Recurring Primary Xanthomatous Hypophysitis Behaving Like Pituitary Adenoma: Additional Case and Literature Review

World Neurosurg. 2020 Jun;138:27-34. doi: 10.1016/j.wneu.2020.02.055. Epub 2020 Feb 17.

Abstract

Background: Xanthomatous hypophysitis (XH) is the rarest histologic type of primary hypophysitis. It is nonlymphocytic and characterized by an infiltration of the pituitary gland by lipid-laden histiocytes and macrophages. The clinical and radiologic features overlap heavily with pituitary adenomas and are prone to misdiagnosis. We describe a rare case of XH recurrence at 1 year, treated surgically. Moreover, we provide an updated review of the literature to further elucidate useful management and detection strategies.

Case description: A 45-year-old woman presented with a history of menstrual irregularity for 9 months, amenorrhea, galactorrhea, and headache for 2 months duration. Preoperative endocrinologic studies showed increased prolactin levels. Magnetic resonance imaging of the sella showed a cystic lesion with suprasellar extension suggestive of a pituitary adenoma. The patient underwent transsphenoidal resection, showing a thick yellowish colloidal material. Histopathology showed necrotic tissue with no definitive diagnosis and no identified microorganisms. At 2 months after surgery, prolactin levels and menstrual cycle normalized. At 1 year after surgery, her menstrual cycle again became irregular. Repeat magnetic resonance imaging showed a recurrent mass and a second transsphenoidal resection was undertaken. Repeat histopathology was consistent with XH. The patient is 9 years postoperative and is doing well without evidence of recurrence.

Conclusions: XH presents similarly to nonfunctional adenomas and diagnosis remains difficult without surgical pathology, requiring meticulous immunohistochemistry to prevent misdiagnosis. Thus, XH should be considered as a rare cause in the differential of disease of the sellar region. Management can parallel that of pituitary neoplasm, with a focus on radiosurgery, re-resection, and high-dose steroids.

Keywords: Pituitary gland; Primary inflammation; Recurrence; Surgical resection; Xanthomatous hypophysitis.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adenoma / diagnosis*
  • Diagnosis, Differential
  • Female
  • Humans
  • Hypophysitis / diagnosis*
  • Hypophysitis / pathology*
  • Hypophysitis / surgery
  • Middle Aged
  • Pituitary Neoplasms / diagnosis*
  • Recurrence
  • Xanthomatosis / diagnosis*
  • Xanthomatosis / pathology
  • Xanthomatosis / surgery