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

Abstract

BACKGROUND Xanthomatous lesions of the pituitary have been linked to ruptured or hemorrhagic Rathke’s cleft cysts. Most cases are reported to resolve following radical resection. When recurrence does occur, there is no established treatment regimen. High-dose glucocorticoids have been reported to be beneficial in several published cases; however, their effects are often not sustained once therapy is discontinued. OBSERVATIONS The authors report the case of an adolescent male who developed recurrent xanthogranulomatous hypophysitis associated with a Rathke’s cleft cyst despite two surgical interventions. He was treated with a short course of dexamethasone followed by a maintenance course of celecoxib and mycophenolate mofetil. This regimen proved to be safe and well-tolerated, and it successfully prevented another recurrence of his xanthogranulomatous hypophysitis. LESSONS This case demonstrates a novel nonsurgical approach to the management of recurrent xanthogranulomatous hypophysitis. It suggests a potential application of a combined corticosteroid-sparing immunosuppressive and anti-inflammatory regimen in other cases of refractory xanthogranulomatous hypophysitis.

Hypophysitis: Defining Histopathologic Variants and a Review of Emerging Clinical Causative Entities

“…The pathogenesis is commonly due to an inflammatory response to components of Rathke cleft cyst that are exposed after rupture, hemorrhage, or leakage [ 4 , 25 , 26 ]. The initial precipitating event causes a secondary response to the mucoid fluid content released from the cyst, and the resulting inflammation becomes a chronic process, recruits macrophages, and accumulates lipids [ 24 , 25 ].…”

“…Xanthogranulomatous lesions contain cholesterol clefts, fibrosis, giant cells, eosinophilic necrotic debris, macrophage accumulation, and hemosiderin deposits, whereas there is no hemosiderin pigment in xanthomatous lesions. This suggests the xanthomatous form can transition to xanthogranulomatous possibly as a chronic process through repeated Rathke cleft cyst hemorrhages [ 26 ].…”

“…On imaging, the lesions display more symmetric enlargement of the pituitary gland than adenomas [ 24 ]. Radical resection is required to remove the cyst, but recurrence is not uncommon [ 26 ].…”

“…Pure steroid therapy may be less effective in granulomatous than lymphocytic hypophysitis [ 7 ]. In xanthomatous hypophysitis, the benefit of high-dose glucocorticoids is controversial [ 26 ]. In a case report by DeCou et al [ 26 ], xanthogranulomatous hypophysitis secondary to a ruptured Rathke cleft cyst was initially treated with dexamethasone, then administered a maintenance course of celecoxib and mycophenolate mofetil.…”

“…In xanthomatous hypophysitis, the benefit of high-dose glucocorticoids is controversial [ 26 ]. In a case report by DeCou et al [ 26 ], xanthogranulomatous hypophysitis secondary to a ruptured Rathke cleft cyst was initially treated with dexamethasone, then administered a maintenance course of celecoxib and mycophenolate mofetil. Steroid-sparing immunosuppressive therapy in combination with an anti-inflammatory can be a safe and effective treatment for recurrent hypophysitis.…”