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‘The worst headache of my life’
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A 30-year-old man was referred for evaluation of a pituitary mass. The patient reported he was previously well until he developed a severe, unrelenting headache. The headache was described as a generalized constant pain/pressure that was worse upon lying down or with Valsalva maneuver. The headache was so severe that he was unable to sleep for several nights. He reported no other associated symptoms, including neck stiffness, nausea, vomiting, abdominal pain, orthostatic dizziness or vision changes.
Stephanie L. Lee
Elizabeth Herman
Pituitary apoplexy
After 8 days of constant headache, the patient saw his primary care provider. A noncontrast CT of the brain revealed a hyperdensity in the pituitary gland. An MRI scan with gadolinium using a pituitary protocol was ordered to further evaluate this area (Figures 1 and 2). The MRI demonstrated a 2.1 cm x 1.2 cm x 1.6 cm peripherally enhancing sellar mass with suprasellar extension, superior displacement of the infundibulum, and possible mass effect on the optic chiasm most consistent with pituitary adenoma. Within the lesion, there was a T1 hyperintensity that did not enhance with contrast, compatible with hemorrhage. These findings were concerning for intratumoral hemorrhage within a pre-existing pituitary adenoma.
The patient’s headache had resolved at the time of the MRI. Based on the abnormal pituitary, he was referred for an outpatient endocrine evaluation without further treatment.
Upon arrival at the endocrine clinic, the patient was completely asymptomatic. Endocrine testing obtained at 8 a.m. documented normal levels of cortisol 10.3 µg/dL, adrenocorticotropic hormone (ACTH) 13 pg/mL, thyroid-stimulating hormone 1.85 mIU/L, free thyroxine index 1.6, prolactin 8 ng/mL, insulin-like growth factor I 147 ng/mL, alpha subunit 0.1 ng/mL, follicle-stimulating hormone 2.3 mIU/mL, luteinizing hormone 1.3 IU/L and total testosterone 303 ng/dL. Urgent ophthalmology consultation with visual field testing revealed no visual field defects.
Figure 1. Initial T1-weighted MRI scan of the pituitary: (A) coronal pre-gadolinium, (B) coronal post-gadolinium, (C) sagittal pre-gadolinium and (D) sagittal post-gadolinium. A hyperintense area is seen within the pituitary adenoma suggesting an area of hemorrhage (red arrow). The hyperintense appearance of hemorrhage on MRI imaging is not changed after gadolinium infusion (A vs. B and C vs. D). The optic chiasm is indicated with a yellow arrow and shows superior deformity due to the enlarged pituitary gland.
Reprinted with permission from: Elizabeth Herman, MD.
Figure 2. Initial axial MRI scan of the pituitary: (A) post-gadolinium T1-weighted MRI image, (B) T2-weighted MRI image. A hyperintense area is seen within the pituitary adenoma on the T1-weighted image suggesting an area of hemorrhage (red arrow). There is an expected loss of signal in the area of hemorrhage on the T2-weighted image (yellow arrow).
Reprinted with permission from: Elizabeth Herman, MD.
Despite the pituitary apoplexy on MRI, the patient had normal endocrine testing and no visual field defects. Dexamethasone and transsphenoidal resection were considered, but because the patient was asymptomatic he was monitored closely. He was given strict instructions about symptoms that should prompt him to return to the ED. A repeat pituitary protocol MRI with gadolinium after 6 weeks showed the pituitary gland had decreased in size but remained mildly enlarged, measuring 1.6 cm x 0.8 cm x 1.2 cm with residual hyperintensity on T1-weighted images. The optic chiasm showed no residual mass effect (Figure 3).
Characteristic imaging findings
Pituitary apoplexy is an acute, potentially life-threatening clinical syndrome caused by sudden hemorrhage into the pituitary gland. The degree of hemorrhage and edema determine the size of the mass and extent of neurologic symptoms. One case study of pituitary apoplexy reported headache was the most common presenting symptom (97%), followed by nausea (80%) and reduction in visual fields (71%).
Figure 3. 6-week follow-up T1-weighted MRI scan of the pituitary: (A) coronal pre-gadolinium, (B) sagittal pre-gadolinium. The size of the pituitary adenoma (red arrow) has decreased significantly since the initial MRI (Figures 1A and 1C). The optic chiasm does not show the deformity compared with Figure 1 (yellow arrow). Pituitary stalk is represented by the green arrow.
Reprinted with permission from: Elizabeth Herman, MD.
Characteristic findings on CT and MRI can confirm a clinically suspected diagnosis (Table 1). MRI is superior to CT in detecting apoplexy. One clinical study showed CT was diagnostic in only 28% of patients while MRI was diagnostic in 91% of cases. On CT imaging, an apoplexy can appear as an area of hyperdensity corresponding to hemorrhage. Post-contrast, the hemorrhagic area will remain unchanged while the periphery may exhibit ring-like enhancement.
On MRI, pre-gadolinium T1-weighted images are the most useful when looking for hemorrhage. On the pre-gadolinium T1 scan there will be evidence of a pituitary lesion with intralesional areas of high-signal intensity suggesting hemorrhage (Figures 1A and 1C). Post-gadolinium T1-weighted images will typically show less enhancement in a pituitary adenoma when compared with the normal pituitary. The area of hemorrhage in the adenoma will have the same intensity as the pre-gadolinium images. Typically, gadolinium-enhanced images do not add to the diagnosis when hemorrhage is seen on noncontrast imaging. The appearance of hemorrhage on T2-weighted imaging can vary depending on the sequence used. In the T2 images from our patient, hemorrhage is seen as complete signal dropout within the adenoma.
Management controversy
Best management of pituitary apoplexy continues to be debated. Initial management with replacement of deficient hormones is generally agreed upon, but the need and timing for surgery and/or high-dose glucocorticoid therapy remains controversial, especially in patients with neuro-ophthalmic symptoms. Patients without neuro-ophthalmic symptoms, as was seen in our patient, can typically be treated conservatively without surgery or steroids. Our patient remains asymptomatic with normal pituitary function. He will be followed with a repeat MRI in 6 months. There are no plans for transsphenoidal surgery at this time.
References:
- Piotin M, et al. Eur Radiol. 1999;9:918-923.
- Randeva HS, et al. Clin Endocrinol (Oxf). 1999;51:181-188.
- Sibal L, et al. Pituitary. 2004;7:157-163.
For more information:
- Stephanie L. Lee, MD, PhD, ECNU, is an Endocrine Today Editorial Board member. She is Associate Professor of Medicine and Associate Chief, in the Section of Endocrinology, Diabetes and Nutrition at Boston Medical Center. Lee can be reached at Boston Medical Center, 88 E. Newton St., Endocrinology Evans 201, Boston, MA 02118; email: stephanie.lee@bmc.org. Lee reports no relevant financial disclosures.
- Elizabeth Herman, MD, is an endocrinology fellow in the Section of Endocrinology, Diabetes and Nutrition at Boston Medical Center. Herman can be reached at Boston Medical Center, 88 E. Newton St., Endocrinology Evans 201, Boston, MA 02118. She reports no relevant financial disclosures.