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Pituitary macroadenoma with apoplexy

Case contributed by Mark Rodrigues
Diagnosis certain

Presentation

Sudden onset headache with reduced vision in the left eye and left ophthalmoplegia

Patient Data

Age: 60 years
Gender: Male

The pituitary fossa is enlarged and remodeled by a heterogeneous mass that extends into the suprasellar cistern. The mass contains areas of increased attenuation. It elevates the optic chiasm. There is possible involvement of the left cavernous sinus. There is no evidence of calcification. No hydrocephalus or subarachnoid hemorrhage.

Mucosal thickening in the adjacent sphenoid sinus. Mild divergent squint, with mild lateral deviation of the left globe relative to the right.

The pituitary fossa is smoothly expanded and remodeled by a heterogeneous mass, which extends into the suprasellar cistern.  The mass shows areas of intrinsic height T1-weighted signal and heterogeneous T2-weighted signal, in keeping with hemorrhage. There are components showing enhancement, as well as nonenhancing, presumably cystic components.  Superiorly, the mass elevates the diaphragma sella and optic chiasm and contacts the cisternal portion of the left optic nerve.  Laterally, the mass protrudes into both cavernous sinuses, more so on the left, but there is no encasement of either internal carotid artery. The normal pituitary gland is not visible separate to the mass.  The infundibulum is elevated and displaced to the right.

There is no aneurysm related to the intracranial portions of the internal carotid arteries or the circle of Willis.

There is diffuse mucosal thickening and enhancement within the sphenoid sinuses, which is likely secondary to the pituitary apoplexy. Similarly, there is thick dural enhancement along the clivus, again presumed related to pituitary apoplexy

Histopathology

Microscopy shows mostly necrotic tissue and hemorrhage together with small areas of viable cells showing a sinusoidal pattern composed of monomorphic epithelioid cells.  A reticulin stain shows expanded nests of cells in these areas.

Immunohistochemistry shows the viable tumor cells express pancytokeratin, very occasional cells showing ACTH and focal expression of FSH.  Alpha subunit, LH, prolactin, TSH, GH are negative.  The proliferation index, shown with MIB1, is low.

Diagnosis

Overall the appearances are of a pituitary adenoma together with pituitary apoplexy in keeping with the clinical impression.  The viable adenoma appears to focally express FSH and is, therefore, a probable gonadotroph cell adenoma.

Pituitary lesion - Probable gonadotroph cell adenoma together with pituitary apoplexy.

Case Discussion

Typical clinical findings (headache, visual loss and ophthalomoplegia) of pituitary apoplexy.

The imaging shows evidence of blood products within an underlying pituitary mass, which has expanded and remodeled the pituitary fossa, in keeping with apoplexy in a pituitary macroadenoma. There is mucsoal thickening in the adjacent sphenoid sinus and localized dural thickening and enhancement, which are also consistent with apoplexy. 

MRI imaging is important to confirm the diagnosis, assess the relationship of the macroadenoma with adjacent structures, such as the chiasm and cavernous sinuses, and exclude an aneurysm.

The patient underwent transsphenoidal decompression which confirmed the diagnosis.

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