Steroid Responsive Xanthomatous Hypophysitis Associated with Autoimmune Thyroiditis: A Case Report

Abstract

We report the case of a 36-year-old woman who presented with headache, fever, and amenorrhea. Laboratory analysis revealed hypopituitarism and autoimmune thyroiditis, while a cerebrospinal fluid study suggested concurrent aseptic meningitis. A magnetic resonance image (MRI) scan revealed a 1.0×0.9 cm cystic mass enlarging the sella turcica. Surgical resection via an endoscopic transsphenoidal route was performed. The histological finding of the excised tissue revealed foamy histiocytes with vacuolated cytoplasm, supporting the diagnosis of xanthomatous hypophysitis. Although a residual soft lesion was observed on the MRI image postoperatively, the patient's headache and fever improved. Ten months after surgery, the patient complained of visual impairment and headache, and the residual mass had enlarged into the suprasellar area. High dose (500 mg intravenous) methylprednisolone was administered for 3 days. During the methylprednisolone pulse therapy, the patient's visual acuity and headache improved. A follow-up MRI taken after methylprednisolone therapy showed a marked mass reduction. Our case supports an autoimmune pathophysiology for xanthomatous hypophysitis and suggests that high dose glucocorticoid therapy as a treatment option.

Xanthomatous hypophysitis

“…In addition to our patient, a review of the literature revealed a total of 17 previously reported patients with XH (Table 1) [1][2][3][4][5][6][7][8][9]. The review revealed a predominance of female patients with XH with a female to male ratio of approximately 3:1, similar to that reported by Gutenberg et al [5].…”

“…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 anterior pituitary disease, however, the presence of DI in some of these patients suggests involvement of the posterior lobe.…”

“…Other reports have also revealed an insidious onset of symptoms, with the exception of two patients who presented with acute headache and vomiting [4,6]. The duration of symptoms varied considerably from a few days to over 14 years but overall it was longest in XH compared to other forms of primary hypophysitis [5].…”

“…However, to our knowledge, no infectious agent has been identified in any reported patient. An autoimmune etiology has also been proposed for XH, with a recent report describing autoimmune thyroiditis noted in one patient with XH [6]. Only Gutenburg and Burt screened their patients for the presence of other autoimmune diseases, which were not present [4,5].…”

“…Glucocorticoid therapy usually provides no benefit in XH compared to lymphocytic hypophysitis [17][18][19]. However, some relief and symptom stabilization was reported in one study [6].…”