Endocrine Abstracts

JOINT109

Introduction: Tolosa-Hunt syndrome (THS) represents a rare inflammatory disease of unknown etiology that affects the cavernous sinus, superior orbital fissure or orbital apex. It most commonly occurs in patients over the age of 40. The clinical presentation is characterized by unilateral headache and paresis of the oculomotor (III), trochlear (IV), and/or abducens (VI) cranial nerves, leading to painful ophthalmoplegia of the ocular muscles. Contrast-enhanced magnetic resonance imaging (MRI) usually reveals lesions in the cavernous sinus corresponding to inflammatory infiltrative changes. Histologically, granulomatous inflammation with fibroblast proliferation, lymphocyte infiltration, and the presence of plasma cells and giant cells is typically observed. Treatment primarily relies on glucocorticoids; however, in the case of recurrence, alternative immunosuppressive therapy or radiotherapy should be considered. THS is a diagnosis of exclusion.

Case Report: A 43-year-old female patient with a history of migrainous headache and an endocrinologically inactive pituitary adenoma noticed a worsening of her vision and diplopia in December 2023. Due to the sudden onset of symptoms and the known sellar lesion, she was urgently examined by an endocrinologist. On physical examination, there was a left-sided abducens nerve (VI) paresis and the development of convergent strabismus. MRI revealed progression of the sellar lesion, necessitating endoscopic transsphenoidal resection. Histological examination of the resected tissue confirmed a PitNET tumor of the corticotropic lineage (T-pit positive), with a secondary finding of granulomatous inflammation with necrosis of unclear etiology. Sarcoidosis, granulomatosis with polyangiitis, mycobacterial infections, and other etiologies were subsequently excluded. The diagnosis of THS was made of exclusion, and it was consistent with the pathological findings.

Therapy: Given the diagnosis of THS, intravenous corticosteroid therapy was initiated, followed by oral corticosteroid therapy, with gradual dose reduction. The patient’s symptoms gradually improved with corticosteroid treatment.

Conclusions: The presented case corresponds to a combined finding of a PitNET and the rare Tolosa-Hunt syndrome.