Brain Tumor Res Treat.  2022 Jan;10(1):48-54. 10.14791/btrt.2022.10.e24.

Xanthomatous Hypophysitis Secondary to a Ruptured Rathke’s Cleft Cyst: A Case Report

• Gezer E
• Çabuk B
• Yaprak Bayrak B
• Cantürk Z
• Çetinarslan B
• Selek A
• Sözen M
• Köksalan D
• Ceylan S

• KMID: 2525303
• DOI: http://doi.org/10.14791/btrt.2022.10.e24

Abstract

Hypophysitis (HP) is a rare disease which develops secondary to chronic or acute inflammation of the pituitary gland and may cause symptoms related to pituitary dysfunction and mass compression. Lymphocytic HP is the most common subtype of primary HP, while xanthomatous HP (XHP) is considered the rarest form, with 35 reported cases, to date. A 35-year-old woman was initially admitted to a Gynecology clinic with a 2-year history of amenorrhea and headache. She was started on cabergoline 0.5 mg twice a week for macroprolactinoma. Due to persistent amenorrhea with low gonadotropins, she was referred to our Endocrinology clinic. Her pituitary function profile revealed panhypopituitarism and a 13×11×12 mm sized sellar mass with diffuse enhancement which sustained toward the infundibulum and dura was observed on the gadolinium-enhanced pituitary MRI. The patient underwent an endoscopic endonasal transsphenoidal approach for tumor resection and thick yellowish fluid draining from the lesion was observed. The histopathological diagnosis was reported as a rupture of an Rathke’s cleft cyst and an XHP. The surgery did not improve the symptoms/pituitary functions, however, headache recovered immediately after the first dose of high dose methylprednisolone treatment. The inflammatory process in a xanthomatous lesion may actually be a secondary response to mucous fluid content release from a ruptured cyst, thus recommended to classify XHP as secondary hypophysitis. Since the differentiation of XHP from other pituitary tumors may be challenging preoperatively, surgery is the major diagnostic tool and also, the most recommended therapeutic option.

Keyword

Xanthomatous; Hypophysitis; Rathke’s cleft cyst; Panhypopituitarism

Figure

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  Fig. 1 Preoperative gadolinium-enhanced MRI scan of xanthomatous hypophysitis secondary to Rathke’s cleft cyst. A: Pre-contrast coronal T1-weighted MR image. B: Coronal T2-weighted MR image. C: Post-contrast coronal. D: Sagittal T1-weighted MR images of the pituitary lesion.

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  Fig. 2 Intraoperative image demonstrating thick yellowish fluid draining (arrow) from the pituitary lesion.

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  Fig. 3 Histological images of the resected pituitary lesion. A: Numerous cholesterol clefts (arrows) in sections obtained from the pituitary mass (H&E, ×200). B: An area of lymphoplasmacytic cells with multinucleate giant cells and microcalcification (H&E, ×100). C: Numerous foamy and hemosiderin-containing macrophages with admixed lymphocytes and histiocytes (asterisk) and higher magnification of the B (H&E, ×200). D: Dense fibrous tissues containing chronic inflammatory cells (arrow) (H&E, ×100). E: Histiocytes stained positively with CD68 (CD68, ×100).

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  Fig. 4 Postoperative gadolinium-enhanced MRI scan of the sellar area. Post-contrast coronal (A) and sagittal (B) T1-weighted MR images of the pituitary region.

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