Xanthomatous Hypophysitis: A Case Report and Comprehensive Literature Review

Abstract

PurposeXanthomatous hypophysitis (XHP) is an extremely rare form of primary hypophysitis for which there is a lack of clinical experience. A comprehensive understanding of its clinical characteristics, diagnosis and treatment is needed.MethodsHere, we report a case study and conduct a systematic review of XHP. Thirty-six cases were included, and their clinical manifestations, endocrine assessment, imaging features, treatment and follow-up data were collected and analyzed.ResultsThe mean age at diagnosis was 39.1 years, and females were predominant (75.0%). The most common symptom was headache (68.6%), and 66.7% of female patients presented menstrual disorders. The most common pituitary dysfunction was growth hormone (GH) deficiency. More than half of patients exhibited central diabetes insipidus (CDI). The majority of patients had an imaging presentation of a cystic lesion with peripheral enhancement. Pituitary stalk thickening was observed in half of the patients. Total lesion resection was achieved in 57.1% of cases. The recurrence rate after partial resection and biopsy was significantly higher than that after total lesion resection (57.1% vs. 0.0%, P = 0.0147). The most common pituitary hormone abnormalities to resolve after surgery were hyperprolactinemia (100.0%) and GH deficiency (91.7%). The typical pathological feature was inflammatory infiltration of foamy histiocytes, which showed positivity for CD68.ConclusionDiagnosis of XHP is difficult when relying on clinical symptoms and imaging features. Therefore, surgical histopathology is necessary. Based on the available evidence, total lesion resection is recommended for treatment. However, the long-term prognosis for this rare disease remains unclear.

Successful immunomodulatory treatment for recurrent xanthogranulomatous hypophysitis in an adolescent: illustrative case

“…When recurrence of XH or XG does occur, most patients are treated with high-dose glucocorticoids, though some undergo reoperation or radiation. 12 , 14 In one case described by Joung et al, 13 a 3-day course of high-dose methylprednisolone was reported to cause mass reduction and symptom improvement in a 36-year-old female with recurrent XH 10 months after initial surgery. In another case reported by Gezer et al, 4 a 35-year-old female with persistent headaches following a successful total resection for RCC-associated XH was treated with high-dose corticosteroids.…”

“…Observations Xanthomatous lesions of the pituitary gland are a rare category of hypophysitis that have been described in several reports. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] Traditionally, these lesions have been divided into two distinct categories: XH and XG; however, there is growing evidence of clinicopathologic overlap between them. 3 XH is histologically characterized by the presence of foamy histiocytes and lymphoplasmacytic infiltrates within the pituitary gland or sellar region with little to no hemosiderin pigment.…”

“…3,6,7 When recurrence does happen, many patients either undergo repeat resection or are treated with high-dose corticosteroids with varying results. [8][9][10][11][12] We report a case of an adolescent male found to have RCC with a postoperative course complicated by recurrent XGH. He experienced complete resolution of the inflammatory reaction on a short course of dexamethasone followed by a maintenance course of celecoxib and mycophenolate mofetil (MMF).…”

Xanthogranulomatous hypophysitis: A rare presentation in a young female patient

“…Xanthomatous hypophysitis has got overall good prognosis if treated promptly through transsphenoidal resection of the mass. However, due to limited data, the real prognosis of this rare disease is still unclear 12 . The resumption of the normal endocrine function however may be delayed or permanently lost in some cases.…”

“…However, due to limited data, the real prognosis of this rare disease is still unclear. 12 The resumption of the normal endocrine function however may be delayed or permanently lost in some cases.…”