Symptomatic Rathke's Cleft Cysts Located Entirely in the Suprasellar Region: Review of Diagnosis, Management, and Pathogenesis

Barrow, Daniel L.; Spector, Robert H.; Takei, Yoshio; Tindall, George T.

doi:10.1227/00006123-198506000-00005

Cited by 87 publications

(32 citation statements)

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“…The vast majority of lesions are intrasellar or intra-and suprasellar, with reports of purely suprasellar lesions occurring in a minority of patients. 6,110 The normal pituitary gland may be displaced in any direction by an RCC, including circumferentially if the cyst arises in and remains encased within the gland. 10,86 In the majority of cases, administration of Gd contrast material demonstrates little or no enhancement of the cyst wall or contents on MR imaging.…”

Section: Typical Imaging Features Of Rccsmentioning

confidence: 99%

Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships

Zada¹,

Lin²,

Ojerholm³

et al. 2010

FOC

232

182

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Object Cystic epithelial masses of the sellar and parasellar region may be difficult to differentiate on a clinical, imaging, or even histopathological basis. The authors review the developmental relationships and differentiating features of various epithelial lesions of the sellar region. Methods The authors performed a review of the literature to identify previous studies describing the etiological relationships and differentiating features of various cystic sellar lesions, including craniopharyngioma (CP), Rathke cleft cyst, xanthogranuloma, and dermoid and epidermoid cysts. Results There is significant evidence in the literature to support a common ectodermal origin of selected sellar and suprasellar cystic lesions, which may account for the overlap of features and transitional states observed in some cases. Research obtained from animal studies and reports of transitional cystic epithelial masses or lesions crossing over from typical to more aggressive pathological subtypes have collectively provided a solid foundation for this theory. Histological features that signify transitional entities beyond simple benign Rathke cleft cysts include squamous metaplasia, stratified squamous epithelium, and ciliated or mucinous goblet cells in squamous-papillary CPs. Several studies have identified key clinical, imaging, and histopathological features that can be used in the differentiation of these lesions. Conclusions The pattern of embryological formation of the hypothalamic-pituitary axis plays a major role in its propensity for developing cystic epithelial lesions. Subsequent inflammatory, metaplastic, and neoplastic processes may promote further progression along the pathological continuum, ranging from benign epithelial cysts to aggressive neoplastic cystic CPs. Selected clinical, imaging, and histopathological features can be used collectively to help differentiate these lesions and assign a formal diagnosis, thus accurately guiding further treatment.

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Section: Typical Imaging Features Of Rccsmentioning

confidence: 99%

Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships

Zada¹,

Lin²,

Ojerholm³

et al. 2010

FOC

232

182

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“…44,51 The pars tuberalis lies above the diaphragm, and the Rathke's pouch remnants in this location can give rise to an entirely suprasellar RCC. 4 Entirely suprasellar RCCs with a normal sella turcica are relatively rare (incidence 0%-20%). 8,12,13,15,20,24,25,31,36,39 Suprasellar, intrasuprasellar, and intrasellar RCCs are distinct entities with respect to their clinical presentation, resectability, postoperative recurrence, and outcomes.…”

mentioning

confidence: 99%

Characteristics of Rathke's cleft cyst based on cyst location with a primary focus on recurrence after resection

Chotai¹,

Liu²,

Qi³

2015

JNS

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Rathke's cleft cysts (RCCs) are nonneoplastic epithelial lesions of the sellar and suprasellar region. They are typically asymptomatic with a slow growth rate. An asymptomatic RCC is most often diagnosed at autopsy with a reported incidence of 5%-33%. 5,[47][48][49] Quite often some RCCs grow over time and can become sufficiently large to cause a compressive effect on surrounding structures, resulting in neurological and endocrine abnormalities. 7,28,37,42 Sixteen patients (18%) had an entirely intrasellar RCC, 21 (24%) had a purely suprasellar cyst, and 50 (58%) had an intrasuprasellar RCC. The mean cyst volume was 2.4 ± 0.9 cm 3 (range 0.36-4.9 cm 3 ). Headache was the most frequent initial symptom (76%) followed by visual disturbance (45%). The transsphenoidal approach was performed for all intrasellar RCCs (16 cysts) and 33 of 50 intrasuprasellar RCCs. The transcranial route was used for all suprasellar cysts (21 cysts) and 17 of 50 intrasuprasellar RCCs. Squamous metaplasia was present in 27 (31%) of 87 RCCs. The occurrence of squamous metaplasia was associated with cyst location (p = 0.027), T1 signal intensity (p = 0.004) and ring enhancement on Gd-enhanced MRI (p = 0.017), and cyst volume (p = 0.045). A suprasellar location (p = 0.048, OR 3.89, 95% CI 1.010-15.020), ring enhancement on Gd-enhanced MRI (p = 0.028, OR 3.922, 95% CI 1.158-13.288), hypointensity on T1-weighted MRI (p = 0.002, OR 6.86, 95% CI 1.972-23.909), and cyst volume (p = 0.01, OR 0.367, 95% CI 0.170-0.789) were independent predictors of squamous metaplasia. The mean time to reaccumulation (11 [12.6%] of 87 cases) and recurrence (7 [8%] of 87 cases) was 14 ± 6 months. Recurrence-free survival was 84.5% at a mean of 98.2 ± 4.6 months after treatment. A suprasellar cyst location (p = 0.007, OR 7.7, 95% CI 1.75-34.54), the occurrence of squamous metaplasia (p = 0.007, OR 19.3,, and isointensity on T2-weighted MRI (p = 0.041, OR 10.29, 95% CI 1.094-96.872) were the independent predictors of RCC recurrence. coNclusioNs A suprasellar cyst location, the occurrence of squamous metaplasia, and isointensity on T2-weighted MRI were independent predictors of RCC recurrence. The extent of resection and type of surgical approach used were not associated with recurrence. A tailored extent of resection based on cyst location and predictive factors is recommended.

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“…However, a few cells may migrate outside the sellar region. 2,10 Under such circumstances, the failure of these ectopic cells to regress during development may lead to the formation of an ectopic RCC. Our pathological examination showed that the cyst was lined partially by columnar epithelium and partially by stratified squamous epithelium, which overlapped histological features with the craniopharyngioma.…”

Section: Discussionmentioning

confidence: 99%

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

Fan¹,

Qi²,

Peng³

et al. 2014

JNS

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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.

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Symptomatic Rathke's Cleft Cysts Located Entirely in the Suprasellar Region: Review of Diagnosis, Management, and Pathogenesis

Cited by 87 publications

References 0 publications

Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships

Craniopharyngioma and other cystic epithelial lesions of the sellar region: a review of clinical, imaging, and histopathological relationships

Characteristics of Rathke's cleft cyst based on cyst location with a primary focus on recurrence after resection

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

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