Article
Granulomatous hypophysitis caused by intrasellar rupture of Rathke's cleft cyst: Case report
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Published: | May 30, 2008 |
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Objective: Rathke's cleft cysts are rare, benign, intra- or juxtasellar tumors derived from remnants of Rathke's pouch. Rathke's cleft cysts is usually asymptomatic even if large cysts could became symptomatic due to a compression of pituitary gland, optic chiasm, or hypothalamus. Hypophysitis associated with Rathke's cleft cysts is rarely described and seems to be due to a rupture of the cyst and leakage of the contents in the area
Methods: We report the case of a 53-year-old woman who first presented a diabetes insipidus and visual impairment. Radiologically, the lesion appeared as an intense contrast-enhanced intra-suprasellar mass with a large cystic component compressing the optic chiasma and the pituitary stalk. The results of laboratory findings were normal.
Results: The patient underwent a trans-sphenoidal approach with total removal of the lesion and intraoperative detection of a firm tumoral capsula, with difficulty in resection, surrounding a cyst containing thick and mucinous fluid. Tumoral capsula was removed and histology confirmed the presence of a Rathke's cleft cyst.
Conclusions: Granulomatous hypophysitis accounts for less than 1% of all pituitary disorders. Generally they are idiopathic forms or in association with other autoimmune disorders or systemic granulomatous disorders such as syphilis, tuberculosis, sarcoidosis or histiocytosis. Only few cases related to rupture of Rathke's cleft cyst are reported. Since usually clinical and radiological features are not specifics, preoperative diagnosis of granulomatous hypophysitis is rarely made and surgical excision is often contemplated. The exact histological diagnosis of granulomatous hypophysitis related to rupture of Rathke's cleft cyst is important in order to avoid inappropriate laboratory and instrumental exams.