ESPE Abstracts

Background: Pituitary apoplexy (PA) is a rare clinical emergency in pediatric population. In patients with apoplexy-like symptoms, clinical and imaging features of PA, caused by hemorrhage in a pre-existing macroadenoma, are sometimes difficult to distinguish from Rathke cleft cyst (RCC).

Case presentation: A 14.5-year-old boy, with an uneventful past medical history except for mild COVID-19 infection six months earlier, presented with fatigue, weakness and exercise intolerance lasting for several months. Over the last few weeks he had lost some weight, had nausea and vomited occasionally. Several days prior to admission, he started complaining of occipital and retro-orbital headache. At physical examination he appeared adynamic with Glasgow coma scale (GCS) score 15/15, and without neurological or visual disturbances. He was hypotensive (90/60 mmHg) with bradycardia (46 beats per minute). Patient's linear growth and pubertal development were appropriate for chronological age. At admission blood glucose was 3.9 mmol/L, serum sodium 132 mmol/l. Endocrine testing revealed secondary adrenal insufficiency, central hypothyroidism and hypogonadotropic hypogonadism with significantly elevated prolactin. Pituitary magnetic resonance imaging (MRI) detected a sellar mass with a suprasellar extension (1.6x1.3x1.7 cm), a caudal displacement of anterior pituitary, and slight pituitary stalk and optic chiasm displacement. Radimorphological characteristics of the mass were in accordance with PA. Ophthalmological testing showed no visual deficits. As a part of nonsurgical treatment strategy, hydrocortisone was immediately introduced, followed by levothyroxine and testosterone, which led to rapid clinical improvement, although excessive fatigue persisted despite ongoing hormone replacement therapy. Follow up MRIs after 3 and 9 months revealed no changes in size of the lesion, while the signal intensities appeared more heterogeneous on T2-weighted imaging, with a visible posterior hypointense area, which is suggestive of a RCC.

Conclusion: RCC may mimic PA, presenting with sudden onset of headache, endocrine dysfunction and, more rarely, visual or neurological disturbances. At the time of diagnosis, pituitary MRI may not be able to differentiate between PA and RCC. In the absence of visual or neurological deficits, conservative therapeutic approach is a valid option. Prompt introduction of corticosteroid therapy is required in patients with signs or symptoms suggestive of adrenal insufficiency. Substitution of other deficient hormones should be employed as needed. In non-surgically treated patients, repeated imaging can provide clues for more accurate distinction between RCC and PA.