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Daily bibliographic and video review of the Neurosurgery Department. La Fe University Hospital. Valencia, Spain

Visual Outcomes After Surgical Treatment of Adult Craniopharyngiomas

Neurosurgery 71:715–721, 2012 DOI: 10.1227/NEU.0b013e318262146b

Craniopharyngiomas (CRPs) often cause visual deterioration (VD) due to the close vicinity of the optic apparatus.

OBJECTIVE: To evaluate longitudinal visual outcomes after surgery of CRP and determine the prognostic factors thereof.

METHODS: One hundred forty-six adult patients who underwent surgery for newly diagnosed CRP were retrospectively reviewed. There were 87 male patients (60%), and the median age was 41 years (range, 18-75). The mean follow-up duration was 88.7 months (range, 24-307). A visual impairment score was used to assess the short-term (,1 month) and long-term (.2 years) visual outcomes.

RESULTS: Gross total removal was performed in 53 patients (36%), and tumor recurrence occurred in 40 patients (27%). The average preoperative, short- and longterm visual impairment scores were 44.4, 38.5, and 38.1, respectively, on a 0- to 100-point scale (with 100 indicating the worst vision). Short- and long-term VD occurred in 28 (19%) and 39 patients (27%), respectively. Subtotal removal (STR) alone (P = .010; OR = 4.8), short-termVD (P<.001; OR = 39.7), and tumor recurrence (P<.001; OR = 28.2) were significant risk factors for long-term VD in the multivariate analysis. Patients undergoing STR alone had higher tumor recurrence rates in comparison with those who underwent gross total removal or STR with adjuvant therapy (P < .001).

CONCLUSION: Short-term VD secondary to the surgical insult and the recurrence of the tumor were strong predictors of long-term visual outcomes after surgical treatment for CRP. STR alone may be an ineffective strategy for achieving tumor control and optimal visual outcomes in patients with CRP.

Results after treatment of craniopharyngiomas: further experiences with 73 patients since 1997

J Neurosurg 116:373–384, 2012. DOI: 10.3171/2011.6.JNS081451

The authors report surgical and endocrinological results of a series of 73 cases of craniopharyngioma that they treated surgically since 1997 to demonstrate their change in treatment strategy and its effect on outcome compared with a previous series and results reported in the literature.

Methods. A total of 73 patients underwent surgery for craniopharyngiomas between May 1997 and January 2005. In patients with poor clinical or neuropsychological condition, even following pretreatment, only stereotactic cyst aspiration took place (8 cases). In the remaining patients, gross-total resection (GTR) was intended and appeared to be possible. The most frequent approaches were subfrontal (27 cases) and transsphenoidal (26 cases); in some cases, a multistep approach was used. The rate of GTR, complications, and functional outcome (comparing pre- and postoperative endocrine and neuropsychological testing) were evaluated. The mean duration of follow-up was 25.2 months.

Results. Gross-total resection was achieved in 88.5% of cases in which a transsphenoidal approach was used and 79.5% of those in which a transcranial approach was used (85.2% of those in which a subfrontal approach was used and 72.7% of those in which a frontolateral approach was used). In the total series, GTR was achieved in 83.1% of cases (vs 49.3% in the authors’ former series). The complication rate was 13.8% without any mortality. New endocrine deficits were observed more frequently in patients treated with transcranial approaches over the years (16.3%– 66.7% vs 2.6%–50.0%) but were less frequent after transsphenoidal approaches (5.2%–19.2% vs 2.9%–45.7%).

Conclusions. Open surgery with intended total resection remains the treatment of choice in most patients. Initial stereotactic cyst aspiration or medical pretreatment to improve the patients’ condition and adequate choice of surgical approach(es) are essential to achieve that goal. Nevertheless, a moderate increase in endocrinological deficits has to be accepted. The authors recommend using radiotherapy only in cases in which there are tumor remnants or disease progression after surgery.

Endoscopic, Endonasal Resection of Craniopharyngiomas: Analysis of Outcome Including Extent of Resection, Cerebrospinal Fluid Leak, Return to Preoperative Productivity, and Body Mass Index

Neurosurgery 70:110–124, 2012 DOI: 10.1227/NEU.0b013e31822e8ffc

The endoscopic, endonasal, extended transsphenoidal approach is a minimal-access technique for managing craniopharyngiomas. Outcome measures such as return to employment and body mass index (BMI) have not been reported and are necessary for comparison with open transcranial approaches. Most prior reports of the endoscopic, endonasal approach have reported unacceptably high cerebrospinal fluid (CSF) leak rates.

OBJECTIVE: To assess the outcome of endoscopic, endonasal surgery in a consecutive series of craniopharyngiomas with special attention to extent of resection, CSF leak, return to employment, and BMI.

METHODS: Twenty-six surgeries were performed on 24 patients at Weill Cornell Medical College-New York Presbyterian Hospital. Five patients had recurrent lesions. Gross-total resection (GTR) was attempted in 21 surgeries. Indications for intended subtotal resection were advanced age, medical comorbidities, preservation of pituitary function, and hypothalamic invasion.

RESULTS: Mean tumor diameter was 2.9 cm. GTR (18 surgeries) or near-total (.95%) resection (2 surgeries) was achieved in 95% when GTR was the goal. Seven patients received postoperative radiation therapy. Mean follow-up was 35 months with no recurrences in GTR cases and stable disease in all patients at last follow-up. Vision improved in 77%. Diabetes insipidus and panhypopituitarism developed in 42% and 38%, respectively. A more than 9% increase in BMI occurred in 39%; 69% returned to their preoperative profession/schooling. The postoperative CSF leak rate was 3.8%.

CONCLUSION: Minimal-access, endoscopic, endonasal surgery for craniopharyngioma can achieve high rates of GTR with low rates of CSF leak. Return to employment and obesity rates are comparable to microscope-assisted transcranial and transsphenoidal reports.

Infundibulo-tuberal or not strictly intraventricular craniopharyngioma: evidence for a major topographical category

Acta Neurochir (2011) 153:2403–2426. DOI 10.1007/s00701-011-1149-4

This study investigates retrospectively the clinical, neuroradiological, pathological and surgical evidence verifying the infundibulo-tuberal topography for craniopharyngiomas (CPs). Infundibulo-tuberal CPs represent a surgical challenge due to their close anatomical relationships with the hypothalamus. An accurate definition of this topographical category is essential in order to prevent any undue injury to vital diencephalic centres.

Methods A systematic review of all scientific reports involving pathological, neuroradiological or surgical descriptions of either well-described individual cases or large series of CPs published in official journals and text books from 1892 to 2011 was carried out. A total of 1,232 documents providing pathological, surgical and/or neuroradiological evidence for the infundibulo-tuberal or hypothalamic location of CPs were finally analysed in this study.

Findings For a total of 3,571 CPs included in 67 pathological, surgical or neuroradiological series, 1,494 CPs (42%) were classified as infundibulo-tuberal lesions. This topography was proved in the autopsy of 122 nonoperated cases. The crucial morphological finding characterizing the tubero-infundibular topography was the replacement of the third ventricle floor by a lesion with a predominant intraventricular growth. This type of CP usually presents a circumferential band of tight adherence to the third ventricle floor remnants, formed by a functionless layer of rective gliosis of a variable thickness. After complete surgical removal of an infundibulo-tuberal CP, a wide defect or breach at the floor of the third ventricle is regularly observed both in the surgical field and on postoperative magnetic resonance imaging studies.

Conclusions Infundibulo-tuberal CPs represent a major topographical category of lesions with a primary subpial development at the floor of the third ventricle. These lesions expand within the hypothalamus itself and subsequently occupy the third ventricle; consequently, they can be classified as not strictly intraventricular CPs. A tight attachment to the hypothalamus and remnants of the third ventricle floor is the pathological landmark of infundibulotuberal CPs.

Surgical Management of Craniopharyngiomas in Children: Meta-analysis and Comparison of Transcranial and Transsphenoidal Approaches

Neurosurgery 69:630–643, 2011 DOI: 10.1227/NEU.0b013e31821a872d

Controversy persists regarding the optimal treatment of pediatric craniopharyngiomas.

OBJECTIVE: We performed a meta-analysis of reported series of transcranial (TC) and transsphenoidal (TS) surgery for pediatric craniopharyngiomas to determine whether comparisons between the outcomes in TS and TC approaches are valid.

METHODS: Online databases were searched for English-language articles reporting quantifiable outcome data published between 1990 and 2010 pertaining to the surgical treatment of pediatric craniopharyngiomas. Forty-eight studies describing 2955 patients having TC surgery and 13 studies describing 373 patients having TS surgery met inclusion criteria.

RESULTS: Before surgery, patients who had TC surgery had less visual loss, more frequent hydrocephalus and increased intracranial pressure, larger tumors, and more suprasellar disease. After surgery, patients in the TC group had lower rates of gross total resection (GTR), more frequent recurrence after GTR, higher neurological morbidity, more frequent diabetes insipidus, less improvement, and greater deterioration in vision. There was no difference in operative mortality, obesity/hyperphagia, or overall survival percentages.

CONCLUSION: Directly comparing outcomes after TC and TS surgery for pediatric craniopharyngiomas does not appear to be valid. Baseline differences in patients who underwent each approach create selection bias that may explain the improved rates of disease control and lower morbidity of TS resection. Although TS approaches are becoming increasingly used for smaller tumors and those primarily intrasellar, tumors more amenable to TC surgery include large tumors with significant lateral extension, those that engulf vascular structures, and those with significant peripheral calcification.

Rathke cleft cysts: a review of clinical and surgical management

Neurosurg Focus 31 (1):E1, 2011. DOI: 10.3171/2011.5.FOCUS1183

The aim of this paper is to provide a comprehensive review of clinical, imaging, and histopathological features, as well as operative and nonoperative management strategies in patients with Rathke cleft cysts (RCCs).

A literature review was performed to identify previous articles that reported surgical and nonsurgical management of RCCs.

Rathke cleft cysts are often incidental lesions found in the sellar and suprasellar regions and do not require surgical intervention in the majority of cases. In symptomatic RCCs, the typical clinical presentation includes headache, visual loss, and/or endocrine dysfunction. Visual field testing and endocrine laboratory studies may reveal more subtle deficiencies associated with RCCs. When indicated, the transsphenoidal approach typically offers the least invasive and safest method for treating these lesions.

Various surgical strategies including cyst wall resection, intralesional alcohol injection, and sellar floor reconstruction are discussed. Although headache and visual symptoms frequently improve after surgical drainage of RCCs, hypopituitarism and diabetes insipidus are less likely to do so.

A subset of more aggressive, atypical RCCs associated with pronounced clinical symptoms and higher recurrence rates is discussed, as well as the possible relationship of these lesions to craniopharyngiomas.

Rathke cleft cysts are typically benign, asymptomatic lesions that can be monitored. In selected patients, transsphenoidal surgery provides excellent rates of improvement in clinical symptoms and long-term cyst resolution. Complete cyst wall resection, intraoperative alcohol cauterization, and sellar floor reconstruction in the absence of a CSF leak are not routinely recommended.

Neurosurgical treatment of craniopharyngioma in adults and children: early and long-term results in a large case series

J Neurosurg 114:1350–1359, 2011.DOI: 10.3171/2010.11.JNS10670

Craniopharyngioma accounts for 2%–5% of all primary intracranial neoplasms. The optimal management of craniopharyngioma remains controversial. The authors evaluated the early results of surgery and the longterm risk of tumor recurrence in a large series of patients undergoing resection of craniopharyngiomas.

Methods. Between 1990 and 2008, 112 consecutive patients (57 male and 55 female patients with a mean [± SEM] age of 33.3 ± 1.8 years) underwent resection of craniopharyngiomas at the authors’ hospital. Recurrence or growth of residual tumor tissue during follow-up was assessed using MR imaging.

Results. There were 3 perioperative deaths (2.7%). Severe adverse events were more frequent in patients who underwent operations via the transcranial route (37%) than the transsphenoidal approach (5.6%; p < 0.001). Magnetic resonance imaging showed radical resection of the tumor in 78 (71.6%) of the remaining 109 patients. Previous surgery and maximum tumor diameter were associated with persistence of disease after surgery. Craniopharyngioma recurred in 26 (24.5%) of 106 patients. Presence of residual tumor on the first postoperative MR imaging, male sex, and no postoperative radiation therapy were associated with a risk of tumor recurrence. Quality-of-life data were assessed in the 91 patients who attended the authors’ institution for follow-up visits. Among them, 8.8% patients were partially or completely dependent on others for daily living activities before surgery. This percentage increased to 14.3% at the last follow-up visit. The 5- and 10-year overall survival rates were 94.4% (95% CI 90.0%–98.8%) and 90.3% (95% CI 83.4%–97.3%), respectively.

Conclusions. Complete surgical removal of craniopharyngioma can be achieved with reasonable safety in more than 70% of patients. Recurrence of craniopharyngioma may occur even after apparent radical excision. Prompt management of residual or recurring disease by radiotherapy, repeat surgery, or a combination of both is usually successful in controlling further tumor growth.

Endocrinologic, neurologic, and visual morbidity after treatment for craniopharyngioma

J Neurooncol (2011) 101:463–476. DOI 10.1007/s11060-010-0265-y

Craniopharyngiomas are locally aggressive tumors which typically are focused in the sellar and suprasellar region near a number of critical neural and vascular structures mediating endocrinologic, behavioral, and visual functions. The present study aims to summarize and compare the published literature regarding morbidity resulting from treatment of craniopharyngioma.

We performed a comprehensive search of the published English language literature to identify studies publishing outcome data of patients undergoing surgery for craniopharyngioma. Comparisons of the rates of endocrine, vascular, neurological, and visual complications were performed using Pearson’s chi-squared test, and covariates of interest were fitted into a multivariate logistic regression model.

In our data set, 540 patients underwent surgical resection of their tumor. 138 patients received biopsy alone followed by some form of radiotherapy. Mean overall follow-up for all patients in these studies was 54 ± 1.8 months. The overall rate of new endocrinopathy for all patients undergoing surgical resection of their mass was 37% (95% CI = 33– 41). Patients receiving GTR had over 2.5 times the rate of developing at least one endocrinopathy compared to patients receiving STR alone or STR + XRT (52 vs. 19 vs. 20%, v2 P<0.00001). On multivariate analysis, GTR conferred a significant increase in the risk of endocrinopathy compared to STR + XRT (OR = 3.45, 95% CI = 2.05–5.81, P<0.00001), after controlling for study size and the presence of significant hypothalamic involvement. There was a statistical trend towards worse visual outcomes in patients receiving XRT after STR compared to GTR or STR alone (GTR = 3.5% vs. STR 2.1% vs. STR + XRT 6.4%, P = 0.11). Given the difficulty in obtaining class 1 data regarding the treatment of this tumor, this study can serve as an estimate of expected outcomes for these patients, and guide decision making until these data are available.

Magnetic resonance imaging–graded hypothalamic compression in surgically treated adult craniopharyngiomas determining postoperative obesity

Neurosurg Focus 28 (4):E3, 2010. DOI: 10.3171/2010.1.FOCUS09303

Obesity as a consequence of management of pediatric craniopharyngioma is a well-described phenomenon related to the degree of hypothalamic involvement. However, weight change and obesity have not been analyzed in adult patients. Therefore, the purpose of this study was 1) to evaluate the pattern of postoperative weight gain related to preoperative body mass index (BMI), 2) determine if postoperative weight gain is an issue in adult patients, and 3) develop an objective MR imaging grading system to predict risk of postoperative weight gain and obesity in adults treated for craniopharyngioma.

Methods. The authors retrospectively screened 296 patients with known craniopharyngioma for the following inclusion criteria: pathologically confirmed craniopharyngioma, index surgery at the authors’ institution, and operative weight and height recorded with at least 3 months of follow-up including body weight measurement. Patients aged 18 years or younger were excluded, yielding 28 cases for analysis. Cases of craniopharyngiomas were compared with age- and sex-matched controls (pituitary adenoma patients) to evaluate the pattern and significance of perioperative weight changes.

Results. Mean age was 46 ± 17 years at surgery, and 64% of the patients were male. Complete resection was achieved in 71% of cases. There was no correlation of preoperative BMI and postoperative weight gain testing in a linear model. Sixty-one percent and 46% of patients had postoperative weight gains greater than 4 and 9%, respectively. Comparing craniopharyngioma patients (cases) to age- and sex-matched controls, the preoperative BMIs were similar (p = 0.93) between cases (mean 28.9 [95% CI 30.9–26.9]) and controls (mean 29.3 [95% CI 31.9–26.7]). However, there was a trend to a greater mean postoperative weight change (percentage) in cases (10.1%) than in controls (5.6%) (p = 0.24). Hypothalamic T2 signal change and irregular contrast enhancement correlated and predicted higher-grade hypothalamic involvement. Furthermore, they can be used to objectively grade hypothalamic involvement as the authors propose. Progressive hypothalamic involvement correlated with larger postoperative weight gains (p = 0.022); however, hypothalamic involvement did not correlate with preoperative BMI (p = 0.5).

Conclusions. Postoperative weight gain in adult patients undergoing surgery for craniopharyngioma is a significant problem and correlates with hypothalamic involvement, as it does in pediatric patients. Finally, objective MR imaging criteria can be used to predict risk of postoperative weight gain and aid in grading of hypothalamic involvement.

Craniopharyngiomas: intratumoral chemotherapy with interferon-α: a multicenter preliminary study with 60 cases

Neurosurg Focus 28 (4):E12, 2010. (DOI: 10.3171/2010.1.FOCUS09310)

The authors assessed the efficacy of intratumoral interferon-α (IFNα)–based chemotherapy in pediatric patients with cystic craniopharyngiomas.

Methods. In a prospective multicenter study of 60 pediatric patients, the authors assessed the efficacy of intratumoral INFα2A-based chemotherapy. The study was conducted between 2000 and 2009 at 3 locations: the Medical School of the Federal University of São Paulo, Catholic University of Rome, and the Neurosurgery Institute of Santiago, Chile. The assessment included clinical and radiological control examinations, side effects observed, and total dose used.

Results. Sixty cases of cystic craniopharyngioma were analyzed. The cohort consisted of 35 male and 25 female children (mean age 11 years). Clinical and radiological improvement was achieved in 76% of the cases. New endocrinological deficits were observed in 13% of the cases. In approximately 30% of the patients, the evolution included some light side effects, the most common being headache (33%) and eyelid edema (28%). The number of cycles varied from 1 to 9 (mean 5 cycles), and the total dose applied per cycle was 36,000,000 IU.

Conclusions. This has been the largest documented series of intratumoral chemotherapy using INFα for the control of cystic craniopharyngiomas. The treatment has proved efficacious; there was no mortality, and morbidity rates were low.


Tolerance of the Optic Apparatus in Single-Fraction Irradiation Using Stereotactic Radiosurgery: Evaluation in 100 Patients With Craniopharyngioma

Neurosurgery 66:688-695, 2010. DOI: 10.1227/01.NEU.0000367554.96981.26

OBJECTIVE: To determine the limiting dose to the optic apparatus in single-fraction irradiation in patients with craniopharyngioma treated with gamma knife radiosurgery (GKRS).

METHODS: One hundred patients with 109 craniopharyngiomas treated with GKRS were evaluated with a median follow-up period of 68 months. Tumor volume varied from 0.1 to 36.0 (median, 3.3) cm3. Marginal doses varied from 10 to 18 (median, 11.4) Gy. Maximum dose to any part of the optic apparatus varied from 2 to 18 (median, 10) Gy.

RESULTS: The actuarial 5- and 10-year overall rates of survival of tumor progression after GKRS were 93% and 88%, respectively. Similarly, the actuarial 5- and 10-year progressionfree survival rates were 62% and 52%, respectively. Among 94 patients in whom visual function was evaluable after GKRS, only 3 patients developed radiation-induced optic neuropathy, indicating an overall Kaplan-Meier radiation-induced optic neuropathy rate of 5%. Of these patients, 2 received 15 Gy or greater to the optic apparatus. Another patient who received 8 Gy or less had undergone previous fractionated radiation therapy with a biologically effective dose of 60 Gy.

CONCLUSION: The optic apparatus seems to be more tolerant of irradiation than previously thought. Careful dose planning is essential, particularly in patients who underwent prior external beam radiation therapy.

The role of radiosurgery in the treatment of craniopharyngiomas

Neurosurg Focus 28 (4):E11, 2010. (DOI: 10.3171/2010.2.FOCUS09311)

The treatment of craniopharyngiomas is composed of an intricate balance of multiple modalities. Resection and radiotherapy have been combined to synergistically control tumor growth while preventing undue harm to crucial neurovascular structures. Although a craniopharyngioma is a benign lesion pathologically, it may induce severe neurological injury due to its location and rate of growth. More recently, the advent of targeted, fractionated radiotherapy has allowed for more aggressive tumor control while reducing the necessity for large resections. Initial studies have demonstrated significant tumor control in patients who are treated with resection combined with radiation therapy, versus surgery alone, with a lower rate of treatment-associated neurological deficits. In this review, a detailed account of the current studies evaluating the role of stereotactic radiosurgery in the management of craniopharyngiomas is presented. The authors also provide a short account of their experience to aid in defining the role of CyberKnife radiosurgery.

Early outcomes of endoscopic transsphenoidal surgery for adult craniopharyngiomas

Neurosurg Focus 28 (4):E9, 2010. (DOI: 10.3171/2010.1.FOCUS09319)

Although the transsphenoidal approach for subdiaphragmatic craniopharyngiomas has been performed for many years, there are few reports describing the role of the endoscopic transsphenoidal technique for suprasellar craniopharyngiomas. The purpose of this study was to report the outcomes of the endoscopic transsphenoidal approach for adults with craniopharyngiomas in whom the goal was gross-total resection.

Methods. Twelve patients were identified who were older than 18 years at the time of their pure endoscopic transsphenoidal surgery. Their medical records and imaging studies were retrospectively reviewed.

Results. Gross-total resection was achieved in 42% of cases when assessed by intraoperative impression alone and in 75% when assessed by the first postoperative MR imaging study. However, 83% of patients achieved at least a 95% resection when assessed by both intraoperative impression and the first postoperative MR imaging study. Permanent diabetes insipidus occurred postoperatively in 44% of patients. Six (67%) of 9 patients who had a functioning hypothalamic-pituitary axis preoperatively developed panhypopituitarism after surgery. Visual improvement or normalization occurred in 78% of patients with preoperative visual deficits. Although no patient experienced a postoperative CSF leak, 1 patient was treated for meningitis.

Conclusions. The authors have achieved a high rate of radical resection and symptomatic improvement with the endoscopic transsphenoidal technique for both subdiaphragmatic (sellar/suprasellar) and supradiaphragmatic (suprasellar) craniopharyngiomas. However, this is also associated with a high incidence of new endocrinopathy. Endoscopic assessment of tumor resection may be more sensitive for residual tumor than the first postoperative MR imaging study.

Anterior Interhemispheric Approach for 100 Tumors in and Around the Anterior Third Ventricle

Neurosurgery 66[ONS Suppl 1]:ons65-ons74, 2010 DOI: 10.1227/01.NEU.0000365550.84124.BB

We report our experience with anterior interhemispheric approach for tumors in and around the anterior third ventricle, including surgical technique, instrumentation, pre- and postoperative hormonal disturbances, and resection rate.

METHODS: One hundred patients with 46 craniopharyngiomas, 12 hypothalamic gliomas, 12 meningiomas, 6 hypothalamic hamartomas, and 24 other lesions were operated on using an anterior interhemispheric approach with or without opening of the lamina terminalis. This surgical approach involves no frontal sinus opening; a narrow (approximately 15–20 mm in width) access between the bridging veins, which is sufficient to remove the tumor totally; and sparing of the anterior communicating artery. Specially designed long bipolar forceps and scissors are necessary for this approach, and concomitant use of angled instruments (endoscope, aspirator, and microforceps) is required frequently. The postsurgical follow-up period varied from 4 months to 18 years. RESULTS: Total removal of the neoplasm was accomplished in 37 of 46 patients with craniopharyngiomas (80.4%), whereas subtotal resection was performed in hypothalamic gliomas. No significant differences in pre- and postoperative hormonal disturbances were observed in 37 craniopharyngiomas and 10 hypothalamic gliomas. There was no operative mortality. Visual acuity was preserved or improved in 68 of 75 patients assessed. The Karnofsky Performance Scale score did not deteriorate in 72 of 75 patients tested.

CONCLUSION: The minimally invasive anterior interhemispheric approach, with or without opening of the lamina terminalis, is useful for removal of tumors in and around the anterior third ventricle, such as craniopharyngiomas and hypothalamic gliomas.

Optimal treatment strategy for craniopharyngiomas based on long-term functional outcomes of recent and past treatment modalities

Neurosurg Rev. DOI 10.1007/s10143-009-0220-6

Although many authors have described treatment strategies for craniopharyngiomas, the optimal treatment of craniopharyngiomas remains controversial. This study aimed to define an adequate surgical strategy for craniopharyngiomas by reviewing the long-term functional performance of patients treated by current and past treatment modalities. Fifty-five patients with longer than

5 years of follow-up were selected for the present long-term study. The duration of follow-up ranged from 5.5 to 33 years (median, 14.8 years). There were 28 adult patients (14 males; median age, 44.4 years) and 27 children younger than 16 years of age (15 males; median age, 8.1 years). The patients were divided into the following treatment groups: single surgery (group A; n=14), multiple surgeries (group B; n=8), surgery or surgeries followed by radiotherapy (group C; n=23), surgery or surgeries (partial removal) followed by radiotherapy + additional treatments (multiple surgeries and/or re-irradiation; group D; n=10). In addition to the routine assessments of neurological, endocrine, and visual outcomes, the level of daily functioning was analyzed using the Karnofsky Performance Scale (KPS). Statistical analysis of relationship between KPS score and treatment mode demonstrated that group D had a significantly lower KPS score (F=5.82, p=0.0017). Furthermore, mortality, cognitive function, and visual function were significantly better in groups A, B, and C than in group D. Multiple regression analysis demonstrated that cognitive dysfunction, visual disturbance, and treatment mode were independent covariates that significantly affected postoperative

KPS score. Adequate primary treatment for craniopharyngiomas is important to avoid subsequent multiple treatments. Craniopharyngiomas should be removed surgically as far as possible but without further deteriorating cognitive and visual functions, either as total resection or subtotal resection with a small remnant that is controllable by radiation therapy.

Adult craniopharyngiomas: surgical results with a special focus on endocrinological outcomes and recurrence according to pituitary stalk preservation

DOI: 10.3171/2008.10.JNS0880

The aim of this study was to evaluate the results of surgical treatment of adult craniopharyngioma with a special focus on the endocrinological outcomes and tumor recurrence in cases of pituitary preservation.

Methods: Between 1993 and February 2008, 41 patients underwent 47 surgical procedures for craniopharyngioma. The male/female ratio was 26:15 and the median age was 45.8 years (range 17–65 years). The median follow-up period was 10.56 years (range 6.2–14.9 years). Patients presented with visual disturbance before 30 (63.8%) of 47 procedures and with endocrinological disturbance before 12 (26%) procedures. Surgery was performed via a subfrontal/pterional approach in 31 procedures (66%), bifrontal interhemispheric in 6 (12.8%), transcallosal/transventricular in 5 (10.6%), combined in 1 (2.1%), and transsphenoidal in 4 (8.5%). The tumor was totally removed in 36 procedures (76.6%), subtotally in 10 (21.3%), and partially in 1 (2.1%).

Results: Postoperatively, the rates of visual improvement and aggravation were 50 and 33.3%, respectively. Of 24 patients in whom the pituitary stalk was preserved, complete hormone replacement was needed in 14 (58.3%), partial replacement in 2 (8.3%), and no replacement in 8 (33.3%). The rate of tumor recurrence was 24.4%. The recurrence-free survival rate was significantly different between patients in whom complete tumor resection was accomplished and those in whom tumor resection was incomplete. Stalk preservation did not affect the recurrence-free survival rate. The morbidity and mortality rates were 8.5 and 2.1%, respectively.

Conclusions: The pituitary stalk must be preserved with maximal tumor resection whenever possible to increase the chance of intact anterior pituitary function being maintained. The results of the present study show that pituitary stalk preservation may not be related to increased recurrence rates.

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