An exceptional presentation of pituicytoma apoplexy: A case report

Abstract

Abstract. Pituicytomas are a rare form of indolent neoplasms, which typically present with visual disturbance and hypopituitarism. Complete resection by a trans-sphenoidal approach is the optimal treatment. Only 80 cases have been described thus far in the current literature and the present case is the first to describe the development of pituitary apoplexy in the context of a pituicytoma. A 77-year-old man presented with fatigue and clinical signs of hypogonadism and a sellar lesion was diagnosed at cerebral magnetic resonance imaging (MRI). A watch-and-wait management was initially decided and 1 year after the initial diagnosis, he presented with a thunderclap headache with images suggestive of pituitary apoplexy. A pituitary adenoma was suspected and an endoscopic resection was decided upon the development of a visual deficit. Pathological analysis established the correct diagnosis of a pituicytoma. Pituicytomas are characterised by dense vascularisation, thus ischaemic and haemorrhagic events may be common. When confronted with a hypervascularised pituitary lesion demonstrating strong contrast enhancement and no abnormal hormonal secretion, one must maintain a high index of suspicion for a pituicytoma. A wide range of differential diagnoses should thus be considered in the context of pituitary apoplexy. IntroductionPituicytomas are rare slow-growing tumours arising from the glial cells of the neurohypophysis and infundibulum (pituicytes). The histogenesis, natural history and prognosis of these lesions still need to be elucidated, as data in the current literature remain scarce.Since the first reported case in 1955 (1), there has been a surge in the number of case reports and small case series describing this rare entity. There are no gender-specific differences in the incidence of pituicytomas, and the average age of presentation is 50 years of age (2). No specific risk factors were identified. These neoplasms generally present with headaches, visual disturbances and manifestations of hypopituitarism (typically sexual dysfunction). The clinical presentation is almost identical to other more frequent sellar lesions, therefore correctly diagnosing these lesions can be challenging. Of about 80 cases described in the literature up until now (3), only one previous case reported an abrupt onset of clinical features and this was due to an intraventricular haemorrhage (4).On imaging, even if pituicytomas present with suspicious radiological peculiarities, their rarity often leads them to be misdiagnosed for more common lesions. Surgical resection represents the mainstay of treatment, and this is typically via an endoscopic endonasal approach (5). However, complete resection may be challenging due to the firm consistency and highly vascularised nature of these tumours.We report a novel case of a pituicytoma presenting as pituitary apoplexy, with the aim to advance a hypothesis to explain this phenomenon. To our knowledge this is the first reported case in the literature that describes the development of pituitary ap...

A Rare Case of Pituicytoma Presenting Cystic Formation

“…Considering this, the apoplexy causing the cyst formation was most likely due to the hemorrhagic transformation or to the intralesional ischemia secondary to the rupture or the occlusion of supplying vessels [ 19 , 20 ].…”

Potential Association Between Anabolic Androgenic Steroid Abuse and Pituitary Apoplexy: A Case Report

“…Pituitary apoplexy (PA) is defined as a hemorrhage or infarction of the pituitary mostly within a pituitary adenoma, associated with intense headache of abrupt onset followed by neurological and endocrinological deterioration with or without altered consciousness, although heterogeneity exists with milder constellations (1,2). It affects 2% to 12% of patients with pituitary neuroendocrine tumors and the majority of cases occur in patients with non-functioning macroadenomas (1,(3)(4)(5). Notably, pituitary apoplexy can be the first presentation of a previously undiagnosed macroadenoma (6).…”