ReviewXanthomatous hypophysitis
Introduction
Primary inflammatory pituitary pathologies are uncommon. These pathologies include lymphocytic, granulomatous, plasmacytic (immunoglobulin G4 associated) and xanthomatous hypophysitis (XH). XH is an inflammatory disorder of the pituitary gland characterized by an infiltration of lipid-laden histiocytes, also known as xanthoma cells [1]. XH is the least common form of primary hypophysitis [2]. In 1998, Folkerth et al. reported the first three patients with XH [3].
Section snippets
Case history
A 69-year-old woman initially presented 4 years ago with chronic headaches. She had no complaints of bone or joint pain and her neurologic exam was otherwise unremarkable. At that time, she was found to have a pituitary mass on MRI (Fig. 1). Her pituitary function profile was unremarkable, however, the exact lab values are not available. Endoscopic resection of the lesion was attempted and biopsy was performed. Complete resection was not possible because of the firm nature of the tissue.
A
Discussion
XH frequently presents with symptoms similar to those seen with pituitary adenomas. Our literature review revealed presenting symptoms ranging from headache, menstrual irregularities (oligomenorrhea, amenorrhea), hyperprolactinemia, impotence, endocrine irregularities (hypothyroidism, hypocortisolism), nausea, and diabetes insipidus (DI). Visual disturbances were less common and were only reported in three patients [4], [5], [6]. It is not surprising that most of these symptoms arise from
Conclusion
XH is an inflammatory process of the pituitary gland characterized by lipid-laden histiocytes. Patients with XH may present with headaches, endocrine irregularities and DI. On MRI, XH is frequently described as a cystic lesion on T1-weighted imaging and CT scans are usually normal but may show reactive bony changes. The diagnosis is established after surgical resection. The current literature, although limited, suggests there is minimal benefit from glucocorticoid therapy in XH. Only one
Conflicts of Interest/Disclosures
The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.
Acknowledgments
The authors would like to thank Dr. Erika Doxatader for her assistance in obtaining the histopathological images to illustrate this unique pathology.
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Cited by (26)
Hypophysitis: A comprehensive overview
2021, Presse MedicaleCitation Excerpt :Histologically, xanthomatous hypophysitis is characterised by infiltration of foamy xanthoma cells and lymphocytes, which probably results from macrophage activation [59,60]. Immunostaining may show CD68 as a marker for macrophage activation [58,60]. Xanthogranulomatous hypophysitis additionally shows infiltration with multinucleated giant cells and epitheliod histiocytes [63].
Xanthomatous Hypophysitis Presenting in an Adolescent Girl: A Long-Term Follow-Up of a Rare Case and Review of the Literature
2021, AACE Clinical Case ReportsCitation Excerpt :She was started on a physiological dose of hydrocortisone after the completion of prednisolone in view of secondary adrenal suppression. Furthermore, visual symptoms are less commonly reported with XH.7–10 The patient’s vision and visual field surveillance had remained normal over the years despite the close proximity of the mass to the optic chiasm.
Recurring Primary Xanthomatous Hypophysitis Behaving Like Pituitary Adenoma: Additional Case and Literature Review
2020, World NeurosurgeryCitation Excerpt :The goal of treatment in primary hypophysitis is symptomatic relief, often using both medical and surgical options to reduce the size of the pituitary gland and extinguish the inflammatory process. Pharmacologic treatment options include the use of steroids, methotrexate, and azathioprine, whereas surgical options include radiotherapy and surgical resection.1-3,6,10-14,16-20 Surgical decompression of the gland often leads to an improvement in pituitary function.
Presence of CD3<sup>+</sup> and CD79a<sup>+</sup> Lymphocytes in the Pituitary Gland of Dogs at Post-mortem Examination
2020, Journal of Comparative PathologyHypophysitis
2018, Encyclopedia of Endocrine Diseases