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Silky Chotai, Yi Liu, Jun Pan and Songtao Qi

OBJECT

Rathke's cleft cysts (RCCs) are benign lesions with a location that is entirely intrasellar, intrasellar with suprasellar extension (intrasuprasellar), or purely suprasellar. The recurrence of RCC is relatively uncommon. The present study was conducted to report clinical characteristics, histological features, and outcomes based on location of the cyst with a primary focus on analyzing the predictors of squamous metaplasia and recurrence in these 3 types of RCCs.

METHODS

A retrospective review of the medical records of patients with symptomatic RCCs who had undergone resection at the authors' institution was conducted. Data points, including clinical presentation, preoperative endocrine status, operative details, imaging findings, pathology, and clinical outcomes, were reviewed. A multivariable regression model was used to identify predictors of recurrence.

RESULTS

The mean age of the 87 eligible patients, 64 females and 23 males, was 41 ± 14 years (range 10–73 years). 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 cm3 (range 0.36–4.9 cm3). 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, 95% CI 2.25–165.18), 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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Song-tao Qi, Jun Fan, Xi-an Zhang and Jun Pan

Object

A precise understanding of the ambient cistern and its associated arachnoid membranes is helpful for accessing perimesencephalic lesions. However, few studies of the arachnoid membranes related to the ambient cistern have been published, and, additionally, some aspects of the ambient cistern also require further examination. The goal of this study was to reinvestigate and expound on the anatomical features of the cistern and membranes.

Methods

The ambient cisterns and its associated arachnoid membranes were examined in 20 adult cadaveric brains using an operative microscope.

Results

The perimesencephalic membrane is a set of inner arachnoid membranes surrounding the midbrain at the level of the tentorial incisura. It arises from the outer arachnoidal membranes covering the tentorial edge and the dorsum sellae and can be subdivided into anterior and posterior portions. The anterior membrane is actually the mesencephalic leaf of Liliequist membrane, which is divided into medial and lateral parts by the oculomotor nerve. The posterior membrane can be divided into horizontal and ascending parts. The ambient cistern is located above the perimesencephalic membrane and contains the anterior choroidal arteries, the posterior cerebral arteries, the basal vein, and sometimes the segments of the superior cerebellar arteries. It communicates with the carotid cistern, the interpeduncular cistern, the oculomotor cistern, the cerebellopontine and cerebellomesencephalic cistern, and the quadrigeminal cistern.

Conclusions

This study updates some information about the ambient cistern and its arachnoid membranes. The perimesencephalic membrane was identified and described in detail. The ambient cistern was verified to be a supratentorial cistern above the perimesencephalic membrane. The borders and contents of this cistern, as well as its relationship with adjacent cisterns, were also redefined.

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Yun Bao, Jun Pan, Song-tao Qi, Yun-tao Lu and Jun-xiang Peng

OBJECT

Craniopharyngiomas are associated with a high rate of recurrence. The surgical management of recurrent lesions has been among the most challenging neurosurgical procedures because of the craniopharyngioma's complex topographical relationship with surrounding structures. The aim of this study was to define the determinative role of the site of origin on the growth pattern and clinical features of recurrent craniopharyngiomas.

METHODS

The authors performed a retrospective analysis of 52 patients who had undergone uniform treatment by a single surgeon. For each patient, data concerning symptoms and signs, imaging features, hypothalamic-pituitary function, and recurrence-free survival rate were collected.

RESULTS

For children, delayed puberty was more frequent in the group with Type I (infradiaphragmatic) craniopharyngioma than in the group with Type TS (tuberoinfundibular and suprasellar extraventricular) lesions (p < 0.05). For adults, blindness was more frequent in the Type I group than in the Type TS group (p < 0.05). Nausea or vomiting, delayed puberty, and growth retardation were more frequent in children than in adults (p < 0.05). Overall clinical outcome was good in 48.07% of the patients and poor in 51.92%. Patients with Type TS recurrent tumors had significantly worse functional outcomes and hypothalamic function than patients with the Type I recurrent tumors but better pituitary function especially in children.

CONCLUSIONS

The origin of recurrent craniopharyngiomas significantly affected the symptoms, signs, functional outcomes, and hypothalamic-pituitary functions of patients undergoing repeated surgery. Differences in tumor growth patterns and site of origin should be considered when one is comparing outcomes and survival across treatment paradigms in patients with recurrent craniopharyngiomas.

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Da Li and Jun-Ting Zhang

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Chao-hu Wang, Song-Tao Qi, Jun Fan, Jun Pan, Jun-Xiang Peng, Jing Nie, Yun Bao, Ya-Wei Liu, Xi’an Zhang and Yi Liu

OBJECTIVE

Nuclear β-catenin, a hallmark of active canonical Wnt signaling, can be histologically detected in a subset of cells and cell clusters in up to 94% of adamantinomatous craniopharyngioma (ACP) samples. However, it is unclear whether nuclear β-catenin–containing cells within human ACPs possess the characteristics of tumor stem cells, and it is unknown what role these cells have in ACP.

METHODS

Primary ACP cells were cultured from 12 human ACP samples. Adamantinomatous CP stem cell–like cells (CSLCs) showing CD44 positivity were isolated from the cultured primary ACP cells by performing magnetic-activated cell sorting. The tumor sphere formation, cell cycle distribution, stemness marker expression, and multidifferentiation potential of the CD44− cells and the CSLCs were analyzed.

RESULTS

Compared with the CD44− cells, the cultured human CSLCs formed tumor spheres and expressed CD44 and CD133; moreover, these cells demonstrated nuclear translocation of β-catenin. In addition, the CSLCs demonstrated osteogenic and adipogenic differentiation capacities compared with the CD44− cells. The CSLCs also displayed the capacity for tumor initiation in human–mouse xenografts.

CONCLUSIONS

These results indicate that CSLCs play an important role in ACP development, calcification, and cystic degeneration.

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Yun-Tao Lu, Song-Tao Qi, Jia-Ming Xu, Jun Pan and Jin Shi

OBJECT

This study aimed to identify the membranous septation between the adeno- and neurohypophysis. The clinical impact of this septation in the surgical removal of infradiaphragmatic craniopharyngioma (Id-CP) is also clarified.

METHODS

The sellar regions from 8 fetal and 6 adult cadavers were dissected. After staining first with H & E and then with picro-Sirius red, the membranous structures were observed and measured under normal light and polarization microscopy. The pre- and postsurgical images and intraoperative procedures in 28 cases of childhood Id-CP were reviewed and analyzed.

RESULTS

There is a significant membranous septation (termed the adenoneurohypophysis septation [ANHS]) lying behind the intermediate lobe to separate the adeno- and neurohypophysis. The average thicknesses are 21.9 ± 16.9 μm and 79.1 ± 43.2 μm in fetal and adult heads, respectively. The median segment of the septation is significantly thicker than the upper and lower segments. The ANHS extends from the suprasellar pars tuberalis to the sellar floor, where it is fused with the pituitary capsule. During Id-CP surgery performed via a transcranial approach, the ANHS can be identified to reserve the neurohypophysis. Moreover, by understanding the anatomy of this membrane, the pituitary stalk was preserved in 3 patients (10.7%).

CONCLUSIONS

There is a significant membrane separating the anterior and posterior lobes of the pituitary gland, which lies behind the intermediate lobe. Understanding the anatomy of this septation is important for identifying and preserving the neurohypophysis and pituitary stalk during Id-CP surgery.

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Song-tao Qi, Yi Liu, Jun Pan, Silky Chotai and Lu-xiong Fang

Object

The completeness of meningioma resection depends on the resection of dura mater invaded by the tumor. The pathological changes of the dura around the tumor can be interpreted by evaluating the dural tail sign (DTS) on MRI studies. The goal of this study was to clarify the pathological characteristics of the DTSs, propose a classification based on the histopathological and radiological correlation, and identify the invasive range of tumor cells in different types of DTS.

Methods

The authors retrospectively reviewed 179 patients with convexity meningiomas who underwent Simpson Grade I resection. All patients underwent an enhanced MRI examination preoperatively. The convexity meningiomas were dichotomized into various subtypes in accordance with the 2007 WHO classification of tumors of the CNS, and the DTS was identified based on the Goldsher criteria. The range of resection of the involved dura was 3 cm from the base of the tumor, which corresponded with the length of DTS on MRI studies. Histopathological examination of dura at 0.5, 1.0, 1.5, 2.0, 2.5, and 3.0 cm from the base of the tumor was conducted, and the findings were correlated with the preoperative MRI appearance of the DTS.

Results

A total of 154 (86%) of 179 convexity meningiomas were classified into WHO Grade I subtype, including transitional (44 [28.6%] of 154), meningothelial (36 [23.4%] of 154), fibrous (23 [14.9%] of 154), psammomatous (22 [14.3%] of 154), secretory (10 [6.5%] of 154), and angiomatous (19 [12.3%] of 154). The other 25 (14%) were non–Grade I (WHO) tumors, including atypical (12 [48%] of 25), anaplastic (5 [20%] of 25), and papillary (8 [32%] of 25). The DTS was classified into 5 types: smooth (16 [8.9%] of 179), nodular (36 [20.1%] of 179), mixed (57 [31.8%] of 179), symmetrical multipolar (15 [8.4%] of 179), and asymmetrical multipolar (55 [30.7%] of 179). There was a significant difference in distribution of DTS type between Grade I and non–Grade I tumors (p = 0.004), whereas the difference was not significant among Grade I tumors (0.841) or among non–Grade I tumors (p = 0.818). All smooth-type DTSs were encountered in Grade I tumors, and the mixed DTS (52 [33.8%] of 154) was the most common type in these tumors. Nodular-type DTS was more commonly seen in non–Grade I tumors (12 [48%] of 25). Tumor invasion was found in 88.3% (158 of 179) of convexity meningiomas, of which the range of invasion in 82.3% (130 of 158) was within 2 cm and that in 94.9% (150 of 158) was within 2.5 cm. The incidence of invasion and the range invaded by tumor cells varied in different types of DTS, and differences were statistically significant (p < 0.001).

Conclusions

Nodular-type DTS on MRI studies might be associated with non–Grade I tumors. The range of dural resection for convexity meningiomas should be 2.5 cm from the tumor base, and if this extent of resection is not feasible, the type of DTS should be considered. However, for skull base meningiomas, in which mostly Simpson Grade II resection is achieved, the use of this classification should be further validated. The classification of DTS enables the surgeon to predict preoperatively and then to achieve the optimal range of dural resection that might significantly reduce the recurrence rate of meningiomas.

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Jun Pan, Songtao Qi, Yi Liu, Yuntao Lu, Junxiang Peng, XiAn Zhang, YiKai Xu, Guang-long Huang and Jun Fan

OBJECT

Craniopharyngiomas (CPs) are rare epithelial tumors that are often associated with an enigmatic and unpredictable growth pattern. Understanding the growth patterns of these tumors has a direct impact on surgical planning and may enhance the safety of radical tumor removal. The aim of this study was to analyze the growth patterns and surgical treatment of CPs with a focus on the involvement of the hypothalamopituitary axis and the relationship of the tumor to the arachnoid membrane and surrounding structures.

METHODS

Clinical data from 226 consecutive patients with primary CP were retrospectively reviewed. Tumor location and the relationship of the tumor to the third ventricle floor and the pituitary stalk were evaluated using preoperative MRI and intraoperative findings. A topographic classification scheme was proposed based on the site of tumor origin and tumor development. The clinical relevance of this classification on patient presentation and outcomes was also analyzed.

RESULTS

The growth of CPs can be broadly divided into 3 groups based on the site of tumor origin and on tumor-meningeal relationships: Group I, infrasellar/infradiaphragmatic CPs (Id-CPs), which mainly occurred in children; Group II, suprasellar subarachnoid extraventricular CPs (Sa-CPs), which were mainly observed in adults and rarely occurred in children; and Group III, suprasellar subpial ventricular CPs (Sp-CPs), which commonly occurred in both adults and children. Tumors in each group may develop complex growth patterns during vertical expansion along the pituitary stalk. Tumor growth patterns were closely related to both clinical presentation and outcomes. Patients with Sp-CPs had more prevalent weight gain than patients with Id-CPs or Sa-CPs; the rates of significant weight gain were 41.7% for children and 16.7% for adults with Sp-CPs, 2.2% and 7.1% for those with Id-CPs, and 12.5% and 2.6% for those with Sa-CPs (p < 0.001). Moreover, patients with Sp-CPs had increased hypothalamic dysfunction after radical removal; 39% of patients with Sp-CPs, 14.5% with Id-CPs, and 17.4% with Sa-CPs had high-grade hypothalamic dysfunction in the first 2 postoperative years (p < 0.001).

CONCLUSIONS

The classification of CPs based on growth pattern may elucidate the best course of treatment for this formidable tumor. More tailored, individualized surgical strategies based on tumor growth patterns are mandatory to provide long-term tumor control and to minimize damage to hypothalamic structures. Differences in the distribution of growth patterns between children and adults imply that hierarchical comparison is necessary when investigating outcomes and survival across treatment paradigms in patients with CP.

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Jun Fan, Songtao Qi, Yuping Peng, Xi-an Zhang, Binghui Qiu and Jun Pan

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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Jun Fan, Yuping Peng, Songtao Qi, Xi-an Zhang, Binghui Qiu and Jun Pan

Object

An assessment regarding both surgical approaches and the extent of resection for Rathke cleft cysts (RCCs) based on their locations has not been reported. The aim of this study was to report the results of a large series of surgically treated patients with RCCs and to evaluate the feasibility of individualized surgical strategies for different RCCs.

Methods

We retrospectively reviewed 87 cases involving patients with RCCs (16 intrasellar, 50 intra- and suprasellar, and 21 purely suprasellar lesions). Forty-nine patients were treated via a transsphenoidal (TS) approach, and 38 were treated via a transcranial (TC) approach (traditional craniotomy in 21 cases and supraorbital keyhole craniotomy in 17). The extent of resection was classified as gross-total resection (GTR) or subtotal resection (STR) of the cyst wall. Patients were thus divided into 3 groups according to the approach selected and the extent of resection: TS/STR (n = 49), TC/STR (n = 23), and TC/GTR (n = 15).

Results

Preoperative headaches, visual dysfunction, hypopituitarism, and diabetes insipidus (DI) resolved in 85%, 95%, 55%, and 65% of patients, respectively. These rates did not differ significantly among the 3 groups. Overall, complications occurred in 8% of patients in TS/STR group, 9% in TC/STR group, and 47% in TC/GTR group, respectively (p = 0.002). Cerebrospinal fluid (CSF) leakage (3%), new hypopituitarism (9%), and DI (6%) were observed after surgery. All CSF leaks occurred in the endonasal group, while the TC/GTR group showed a higher rate of postoperative hypopituitarism (p = 0.7 and p < 0.001, respectively). It should be particularly noted that preoperative hypopituitarism and DI returned to normal, respectively, in 100% and 83% of patients who underwent supraorbital surgery, and with the exception of 1 patient who had transient postoperative DI, there were no complications in patients treated with supraorbital surgery. Kaplan-Meier 3-year recurrence-free rates were 84%, 87%, and 86% in the TS/STR, TC/STR, and TC/GTR groups, respectively (p = 0.9).

Conclusions

It is reasonable to adopt individualized surgical strategies for RCCs based on cyst location. Gross-total resection does not appear to reduce the recurrence rate but increase the risk of postoperative complications. The endonasal approach seems more appropriate for primarily intrasellar RCCs, while the craniotomy is recommended for purely or mainly suprasellar cysts. The supraorbital route appears to be preferred over traditional craniotomy for its minimal invasiveness and favorable outcomes. The endoscopic technique is helpful for either endonasal or supraorbital surgery.