Abstract
BackgroundThe differential diagnosis of IgG4-related hypophysitis and other inflammatory diseases or tumors involving sellar region is challenging even after sellar biopsy. Sellar germinoma is usually infiltrated by lymphocytes or plasma cells, and may be confused with hypophysitis.Case presentationA 36-year-old man with diabetes insipidus, elevated serum IgG4 level (336 mg/dl), and sellar mass was suspected to have IgG4-related hypophysitis, and no other lesion of IgG4-related disease was detected. After treated by prednisone and mycophenolate mofetil, the serum IgG4 decreased to 214 mg/dl. However, after withdrawal of the drugs, the IgG4 level increased to 308 mg/dl. Endocrine assessments revealed panhypopituitarism, and the sellar mass enlarged. Transsphenoidal sellar exploration and biopsy was conducted. Pathological examination showed that the lesion was germinoma with lymphocytes and plasma cells infiltration, and IgG4-staining was positive (70/HPF, IgG4/IgG ratio = 10%). The patient was then treated by cisplatin and etoposide. After four cycles of chemotherapy, the serum IgG4 was 201 mg/dl, and the sellar mass was invisible.ConclusionSellar germinoma can mimic the clinical characteristics of IgG4-related hypophysitis. Poor response to glucocorticoids can be used as an exclusion criterion in the clinical diagnosis of IgG4-related hypophysitis.
Highlights
The differential diagnosis of immunoglobulin G4 (IgG4)-related hypophysitis and other inflammatory diseases or tumors involving sellar region is challenging even after sellar biopsy
Sellar germinoma can mimic the clinical characteristics of IgG4-related hypophysitis
We report here a patient presented with diabetes insipidus and hypopituitarism who was initially misdiagnosed as IgG4-related hypophysitis
Summary
The differential diagnosis of IgG4-related hypophysitis and other inflammatory diseases or tumors involving sellar region is challenging even after sellar biopsy. Similar to other types of hypophysitis, IgG4-related hypophysitis manifests as anterior pituitary hormone deficiency and/or central diabetes insipidus, as well as symptoms caused by mass effects of the lesion [10, 11]. Pathological examination played a critical role in the current diagnostic criteria of IgG4-related hypophysitis [10, 11], but sometimes its differential diagnosis might remain
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