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Xanthomatous Hypophysitis Is Associated with Ruptured Rathke’s Cleft Cyst

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Abstract

Xanthomatous hypophysitis is a rare inflammatory disease of the pituitary gland that can mimic a neoplastic lesion clinically and radiologically. Its pathogenesis remains largely unknown, although recent evidence suggests that pituitary inflammation may occur as a secondary reaction to mucous content released from a ruptured cyst. In a series of 1221 pituitary specimens, we identified seven cases of xanthomatous hypophysitis. Six patients had complete radiological and biochemical workup preoperatively: a cystic-appearing pituitary mass was identified in all six patients (100%) with a mean size of 2.0 cm (range 1.4–2.5 cm) on imaging, and pituitary endocrine dysfunction was noted in five patients (83.3%). In all cases, the pituitary mass was resected through an endoscopic transsphenoidal approach. Pathological examination revealed the presence of foamy macrophages admixed with variable amounts of giant cells and chronic inflammatory cells, confirming the diagnosis of xanthomatous hypophysitis. Additionally, all cases presented with concurrent findings of ruptured Rathke’s cleft cyst, with the exception of one patient who had previous surgery for a Rathke’s cleft cyst, followed by recurrence and diagnosis of xanthomatous hypophysitis. While accurate distinction of hypophysitis from a pituitary neoplasm can be problematic in the preoperative setting, the identification of a cystic lesion in the sella turcica should raise the possibility of such an entity in the clinical and radiological differential diagnosis. The current series provides further evidence that xanthomatous hypophysitis predominantly occurs as a secondary reaction to a ruptured Rathke’s cleft cyst; thus, it is best classified as a secondary (reactive) hypophysitis.

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Authors and Affiliations

  1. Department of Pathology, University Health Network, 200 Elizabeth Street, 11th floor, Toronto, ON, M5G 2C4, Canada

    Kai Duan, Sylvia L. Asa, Daniel Winer & Ozgur Mete

  2. Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada

    Kai Duan, Sylvia L. Asa, Daniel Winer & Ozgur Mete

  3. Endocrine Oncology Site Group, Princess Margaret Cancer Centre, Toronto, ON, Canada

    Sylvia L. Asa, Zadeh Gelareh, Fred Gentili & Ozgur Mete

  4. Department of Neurosurgery, University Health Network, Toronto, ON, Canada

    Zadeh Gelareh & Fred Gentili

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  1. Kai Duan
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Duan, K., Asa, S.L., Winer, D. et al. Xanthomatous Hypophysitis Is Associated with Ruptured Rathke’s Cleft Cyst. Endocr Pathol 28, 83–90 (2017). https://doi.org/10.1007/s12022-017-9471-x

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