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Neuroradiology Case of the Week

Case 182

Guruprasad Srinath, MD, and Per-Lennart Westesson, MD, PhD, DDS

Clinical Presentation: An 89-year-old male presented to the hospital with an acute onset of right ocular dryness, lid ptosis, and monocular blindness, as well as temporal pain.

Radiological Findings: Nonenhanced axial CT scan demonstrates hyperdensity within the sella (Fig.1A) with widening of the sella on bony window (Fig.1B), suggestive of long standing intrasellar mass with hemorrhage. Sagittal T1W image shows heterointense intrasellar mass with suprasellar extension (Fig. 2A), compressing the right optic nerve. Gradient echo images revealed a focal hypointensity anteriorly within the pituitary mass, focal hemorrhage (Fig. 2B). Coronal post-contrast images show deviation of the infundibulum to the left (Fig. 3A) and nonenhancing component at the inferior aspect of the adenoma at the site of hemorrhage (Fig. 3B).

      She was electively taken to the operating room and the pituitary mass was resected and specimen submitted for histopathological evaluation.
      On pathology monotonous neoplastic cells with abundant glassy cytoplasm and several foci of frank necrosis was seen. The remaining tumor cells were in a stage of early cell death. Foci of acute inflammation and hemorrhage were seen. Histopathology revealed pluri hormonal pituitary macroadenoma. Special stains performed revealed the cells to be strongly positive for FSH, positive for ACTH and prolactin.

Diagnosis: Pituitary macroadenoma with apoplexy

Discussion: Pituitary apoplexy is an uncommon complication of pituitary adenomas. The syndrome is manifest by an abrupt onset of signs and symptoms associated with infarction or hemorrhage into a preexisting pituitary adenoma. The lesion swells and expands, leading to compression of local suprasellar and parasellar structures. The incidence of pituitary apoplexy ranges from 1 to 20% in surgically verified pituitary adenomas, with a slight male predominance. Most frequently, pituitary apoplexy occurs in previously undiagnosed pituitary adenomas.
      In classical pituitary apoplexy, headache is the commonest presenting symptom and hypertension may be an important predisposing factor. MRI is the imaging method of choice. Transsphenoidal surgery is safe and effective. It is indicated if there are associated abnormalities of visual acuity or visual fields because, when performed within 8 days, it resulted in significantly greater improvement in visual acuity and fields than if surgery was performed after this time.
      The diagnosis may often be confused with aneurysmal subarachnoid hemorrhage or meningitis. When pituitary apoplexy is misdiagnosed and left untreated, the neurological sequelae can be devastating, resulting in permanent blindness, coma, or death.
      Glucocorticoid administration and transsphenoidal decompression remain the mainstay of treatment in this acute event. Radiotherapy is not indicated immediately as the risk of tumor recurrence is small, but careful follow-up initially with annual imaging is indicated in this group.

References:

1. Liu JK, Rovit RL, Couldwell WT. Pituitary apoplexy, Seminars in Neurosurgery 2001; 12:315-320. [Abstract]
2. Randeva HS, Schoebel J, Byrne J, Esiri M, Adams CB, Wass JA. Classical pituitary apoplexy: clinical features, management and outcome. Clin Endocrinol (Oxf). 1999 Aug;51(2):181-8. [Medline]