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Xanthomatous Hypophysitis: A Case Report and Comprehensive Literature Review
Jianyu Zhu1,2, Zhicheng Wang2, Wenze Wang3
1Department of Neurosurgery, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Frontiers in Endocrinology
|October 18, 2021
Summary
Xanthomatous hypophysitis (XHP) is rare, often presenting with headaches and hormonal issues like GH deficiency. Total lesion resection is recommended for treatment, though long-term outcomes require further study.
Area of Science:
- Endocrinology
- Neurosurgery
- Pathology
Background:
- Xanthomatous hypophysitis (XHP) is an extremely rare pituitary disorder with limited clinical data.
- A comprehensive understanding of XHP's clinical characteristics, diagnosis, and treatment is needed.
Observation:
- A systematic review analyzed 36 XHP cases, noting a mean age of 39.1 years, with females predominantly affected (75%).
- Common symptoms included headache (68.6%) and menstrual disorders in females (66.7%).
- Pituitary dysfunction, particularly growth hormone (GH) deficiency and central diabetes insipidus (CDI), was prevalent.
Findings:
- Imaging often revealed cystic lesions with peripheral enhancement and pituitary stalk thickening.
- Total lesion resection (57.1% of cases) showed significantly lower recurrence rates (0%) compared to partial resection (57.1%).
- Post-surgical resolution was noted for hyperprolactinemia (100%) and GH deficiency (91.7%).
Implications:
- Diagnosis of XHP is challenging, necessitating surgical histopathology.
- Total lesion resection is the recommended treatment approach for XHP.
- Long-term prognosis for XHP remains uncertain, warranting further investigation.
