MANAGEMENT OF ENDOCRINE DISEASE: Pituitary tumour apoplexy

Abstract

Pituitary tumour apoplexy (PA) is a rare clinical syndrome that occurs as a result of acute haemorrhage and/or infarction within a frequently undiagnosed pituitary tumour. The sudden enlargement of the pituitary mass undergoing PA is responsible for a wide range of acute symptoms/signs (severe headache, visual loss, diplopia, hypopituitarism, impaired consciousness) which, together with the radiological evidence of a pituitary lesion, establish the diagnosis. The optimal care of PA requires involvement of a multidisciplinary team including endocrinologist, neurosurgeon, neuroophthalmologist and the management strategy that depends on the clinical manifestations, as well as the presence of co-morbidities. Prompt surgical decompression is initially indicated in cases with severe or progressive impairment of the visual acuity or the visual fields or with altered mental state and leads to visual and neurological recovery in most of the patients. The patients with mild, stable clinical picture (including those with isolated ocular palsies) can be managed conservatively (support of fluid and electrolyte balance and stress doses of steroids in most cases) with favourable visual and neurological outcome. Frequent reassessment is mandatory because the clinical course can be unpredictable; if progression of symptoms occurs, later elective surgery is indicated and is beneficial, especially in terms of visual outcome. The endocrinological outcome is less favourable, irrespective of the treatment option, with many patients remaining on long-term replacement therapy. Despite the above guidelines, clear proof of optimal outcomes in the form of randomised controlled trials is lacking. Regrowth of the pituitary tumour years after a PA episode is possible and patients require long-term surveillance.

Pituitary Adenoma and Non-acute Headache: Is There an Association, and Does Treatment Help?

“…14,15 Acute headaches lasting less than a week before the diagnosis were excluded since pituitary apoplexy is a known cause of headache and could act as a confounder. 12,16,17 In six patients, a bleeding/apoplexy was seen on the initial MRI, making pituitary apoplexy the likely cause of the acute headache. In one patient an acute sinus infection was the likely cause of the acute headache.…”

Multidisciplinary Management of Pituitary Apoplexy

“…It is estimated that the incidence of intralesional bleeding in a pituitary adenoma is five times higher than that in other intracranial neoplasms [6]. However, apoplexy can also occur in nonadenomatous lesions, such as hypophysitis [7, 8], craniopharyngioma, Rathke's cleft cyst, sellar tuberculoma [9], and sellar metastasis [10], or even in normal pituitary gland, as a consequence of sever hypovolemic events during the delivery or the puerperium (Sheehan's Syndrome) [11, 12]. …”

Pituitary apoplexy: pathophysiology, diagnosis and management

“…Although PA usually occurs in adenomas; it has also been described in non-adenomatous lesions including hypophysitis (11,12), metastasis to pituitary, especially from renal cell carcinoma (13), craniopharyngioma, Rathke's cleft cyst and sellar tuberculoma (14).…”

“…As ACTH deficiency is life-threatening condition, glucocorticoid replacement is recommended and a supraphysiological dose is indicated to control edema on parasellar structures: dexamethasone 8 to 16 mg per day or hydrocortisone 50 mg intravenously every 6 hours (30). Anterior pituitary deficiencies occur in nearly 80% of patients: ACTH in up to 70%, TSH in 50% and gonadotrophin in 75% of cases (14,28,31). Patients with low levels of prolactin exhibit a lower probability of pituitary function recovery after surgery.…”