Brain Tumor Res Treat.  2019 Apr;7(1):57-61. 10.14791/btrt.2019.7.e22.

Idiopathic Granulomatous Hypophysitis with Rapid Onset: A Case Report

• Park HJ
• Park SH
• Kim JH
• Kim YH

• KMID: 2444798
• DOI: http://doi.org/10.14791/btrt.2019.7.e22

Abstract

Idiopathic granulomatous hypophysitis (IGH), a rare disease, requires differentiation from more common mass lesions of the sella such as pituitary adenoma, craniopharyngioma, Rathke's cleft cyst, or pituitary tuberculoma. IGH usually presents with an insidious onset of visual defects and headaches. On the other hand, rapid onset of neurologic and visual symptoms in an IGH patient is exceptionally rare. Here, we present a biopsy-proven case of IGH with rapid onset and satisfactory outcome after high dose steroid treatment.

Keyword

Pituitary gland; Hypophysitis; Glucocorticoids; Sella turcica; Endoscopy; Adult

MeSH Terms

Adult
Autoimmune Hypophysitis*
Craniopharyngioma
Endoscopy
Glucocorticoids
Hand
Headache
Humans
Pituitary Gland
Pituitary Neoplasms
Rare Diseases
Sella Turcica
Tuberculoma
Glucocorticoids

Figure

• 

  Fig. 1 MR at presentation (A) and post-operative 3 months (B). A: The lesion in the pituitary gland without the invasion of the bilateral cavernous sinus is moderately enhanced with gadolinium. The pituitary gland is not demarcated definitely, in contrast to the features of pituitary adenoma. The lesion is symmetric, encases both the left and the right internal carotid artery and compresses the optic chiasm. B: The thick pituitary stalk returned to the normal size and the pituitary gland has shrunken 3 months after steroid treatment. Note that the involvement of bilateral cavernous sinuses and mass effect on the optic chiasm has resolved.

• 

  Fig. 2 Intraoperative (A) and pathologic (B) photographs. A: Gray lesion is exposed after dural incision. The pituitary gland surrounding the lesion lost its original yellow color and its consistency was mushy. B: Pituitary biopsy showing chronic granulomatous inflammation with central necrosis and giant cells (red arrows). There are atrophic pituitary lobules seen mainly in the right upper quadrant of this photo (hematoxylin-eosin staining, ×70).

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