Two cases of symptomatic secondary hypophysitis due to Rathke’s cleft cysts treated with glucocorticoids: long-term follow-up

Abstract

Rathke's cleft cyst (RCC) is a common incidental tumor in the hypothalamic-pituitary region. Some reports have shown that the clinical symptoms and endocrine functions of symptomatic RCCs are temporarily improved by glucocorticoid administration. However, it is still unknown whether glucocorticoid treatment is effective for symptomatic RCCs according to long-term observations. In this study, we describe the long-term clinical outcomes of two cases of glucocorticoid-treated biopsy-proven secondary hypophysitis caused by RCCs. We summarize the symptoms, imaging findings, and endocrine evaluations of two symptomatic RCC patients with concomitant hypophysitis before and after prednisolone treatment. In both evaluated cases, visual impairments and altered endocrine parameters were present due to chiasm and stalk compression; these outcomes improved after shrinkage of RCCs in response to prednisolone administration, and partial recovery of anterior pituitary hormone secretion was observed. However, in both cases, the deficits in anterior pituitary hormone secretion recurred, possibly due to persistent inflammatory infiltration in the RCCs and pituitary glands. After relapse of hypophysitis, anterior hormone secretion did not fully recover. In our cases of secondary hypophysitis caused by RCCs, prednisolone administration had an early effect of cyst shrinkage, followed by partial improvements in clinical symptoms and pituitary functions. However, long-term observation showed that prednisolone treatment did not contribute to complete improvement in anterior pituitary hormone dysfunction.

Secondary hypophysitis associated with Rathke’s cleft cyst resembling a pituitary abscess

“…[ 40 ] Ring-shaped contrast enhancement is also a possible finding of secondary hypophysitis due to RCC. [ 14 , 16 , 30 , 44 ] Visual disturbance, headache, and polyuria are typical symptoms of secondary hypophysitis due to RCC;[ 14 , 16 , 30 , 44 ] however, high fever is rare. Our patient had a severe headache, fever, and polyuria, with an MRI showing a cystic sellar mass and ring-shaped contrast enhancement, as well as an increased signal on DWI, consistent with PA.…”

“…A typical MRI result for IgG4-RH is an enlarged pituitary stalk or gland that is homogeneously enhanced,[ 1 , 7 ] whereas ring-shaped contrast enhancement is a possible finding of secondary hypophysitis due to RCC. [ 14 , 16 , 30 , 44 ] According to epidemiological data, most cases of IgG4-RH are represented by middle-aged and older males. Moreover, IgG4-RH is often associated with manifestations in other systemic organs.…”

Inflammatory and Infectious Disorders in Endocrine Pathology

“…Moreover, the stand-alone term “ lymphocytic hypophysitis ” is commonly used interchangeably with “primary lymphocytic hypophysitis” in the literature [ 109 , 111 , 131 , 145 , 146 ]. However, “ lymphocytic hypophysitis ” may also refer to lymphocytic variants of secondary hypophysitis [ 120 , 138 , 147 ]. Previous studies have recognized that these differing approaches to classification terminology may cause reader confusion [ 121 ].…”