmologic manifestations. Invasive PitNETs are typically associated with superior and/or lateral extension, which may result in cranial nerve abnormalitiespredominately affecting the optic nerve, obstructive hydrocephalus (rare), and headache (non-specific). Here we describe an unusualcase of invasive PitNETin which the chief complaint (unilateral nasal obstruction) was related to inferior extension into the nasal cavity.
Case(s) Description : A 45-year-old male presented with complaint of difficulty breathing through the right nostril for a year. He had a history of hypertension, class 1 obesity, hyperlipidemia, prediabetes, and obstructive sleep apnea. Left nasal septum deviation was observed on exam. CT scan demonstrated an expansile lesion involving the sphenoid sinuses, sella, and right posterior superior nasal airway. FDG-PET/CT scan was performed for suspected lymphomaand demonstrated metabolic activity of the mass (SUV=9.2) without other foci. MRI confirmed an enhancing sellarmass (4.3 x 3 x 3.1 cm) with invasion of clivus, sphenoid sinus, posterior nasal cavity, and left cavernous sinus. Clinical and laboratory evaluation demonstrated normal thyroid and gonadal function; α-subunit, prolactin, and IGF-1 levels were also normal. Plasma ACTH was elevated (112 pg/mL, reference range 6-50), morning cortisol was 17.5 mg/dL, and 24-hour urinary free cortisol was elevated (114.8 mcg, reference range 4.0-50.0). There were no overt clinical features of cortisol excess.Endonasal biopsy of the posterior nasal mass demonstrated pituitary adenoma of sparsely granulated corticotroph type with immunostaining positive for ACTH and TPIT.
Discussion : Thedifferential diagnosis of invasive obstructing nasal mass is broad and included lymphoma. However, biopsy and immunostaining confirmed pituitary neuroendocrine tumor (PitNET) characterized as sparsely granulated, corticotroph adenoma (TPIT lineage). Invasive PitNET tumors often present with clinical/laboratory evidence of anterior pituitary hormone excess or deficiency, and typically extend superiorly and/or laterally. Inferior extension of PitNET tumors is less common and may result in uncommon clinical presentations, such as CSF leakand, as illustrated in the present case, invasion into sphenoid sinus leading to local obstruction of the nasal passage and difficulty breathing.