An isolated primary Rathke's cleft cyst in the cerebellopontine angle

Abstract

Rathke's cleft cysts (RCCs) are benign cysts typically located in the sellar or suprasellar region; ectopic isolated lesions are extremely rare. The authors describe the case of a 25-year-old man with a giant symptomatic RCC arising primarily at the cerebellopontine angle (CPA), only the second case reported thus far. The patient presented with a 2-year history of right hearing impairment and tinnitus accompanied by vertigo and headache and a 2-week history of right facial numbness. Subsequently, he underwent total cyst removal via retrosigmoid craniotomy with a good recovery. He experienced no recurrence during a 64-month follow-up period. The possible pathogenesis, differential diagnosis, and surgical treatment of such cysts are discussed in this article. Isolated ectopic RCCs can arise from the ectopic migration of Rathke's pouch cells during the embryonic period. It is still difficult to distinguish ectopic RCCs from other cystic lesions of the CPA given the lack of specific imaging features. Aggressive resection of the cyst wall is not recommended, except when lesions do not closely adhere to adjacent structures.

An entirely suprasellar Rathke’s cleft cyst: a rare case report with review of literature

“…Rathke's cyst at ectopic locations [11][12][13][14][15][16][17][18], other than the sellar suprasellar area, have been reported.…”

“…Follow-up perimetry after 6 months showed complete resolution of the scotomas in both the eyes (Figs. 17,18).…”

Enlarging pediatric ectopic Rathke’s cleft cyst in the prepontine cistern: case report

“…3 To date, only 3 reports appear to have described intracranial ectopic Rathke's cleft cyst. Two lesions were located at the cerebellopontine angle, 6,16 including one pediatric case, 16 and in one adult case the lesion was located in the prepontine cistern 12 (Table 1).…”

Pediatric symptomatic Rathke cleft cyst compared with cystic craniopharyngioma

“…The mean age of these patients was 12.2 years (range, [5][6][7][8][9][10][11][12][13][14][15][16][17][18]. Of the 15 patients with CP that were reviewed, 8 were male and 7 were female.…”

“…RCCs diagnosed neuroradiologically had to meet all of the following conditions to qualify for a conservative treatment course; 1) the cystic lesion is oval or dumbbell-shaped and is located in the sellar or suprasellar region, 2) the cystic lesion is circumferentially surrounded by the normal pituitary gland, and 3) the cyst wall has little or no enhancement, has no signs of invasion, and has no calcification [18]. Cystic CPs are considered those in which the cystic portion predominantly accounted for at least 60% of the tumors.…”