Neurological Presentation of Giant Pituitary Tumour Apoplexy: Case Report and Literature Review of a Rare but Life-Threatening Condition.

Valentina Puglisi,Giuseppe Lanza,Luisa Vinciguerra,Fiammetta Biasini,Placido Bramanti,Elisabetta Morini

doi:10.3390/jcm11061581

Abstract

Background: Giant pituitary adenomas are benign intracranial tumours with a diameter ≥4 cm. Even if hormonally non-functional, they may still cause local extension, leading to symptoms that include mostly gland dysfunction, mass effects, and, much less frequently, apoplexy due to haemorrhage or infarction. Neurological presentation of giant pituitary tumour apoplexy is even more rare and has not been systematically reviewed. Case Presentation: An 81-year-old woman was admitted to the Emergency Department because of acute onset headache, bilateral visual deficit, and altered consciousness. Computed tomography showed a giant mass lesion (>5.5 cm diameter) expanding upward to the suprasellar cistern, optic chiasm, and third ventricle, over-running the sphenoid sinus, and with lateral invasion of the cavernous sinus. Laboratory investigations revealed central adrenal and hypothyroidism insufficiency, while magnetic resonance imaging confirmed a voluminous suprasellar tumour (~6 cm diameter), with signs of pituitary tumour apoplexy. Neurological manifestations and gland-related deficits improved after hormonal replacement therapy with a high dose of intravenous hydrocortisone, followed by oral hydrocortisone and levo-thyroxine. The patient declined surgical treatment and follow-up visit. Conclusions: Giant pituitary tumour apoplexy is a rare but potentially life-threatening condition. Prompt diagnosis and multidisciplinary management may allow a remarkable clinical improvement, as seen in this case.

Highlights

Giant pituitary adenomas are a subset of benign tumours that present mainly as non-functioning pituitary adenomas (NFPAs) and account for approximately 6–10% of all pituitary tumours [1]
It is worth noting that very rare, apoplexy represents the first clinical manifestation in 80% of cases of previously unknown pituitary adenomas [6] and, among NFPAs, 8% of cases presented with clinical evidence of pituitary apoplexy (PA) [7]
We report here the case of a patient with a rare giant pituitary adenoma (~6 cm diameter) who experienced the even more rare complication of a PA-associated neurological presentation, likely due to an uncontrolled anticoagulation therapy-related haemorrhage

Summary

Introduction

Giant pituitary adenomas are a subset of benign tumours that present mainly as non-functioning pituitary adenomas (NFPAs) and account for approximately 6–10% of all pituitary tumours [1]. It is worth noting that very rare, apoplexy represents the first clinical manifestation in 80% of cases of previously unknown pituitary adenomas [6] and, among NFPAs, 8% of cases presented with clinical evidence of PA [7]. The rapid enlargement of pituitary tumours causes gradual and progressive compression of the adjacent parasellar structures and typically manifests with acute-onset headache, visual defects, cranial nerve palsy, changes in consciousness, and endocrine dysfunctions [8]. A recent single-centre case series of 33 patients with PA as the first manifestation of both functioning adenomas and NFPAs [10] confirmed this condition was rare. Even more rare and not systematically described in the literature is the neurological presentation of a giant pituitary tumour apoplexy. We report such a case and review the literature in order to draw attention to this rare but challenging condition

Case Presentation

Discussion

Neuroimaging Management and Findings

Conclusions