Pituitary apoplexy constitutes a medical emergency, and all patients presenting with symptoms suggestive of pituitary apoplexy need to be assessed urgently, regarding their pituitary hormone reserve (random cortisol, thyroid function tests, LH, FSH, prolactin, testosterone in men/oestradiol in women, IGF-1, GH), serum electrolytes, renal and liver function, clotting and full blood count (Rajasekaran et al. 2011). Haemodynamic and visual function assessment (visual acuity, visual fields, oculomotor nerves) are mandatory.

MRI is the radiological investigation of choice and has been found to confirm the diagnosis of pituitary apoplexy in over 90 % of the patients. The appearance on different MRI sequences can be used to date the haemorrhage. In the first 1–2 days post apoplexy, intra-parenchymal haemorrhage is hyperintense on T1-weighted images and hypointense on T2-weighted images (Tosaka et al. 2007; Semple et al. 2008). On days 3–15, haemorrhage appears bright on both T1- and T2-weighted images. After day 15, a fluid level within the haemorrhage may be visualized (Semple et al. 2008). CT scan which is the modality commonly available in the acute setting is diagnostic in a lower proportion of patients, although it can identify macroadenomas (Davis et al. 1985; Semple et al. 2008). In Fig. 4.1, CT and MR imaging from our centre’s archive for a patient who presented with classical apoplexy and was found to have a NFPA is shown.

In patients who are haemodynamically unstable, hydrocortisone should be administered after blood sampling for pituitary hormones. Hydrocortisone 100–200 mg as an intravenous bolus is appropriate followed either by 2–4 mg/h by continuous intravenous infusion or by 50–100 mg six hourly by intramuscular injection. Ideally patients should be managed in a Neurosurgical High Dependency Unit, with access to an experienced neurosurgical team and input from endocrinology and ophthalmology. The decision for conservative or surgical management should be taken jointly by neurosurgeons, endocrinologists, ophthalmologists and neuroradiologists in a multidisciplinary manner (Rajasekaran et al. 2011).

In one study, surgery within the first 8 days of presentation was associated with improved visual acuity and visual field defects, compared with surgical management at later stages (Randeva et al. 1999). Surgery by an experienced neurosurgeon in pituitary disease is recommended as opposed to a non-specialist on-call neurosurgical team. Deterioration of the patients visual or neurological function warrants further assessment for surgical intervention (Rajasekaran et al. 2011).

Recurrence of NFPA following apoplexy is reported as 10.8 % (4/37 – a mean follow-up of 5.5 years) (Chen et al. 2010), 11.1 % (all patients had incomplete tumour removal following surgery and mean follow-up 6.6 years) (Pal et al. 2011) and 12.4 % in 185 patients with subclinical pituitary adenoma apoplexy (37.3 % of these patients had a nonfunctioning adenoma – mean follow-up 7.4 years) (Zhang et al. 2009).

Non-apoplectic NFPAs not treated with radiotherapy seem to relapse more often compared with apoplectic nonfunctioning adenomas. Recurrence rates that have been reported include 32 % at a mean of 5.4 years (Turner et al. 1999), 34.8 % at a mean time of 6.1 years in a series of 155 patients with relapse in 20.4 % of cases 20 years or longer after the initial operation (Reddy et al. 2011) and 33.5 % at median follow-up of 4.1 years with overall recurrence rates of 24.4 and 51.5 % at 5 and 10 years, respectively (O’Sullivan et al. 2009). The relapse rate at 5 years was 53 % in those with a residual post-operative extrasellar tumour compared with 20 % with an intrasellar tumour (Reddy et al. 2011). The time to regrowth was shorter for those with an extrasellar remnant post-operatively, 3.3 ± 2.17 years for those with a cavernous sinus remnant and 5.3 ± 3.1 years for those without cavernous involvement. There were only two recurrences observed in those who had no residual tumour on the post-operative scans, one at 5.3 years and the other at 25.8 years (Reddy et al. 2011). Whether a conservative versus a surgical approach in nonfunctioning adenomas pituitary apoplexy alters the rate of recurrence is not known.

Nonfunctioning pituitary adenomas with silent ACTH staining do not seem to relapse more often compared with null-cell adenomas (Bradley et al. 2003; Cho et al. 2010; Reddy et al. 2011), although not all cohort studies have shown that (Cooper et al. 2010). However, when they do relapse their course tends to be more aggressive (Bradley et al. 2003; Cho et al. 2010).