Pituitary apoplexy

Case contributed by Frank Gaillard
Diagnosis certain

Presentation

Rapid visual loss and headache.

Patient Data

Age: 40 years
Gender: Male
ct

The fossa is enlarged, and remodeled and filled with soft tissue, denser on the left. The scout demonstrates prominence of the frontal sinuses, nose and jaw. 

mri

A pituitary macroadenoma is present with a small amount of central high T1 signal consistent with blood. On post-contrast T1 weighted imaging, best seen on coronal, only heterogeneous peripheral enhancement is present. Prominent extension of tumor into the left cavernous sinus is present. 

Pituitary function tests

Pituitary function tests demonstrated elevation of IGH-1 with other levels been normal/low. 

  • THYROID FUNCTION
    • FT4 10.9 pmol/L (normal: 9.0-26.0) 
    • FT3 3.6 pmol/L (normal: 3.5-6.5)
    • TSH 0.52 mIU/L (normal: 0.1-4.0)
  • REPRODUCTIVE HORMONES
    • LH 4.5 IU/L (normal: 1.5-9.3)
    • FSH 8.8 IU/L (normal: 1.4-18.1)
    • Testosterone 1.5L nmol/L (normal: 7-28)
    • Prolactin 21L mIU/L (normal: 45-375)
  • GROWTH HORMONES
    • IGF-1 141.0H nmol/L (normal: 14.2-36.9)
  • CORTISOL
    • Plasma Cortisol 14L nmol/L (normal: 120-650)

Histology

The patient went onto have a transsphenoidal resection.

MICROSCOPIC DESCRIPTION: Paraffin sections show fragments of a moderately hypercellular adenoma. Tumor cells have uniform round and oval nuclei and a variable amount of pale granular eosinophilic cytoplasm. These are arranged in diffuse sheets within a vascular stroma. No mitotic figures are identified. Areas of hemorrhagic infarction are present. No normal anterior pituitary tissue is included.

Immunohistochemistry shows patchy weak staining in viable cells for growth hormone. There is also strong perinuclear staining for CAM5.2 in the majority of tumor cells. A moderate number of cells show staining for prolactin.

No staining for ACTH, LH, FSH, TSH, GFAP or S-100 protein is seen. The topoisomerase labeling index is approximately 1%.

FINAL DIAGNOSIS:

Pituitary adenoma with partial hemorrhagic infarction and immunohistochemical profile suggestive of sparsely granulated growth hormone secreting adenoma.

Case Discussion

Although typically acromegaly is encountered with patients with GH secreting microadenomas, in this case, the low-level secretions resulted in a macroadenoma being present by the time the diagnosis was made. 

The presentation was acute due to infarction and hemorrhage, consistent with pituitary apoplexy

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