The influence of tumor topography on the surgical outcome of craniopharyngiomas

J Neurosurg 139:1247–1257, 2023

Various topographical classifications for craniopharyngioma have been proposed based on their relationship with optic chiasm and the third ventricular floor. There is a paucity of literature evaluating the surgical outcome based on tumor topography. This study aims to compare the surgical outcomes of retrochiasmatic craniopharyngiomas (RCPs) and nonretrochiasmatic craniopharyngiomas (non-RCPs).

METHODS This retrospective study includes newly diagnosed patients with craniopharyngioma who underwent surgery between January 2000 and December 2015. Clinical features, the extent of resection (EOR), surgical outcomes, tumor recurrence, and progression-free survival (PFS) of craniopharyngiomas were compared with respect to their relationship to the optic chiasm and third ventricular floor.

RESULTS The authors identified RCPs in 104 and non-RCPs in 33 patients. RCPs were significantly larger and more associated with hydrocephalus than were non-RCPs (p < 0.001) at the time of diagnosis. Puget grade 2 hypothalamic involvement was more frequent with RCPs. EOR and PFS following either subtotal resection (p = 0.07) or gross-total resection (p = 0.7) were comparable between RCPs and non-RCPs. There was no significant difference in the postoperative visual outcome. Resection of RCPs resulted in higher postoperative hypopituitarism (64% vs 42%, p = 0.01) and hypothalamic dysfunction (18% vs 3%, p = 0.02). Location of the tumor, either retrochiasmatic (HR 0.5; 95% CI 0.14–2.2; p = 0.4) or nonretrochiasmatic (HR 1.3; 95% CI 0.3–5.5; p = 0.6), did not show association with recurrence. RCPs with extraand intraventricular components (type 3b) had a higher incidence of postoperative hypothalamic morbidities (p = 0.01) and tumor recurrence (36% vs 19%; p = 0.05) during follow-up than the extraventricular (type 3a) RCP. Between prechiasmatic and infrachiasmatic/intrasellar craniopharyngiomas, EOR (p = 0.7), postoperative diabetes insipidus (p = 0.4), endocrinological outcome (p = 0.7), and recurrence (p = 0.1) were comparable. The patients with complex multicompartmental tumors had a lower rate of gross-total resection (25%, p = 0.02) and a higher incidence of tumor recurrence (75%, p = 0.004) than the rest.

CONCLUSIONS The tumor topography can influence the postoperative outcome. RCPs can be associated with a higher incidence of hypopituitarism and hypothalamic morbidities postoperatively. The influence of topography on EOR and tumor recurrence is controversial. However, this study did not find a significant difference in EOR and tumor recurrence between RCPs and non-RCPs. PFS and overall mortality are also comparable.

Predictors of extent of resection and recurrence following endoscopic endonasal resection of craniopharyngioma

J Neurosurg 139:1235–1246, 2023

Craniopharyngioma is a benign but surgically challenging brain tumor. Controversies exist regarding its ideal treatment strategy, goals of surgery, efficacy of radiation, and the long-term outcomes of these decisions. The authors of this study performed a detailed analysis of factors predictive of the extent of resection and recurrence in large series of craniopharyngiomas removed via an endoscopic endonasal approach (EEA) with long-term follow-up.

METHODS From a prospective database of all EEAs done at Weill Cornell Medical College by the senior author from 2004 to 2022, a consecutive series of histologically proven craniopharyngiomas were identified. Gross-total resection (GTR) was generally the goal of surgery. Radiation was often given if GTR had not been achieved. The stalk was preserved if not infiltrated with tumor but was sacrificed to achieve GTR. Intentional subtotal resection (STR) was performed in select cases to avoid hypothalamic injury.

RESULTS Among the 111 identified cases were 88 adults and 23 children. Newly diagnosed cases comprised 58.6% of the series. GTR was attempted in 77.5% of the patients and among those cases was achieved in 89.5% of treatmentnaive tumors and 72.4% of recurrent tumors. An inability to achieve GTR was predicted by prior surgical treatment (OR 0.13, 95% CI 0.03–0.6, p = 0.009), tumor diameter ≥ 3.5 cm (OR 0.11, 95% CI 0.02–0.53, p = 0.006), and encasement of the optic nerve or a major artery (OR 0.11, 95% CI 0.01–0.8, p = 0.03). GTR with stalk preservation maintained some anterior pituitary function in 64.5% of cases and prevented diabetes insipidus in 25.8%. After a median follow-up of 51 months (IQR 17–80 months), the recurrence rate after GTR was 12.5% compared with 38.5% after non-GTR. The median recurrence-free survival was 5.5 years after STR, 8.3 years after near-total resection (≥ 98%), and not reached after GTR (p = 0.004, log-rank test). GTR was the strongest predictor of recurrence-free survival (OR 0.09, 95% CI 0.02–0.42, p = 0.002), whereas radiation did not show a statistically significant impact (OR 1.17, 95% CI 0.45–3.08). In GTR cases, the recurrence rate was higher if the stalk had been preserved (22.6%) as opposed to a sacrificed stalk (4.9%; OR 5.69, 95% CI 1.09–29.67).

CONCLUSIONS The study data show that GTR should be the goal of surgery in craniopharyngiomas if it can be achieved safely. Although stalk preservation can maintain some endocrine function, the risk of recurrence is higher in such cases. Radiation may not be as effective as previously reported.

Expanded endoscopic endonasal approach for the resection of midline craniopharyngiomas with hypothalamic involvement

Acta Neurochirurgica (2022) 164:3291–3296

With relevant surrounding neurological structures and potential involvement of the hypothalamus, the surgical management of craniopharyngiomas is complex. Compared to the transcranial approach, the expanded endoscopic endonasal approach provides direct access to the supradiaphragmatic and retrochiasmatic areas without crossing nerves and arteries.

Method Based on our substantial experience of 68 patients operated on between 2008 and 2022 by endoscopic surgery, our strategy has evolved such that all of our midline infundibular craniopharyngiomas with hypothalamic involvement are currently treated with an expanded endonasal route, except for tumours isolated to the third ventricle. Vascularized mucosal nasoseptal flaps are required for closure. Fine details of the related anatomy and surgical technique are described.

Conclusion Expanded endoscopic endonasal approach is a safe and effective route for resection of midline suprasellar craniopharyngiomas with hypothalamic involvement in centres of expertise.

Endoscopic endonasal versus transcranial surgery for primary resection of craniopharyngiomas based on a new QST classification system

J Neurosurg 135:1298–1309, 2021

An assessment of the transcranial approach (TCA) and the endoscopic endonasal approach (EEA) for craniopharyngiomas (CPs) according to tumor types has not been reported. The aim of this study was to evaluate both surgical approaches for different types of CPs.

METHODS A retrospective review of primary resected CPs was performed. A QST classification system based on tumor origin was used to classify tumors into 3 types as follows: infrasellar/subdiaphragmatic CPs (Q-CPs), subarachnoidal CPs (S-CPs), and pars tuberalis CPs (T-CPs). Within each tumor type, patients were further arranged into two groups: those treated via the TCA and those treated via the EEA. Patient and tumor characteristics, surgical outcomes, and postoperative complications were obtained. All variables were statistically analyzed between surgical groups for each tumor type.

RESULTS A total of 315 patients were included in this series, of whom 87 were identified with Q-CPs (49 treated via TCA and 38 via EEA); 56 with S-CPs (36 treated via TCA and 20 via EEA); and 172 with T-CPs (105 treated via TCA and 67 via EEA). Patient and tumor characteristics were equivalent between both surgical groups in each tumor type. The overall gross-total resection rate (90.5% TCA vs 91.2% EEA, p = 0.85) and recurrence rate (8.9% TCA vs 6.4% EEA, p = 0.35) were similar between surgical groups. The EEA group had a greater chance of visual improvement (61.6% vs 35.8%, p = 0.01) and a decreased risk of visual deterioration (1.6% vs 11.0%, p < 0.001). Of the patients with T-CPs, postoperative hypothalamic status was better in the TCA group than in the EEA group (p = 0.016). Postoperative CSF leaks and nasal complication rates occurred more frequently in the EEA group (12.0% vs 0.5%, and 9.6% vs 0.5%; both p < 0.001). For Q-CPs, EEA was associated with an increased gross-total resection rate (97.4% vs 85.7%, p = 0.017), decreased recurrence rate (2.6% vs 12.2%, p = 0.001), and lower new hypopituitarism rate (28.9% vs 57.1%, p = 0.008). The recurrence-free survival in patients with Q-CPs was also significantly different between surgical groups (log-rank test, p = 0.037). The EEA required longer surgical time for T-CPs (p = 0.01).

CONCLUSIONS CPs could be effectively treated by radical surgery with favorable results. Both TCA and EEA have their advantages and limitations when used to manage different types of tumors. Individualized surgical strategies based on tumor growth patterns are mandatory to achieve optimal outcomes.

An endoscopic endonasal approach to craniopharyngioma via the infrachiasmatic corridor

Acta Neurochirurgica (2021) 163:2253–2268

The infrachiasmatic corridor is the most important surgical access route for craniopharyngiomas and was identified and used in clinical series. The aims of this study were to describe the characteristics that assist dissection and resection rates in endoscopic surgery of solid, cystic, and recurrent cases and their importance in the infrachiasmatic corridor in endoscopic surgery.

Methods One hundred operations on 84 patients with pathologically identified craniopharyngioma were included in the study. The MRI findings were evaluated, and the location of the lesions was classified as (1) infrasellar; (2) sellar; or (3) suprasellar. In the sagittal plane, we measured the longest diameter of cystic and solid components and the height of chiasm-sella. Images were assessed for the extent of resection and were classified as gross total resection. This was deemed as the absence of residual tumor and subtotal resection, which had residual tumor.

Results The infrasellar location was reported in 7/84 (8.3%) patients, the sellar location in 8/84 (9.5%), and the suprasellar location in 69/84 (82.1%) patients. The narrow and high chiasm-sella were observed in 28/69 (40.5%) and 41/69 patients (59.4%), respectively. The mean distance of the chiasm-sella was 9.46± 3.76. Gross total tumor resection was achieved in 60/ 84 (71.4%) and subtotal tumor resection was performed in 24/84 (28.6%) patients. The results revealed that suprasellar location (OR: 0.068; p = 0.017) and recurrent cases (OR: 0.011; p<0.001) were negative predictive factors on GTR. Increasing the experience (OR: 42,504; p = 0.001) was a positive predictor factor for GTR.

Conclusion An EETS approach that uses the infrachiasmatic corridor is required for skull base lesions extending into the suprasellar area. The infrachiasmatic corridor can determine the limitations of endoscopic craniopharyngioma surgery. This corridor is a surgical safety zone for inferior approaches.

Diabetes Insipidus After Endoscopic Transsphenoidal Surgery

Neurosurgery 2020 DOI:10.1093/neuros/nyaa148

Diabetes insipidus (DI) is a recognized transient or permanent complication following transsphenoidal surgery (TSS) for pituitary tumors.

OBJECTIVE: To describe significant experience with the incidence of DI after TSS, identifying predictive characteristics and describing our diagnosis and management of postoperative DI.

METHODS: A retrospective analysis was performed of 700 patients who underwent endoscopic TSS for resection of pituitary adenoma (PA), Rathke cleft cyst (RCC), or craniopharyngioma. Inclusion criteria included at least 1 wk of follow-up for diagnosis of postoperative DI. Permanent DI was defined as DI symptoms and/or need for desmopressinmore than 1 yr postoperatively. All patients with at least 1 yr of follow-up (n=345) were included in analyses of permanent DI. Multivariable logistic regression models were constructed to identify predictors of transient or permanent postoperative DI.

RESULTS: The overall rate of any postoperative DI was 14.7% (103/700). Permanent DI developed in 4.6% (16/345). The median follow-up was 10.7mo (range: 0.2-136.6). Compared to patients with PA, patients with RCC (odds ratio [OR] = 2.2, 95% CI: 1.2-3.9; P = .009) and craniopharyngioma (OR = 7.0, 95% CI: 2.9-16.9; P ≤ .001) were more likely to develop postoperative DI. Furthermore, patients with RCC (OR = 6.1, 95% CI: 1.8-20.6; P = .004) or craniopharyngioma (OR = 18.8, 95% CI: 4.9-72.6; P ≤ .001) were more likely to develop permanent DI compared to those with PA.

CONCLUSION: Although transient DI is a relatively common complication of endoscopic and microscopic TSS, permanent DI is much less frequent. The underlying pathology is an important predictor of both occurrence and permanency of postoperative DI.



Clinical features and operative technique of transinfundibular craniopharyngioma

J Neurosurg 133:119–128, 2020

Transinfundibular craniopharyngioma (TC) is one of the 4 subtypes of suprasellar craniopharyngioma. In this study, the authors analyzed the clinical features of and operative technique for TC.

METHODS A total of 95 consecutive cases of suprasellar craniopharyngioma that had been resected via the endoscopic expanded endonasal approach were retrospectively reviewed. Patients were divided into 2 groups: 34 in the TC group and 61 in the nontransinfundibular craniopharyngioma (NC) group. Clinical and radiographic features, intraoperative findings, histopathological and genetic findings, and surgical outcomes were analyzed and compared between groups.

RESULTS Compared with NC, TC was mostly seen in adult patients (97.1%); it was rare in children (2.9%). Clinical presentations tended toward headache, hydrocephalus, and diabetes insipidus. The relatively smaller volume, midline location (consistent with the stalk position), unidentifiable stalk, no shift of the third ventricle, and greater likelihood to involve the third ventricle and cause hydrocephalus were the characteristic features of TC in the preoperative MRI study. According to the degree of vertical extension of the tumor, the 34 TCs could be classified into 3 subtypes: type 1, entity was limited to stalk (n = 2, 5.9%); type 2, tumor extended up to the third ventricle (type 2a) or down to the subdiaphragmatic cavity (type 2b) (n = 23, 67.6%); and type 3, tumor extended in both directions (n = 9, 26.5%). For TC resection, the chiasm–pituitary corridor, lamina terminalis corridor, and pituitary corridor could be used separately or jointly. Most of the TCs originated from the infundibulum–tuber cinereum, grew within and along the long axis of the infundibulum, and the pituitary stalk was not usually preserved in TCs (20.6%), whereas the rate of preservation was higher (80.3%) in NCs. Bilateral hypothalamic injury was found in nearly all TCs if radical resection was performed, whereas the relationship between NCs and hypothalamus was either compression (32.8%) or unilateral invasion (67.2%). Meanwhile, the postoperative endocrine and neuropsychological function outcomes in patients with TC were worse than in patients with NC. The genetic analysis with whole-exome sequencing studies showed no differential mutations of CTNNB1 (b-catenin) and BRAF (V600E) between TC and NC subtypes, but there was a difference between adamantinomatous craniopharyngioma and papillary craniopharyngioma.

CONCLUSIONS TC is a special subtype of suprasellar craniopharyngioma, which is remarkably different from NC. Identification of this type of tumor preoperatively is essential for the planning of appropriate surgical approach and degree of excision.

Fully endoscopic combined transsphenoidal and supraorbital keyhole approach for parasellar lesions

J Neurosurg 128:685–694, 2018

Parasellar tumors that extend far laterally beyond the internal carotid artery or that are fibrous and adhere firmly to critical structures are difficult to remove totally via the endoscopic transsphenoidal approach alone. In such cases, a combined transsphenoidal-transcranial approach is effective to achieve maximal resection in a single stage. In this paper, a new minimally invasive surgical technique for complicated parasellar lesions, a fully endoscopic combined transsphenoidal–supraorbital keyhole approach, is presented.

METHODS A retrospective review of patients who had been treated via a fully endoscopic combined transsphenoidal– supraorbital keyhole approach for complicated parasellar lesions was performed. The data for resection rate, perioperative mortality and morbidity, and postoperative outcomes were analyzed.

RESULTS A total of 12 fully endoscopic combined transsphenoidal–supraorbital keyhole approaches were performed from March 2013 to February 2016; 10 were for pituitary adenomas and 2 were for craniopharyngiomas. Gross-total resection or near-total resection was achieved in 7 of 12 cases. Among the 11 patients who had presented with preoperative visual disturbances, 7 had visual improvement. However, 1 patient showed deterioration in visual function. No patient experienced postoperative hemorrhage, needed additional surgical treatment, or had postoperative CSF leakage.

CONCLUSIONS In the combined transsphenoidal and transcranial approach, safe and effective cooperative manipulation with 2 surgical corridors can be performed for complicated parasellar lesions. The goal of this procedure is not to achieve gross-total resection, but to achieve safe resection. Moreover, this new surgical approach offers neurosurgeons a simpler operative field with less invasiveness than the conventional microscopic combined approach. The fully endoscopic combined endonasal–supraorbital keyhole approach is an efficacious procedure for complicated parasellar lesions with acceptable results.


Cystic Craniopharyngiomas: Microsurgical or Stereotactic Treatment?

Neurosurgery (2017) 80 (5): 733-743

Prognosis and treatment of cystic craniopharyngiomas are poorly defined.

OBJECTIVE: To analyze progression-free survival (PFS) and safety profile of cystic craniopharyngiomas undergoing resection or minimally invasive drainage procedures. We compared further outcome measurements for cystic and solid tumors undergoing resection to elucidate the impact of the initial tumor composition on both PFS and the toxicity profile.

METHODS: All patients with craniopharyngiomas consecutively treated between 1999 and 2014 were included. A treatment decision in favor of microsurgery or stereotactic treatment was made interdisciplinarily. For stereotactic drainage, a catheter was implanted, allowing both permanent upstream (into ventricular spaces) and downstream (into prepontine cistern) drainage. Study endpoints were tumor progression, functional outcome, and treatment toxicity. Functional endocrinological and visual outcome analyses referred to data obtained preoperatively and 6 weeks after treatment. The Kaplan-Meier method was used for survival analysis. Prognostic factors were obtained from proportional hazard models.

RESULTS: Seventy-nine patients were included. The distribution of clinical and tumor-related data was well balanced among patients with solid (n = 35) and cystic (n = 44) tumors and those undergoing microsurgical or stereotactic treatment. Cystic tumors had shorter PFS (5-year PFS: 53.6% vs 66.8%, P= .10) and needed significantly more therapeutic interventions, which was independent of the initial treatment mode. The endocrinological deterioration rate was high for both solid and cystic tumors after microsurgery (59.4% and 85.7%, respectively), whereas it was significantly lower for cystic tumors undergoing stereotactic treatment (23.1%, P < .001).

CONCLUSION: Stereotactic bidirectional drainage of cystic craniopharyngiomas is effective and provides a better endocrinological outcome than conventional microsurgery.

Outcomes in craniotomy vs endoscopic craniopharyngioma resection

Neurosurg Focus 41 (6):E6, 2016

Craniopharyngiomas have historically been resected via transcranial microsurgery (TCM). In the last 2 decades, the extended endoscopic endonasal (transtuberculum) approach to these tumors has become more widely accepted, yet there remains controversy over which approach leads to better outcomes. The purpose of this study is to determine whether differences in outcomes were identified between TCM and extended endoscopic endonasal approaches (EEEAs) in adult patients undergoing primary resection of suprasellar craniopharyngiomas at a single institution.

Methods A retrospective review of all patients who underwent resection of their histopathologically confirmed craniopharyngiomas at the authors’ institution between 2005 and 2015 was performed. Pediatric patients, revision cases, and patients with tumors greater than 2 standard deviations above the mean volume were excluded. The patients were divided into 2 groups: those undergoing primary TCM and those undergoing a primary EEEA. Preoperative patient demographics, presenting symptoms, and preoperative tumor volumes were determined. Extent of resection, tumor histological subtype, postoperative complications, and additional outcome data were obtained. Statistical significance between variables was determined utilizing Student t-tests, chi-square tests, and Fisher exact tests when applicable.

Results After exclusions, 21 patients satisfied the aforementioned inclusion criteria, 12 underwent TCM for resection while 9 benefitted from the EEEA. There were no significant differences in patient demographics, presenting symptoms, tumor subtype, or preoperative tumor volumes, no tumors had significant lateral or prechiasmatic extension. The extent of resection was similar between these 2 groups, as was the necessity for additional surgery or adjuvant therapy. CSF leakage was encountered only in the EEEA group (2 patients). Importantly, the rate of postoperative visual improvement was significantly higher in the EEEA group than in the TCM group (88.9% vs 25.0%, p = 0.0075). Postoperative visual deterioration only occurred in the TCM group (3 patients). Recurrence was uncommon, with similar rates between the groups. Other complication rates, overall complication risk, and additional outcome measures were similar between these groups as well.

Conclusions Based on this study, most outcome variables appear to be similar between TCM and EEEA routes for similarly sized tumors in adults. The multidisciplinary EEEA to craniopharyngioma resection represents a safe and compelling alternative to TCM. The authors’ data demonstrate that postoperative visual improvement is statistically more likely in the EEEA despite the increased risk of CSF leakage. These results add to the growing evidence that the EEEA may be considered the approach of choice for resection of select confined primary craniopharyngiomas without significant lateral extension in centers with experienced surgeons. Further prospective, multiinstitutional collaboration is needed to power studies capable of fully evaluating indications and appropriate approaches for craniopharyngiomas.

Microscopic versus endoscopic approaches for craniopharyngiomas


Neurosurg Focus 41 (6):E5, 2016

Resection remains the mainstay of treatment for craniopharyngiomas with the goal of radical resection, if safely possible, to minimize the rate of recurrence. Endoscopic endonasal and microscopic transcranial surgical approaches have both become standard methods for the treatment for craniopharyngiomas. However, the approach selection paradigm for craniopharyngiomas is still a point of discussion. Choosing the optimal surgical approach can play a significant role in maximizing the extent of resection and surgical outcome while minimizing the risks of potential complications.

Craniopharyngiomas can present with a variety of different sizes, locations, and tumor consistencies, and each individual tumor has distinct features that favor one specific approach over another.

The authors review standard cranial base techniques applied to craniopharyngioma surgery, using both the endoscopic endonasal approach and traditional open microsurgical approaches, and analyze factors involved in approach selection. They discuss their philosophy of approach selection based on the location and extent of the tumor on preoperative imaging as well as the advantages and limitations of each surgical corridor, and they describe the operative nuances of each technique, using a personalized, tailored approach to the individual patient with illustrative cases and videos.

Optic nerve mobilization to enhance the exposure of the pituitary stalk during craniopharyngioma resection


J Neurosurg 125:683–688, 2016

Preservation of the pituitary stalk and its vasculature is a key step in good postoperative endocrinological outcome in patients with craniopharyngiomas. In this article, the authors describe the surgical technique of medial optic nerve mobilization for better inspection and preservation of the pituitary stalk.

Methods This operative technique has been applied in 3 patients. Following tumor exposure via a frontolateral approach, the pituitary stalk could be seen partially hidden under the optic nerve and the optic chiasm. The subchiasmatic and opticocarotid spaces were narrow, and tumor dissection from the pituitary stalk under direct vision was not possible. The optic canal was therefore unroofed, the falciform ligament was incised, and the lateral part of the tuberculum sellae was drilled medial to the optic nerve. The optic nerve could be mobilized medially to widen the opticocarotid triangle, which enhanced visualization of and access to the pituitary stalk.

Results By using the optic nerve mobilization technique, the tumor could be removed completely, and the pituitary stalk and its vasculature were preserved in all patients. In 2 patients, vision improved after surgery, while in 1 patient it remained normal, as it was before surgery. The hormonal status remained normal after surgery in 2 patients. In the patient with preoperative hormonal deficiencies, improvement occurred early after surgery and hormonal levels were normal after 3 months. No approach-related complications occurred.

Conclusions This early experience shows that this technique is safe and could be used as a complementary step during microsurgery of craniopharyngiomas. It allows for tumor dissection from the pituitary stalk under direct vision. The pituitary stalk can thus be preserved without jeopardizing the optic nerve.

Comparative analysis of outcomes following craniotomy and expanded endoscopic endonasal transsphenoidal resection of craniopharyngioma and related tumors

Endoscopic endonasal surgery for craniopharyngiomas

J Neurosurg 124:627–638, 2016

Craniopharyngiomas and similar midline suprasellar tumors have traditionally been resected via transcranial approaches. More recently, expanded endoscopic endonasal transsphenoidal approaches have gained interest. Surgeons have advocated for both approaches, and at present there is no consensus whether one approach is superior to the other. The authors therefore compared surgical outcomes between craniotomy and endoscopic endonasal transsphenoidal surgery (EETS) for suprasellar tumors treated at their institution.

Methods: A retrospective review of patients undergoing resection of suprasellar lesions at Cedars-Sinai Medical Center between 2000 and 2013 was performed. Patients harboring suspected craniopharyngioma were selected for extensive review. Other pathologies or predominantly intrasellar masses were excluded. Cases were separated into 2 groups, based on the surgical approach taken. One group underwent EETS and the other cohort underwent craniotomy. Patient demographic data, presenting symptoms, and previous therapies were tabulated. Preoperative and postoperative tumor volume was calculated for each case based on MRI. Student t-test and the chi-square test were used to evaluate differences in patient demographics, tumor characteristics, and outcomes between the 2 cohorts. To assess for selection bias, 3 neurosurgeons who did not perform the surgeries reviewed the preoperative imaging studies and clinical data for each patient in blinded fashion and indicated his/her preferred approach. These data were subject to concordance analysis using Cohen’s kappa test to determine if factors other than surgeon preference influenced the choice of surgical approach.

Results: Complete data were available for 53 surgeries; 19 cases were treated via EETS, and 34 were treated via craniotomy. Patient demographic data, preoperative symptoms, and tumor characteristics were similar between the 2 cohorts, except that fewer operations for recurrent tumor were observed in the craniotomy cohort compared with EETS (17.6% vs 42.1%, p = 0.05). The extent of resection was similar between the 2 groups (85.6% EETS vs 90.7% craniotomy, p = 0.77). An increased rate of cranial nerve injury was noted in the craniotomy group (0% EETS vs 23.5% craniotomy, p = 0.04). Postoperative CSF leak rate was higher in the EETS group (26.3% EETS vs 0% craniotomy, p = 0.004). The progression-free survival curves (log-rank p = 0.99) and recurrence rates (21.1% EETS vs 23.5% craniotomy, p = 1.00) were similar between the 2 groups. Concordance analysis of cases reviewed by 3 neurosurgeons indicated that individual surgeon preference was the only factor that determined surgical approach (kappa coefficient -0.039, p = 0.762)

Conclusions: Surgical outcomes were similar for tumors resected via craniotomy or EETS, except that more CSF leaks occurred in the EETS cohort, whereas more neurological injuries occurred in the craniotomy cohort. Surgical approach appears to mostly reflect surgeon preference rather than specific tumor characteristics. These data support the view that EETS is a viable alternative to craniotomy, providing a similar extent of resection with less neurological injury.

Endoscopic endonasal treatment of 103 craniopharyngiomas

Endoscopic endonasal treatment of 103 craniopharyngiomas

J Neurosurg 121:100–113, 2014

Despite their benign histological appearance, craniopharyngiomas can be considered a challenge for the neurosurgeon and a possible source of poor prognosis for the patient. With the widespread use of the endoscope in endonasal surgery, this route has been proposed over the past decade as an alternative technique for the removal of craniopharyngiomas.

Methods. The authors retrospectively analyzed data from a series of 103 patients who underwent the endoscopic endonasal approach at two institutions (Division of Neurosurgery of the Università degli Studi di Napoli Federico II, Naples, Italy, and Division of Neurosurgery of the Bellaria Hospital, Bologna, Italy), between January 1997 and December 2012, for the removal of infra- and/or supradiaphragmatic craniopharyngiomas. Twenty-nine patients (28.2%) had previously been surgically treated.

Results. The authors achieved overall gross-total removal in 68.9% of the cases: 78.9% in purely infradiaphragmatic lesions and 66.3% in lesions involving the supradiaphragmatic space. Among lesions previously treated surgically, the gross-total removal rate was 62.1%. The overall improvement rate in visual disturbances was 74.7%, whereas worsening occurred in 2.5%. No new postoperative defect was noted. Worsening of the anterior pituitary function was reported in 46.2% of patients overall, and there were 38 new cases (48.1% of 79) of postoperative diabetes insipidus. The most common complication was postoperative CSF leakage; the overall rate was 14.6%, and it diminished to 4% in the last 25 procedures, thanks to improvement in reconstruction techniques. The mortality rate was 1.9%, with a mean follow-up duration of 48 months (range 3–246 months).

Conclusions. The endoscopic endonasal approach has become a valid surgical technique for the management of craniopharyngiomas. It provides an excellent corridor to infra- and supradiaphragmatic midline craniopharyngiomas, including the management of lesions extending into the third ventricle chamber. Even though indications for this approach are rigorously lesion based, the data in this study confirm its effectiveness in a large patient series.

Endoscopic endonasal surgery for craniopharyngiomas: surgical outcome in 64 patients

Endoscopic endonasal surgery for craniopharyngiomas

J Neurosurg 119:1194–1207, 2013

The proximity of craniopharyngiomas to vital neurovascular structures and their high recurrence rates make them one of the most challenging and controversial management dilemmas in neurosurgery. Endoscopic endonasal surgery (EES) has recently been introduced as a treatment option for both pediatric and adult craniopharyngiomas. The object of the present study was to present the results of EES and analyze outcome in both the pediatric and the adult age groups.

Methods. The authors retrospectively reviewed the records of patients with craniopharyngioma who had undergone EES in the period from June 1999 to April 2011.

Results. Sixty-four patients, 47 adults and 17 children, were eligible for this study. Forty-seven patients had presented with primary craniopharyngiomas and 17 with recurrent tumors. The mean age in the adult group was 51 years (range 28–82 years); in the pediatric group, 9 years (range 4–18 years). Overall, the gross-total resection rate was 37.5% (24 patients); near-total resection (> 95% of tumor removed) was 34.4% (22 patients); subtotal resection (≥ 80% of tumor removed) 21.9% (14 patients); and partial resection (< 80% of tumor removed) 6.2% (4 patients). In 9 patients, EES had been combined with radiation therapy (with radiosurgery in 6 cases) as the initial treatment. Among the 40 patients (62.5%) who had presented with pituitary insufficiency, pituitary function remained unchanged in 19 (47.5%), improved or normalized in 8 (20%), and worsened in 13 (32.5%). In the 24 patients who had presented with normal pituitary function, new pituitary deficit occurred in 14 (58.3%). Nineteen patients (29.7%) suffered from diabetes insipidus at presentation, and the condition developed in 21 patients (46.7%) after treatment. Forty-four patients (68.8%) had presented with impaired vision. In 38 (86.4%) of them, vision improved or even normalized after surgery; in 5, it remained unchanged; and in 1, it temporarily worsened. One patient without preoperative visual problems showed temporary visual deterioration after treatment. Permanent visual deterioration occurred in no one after surgery. The mean follow-up was 38 months (range 1–135 months). Tumor recurrence after EES was discovered in 22 patients (34.4%) and was treated with repeat surgery (6 patients), radiosurgery (1 patient), combined repeat surgery and radiation therapy (8 patients), interferon (1 patient), or observation (6 patients). Surgical complications included 15 cases (23.4%) with CSF leakage that was treated with surgical reexploration (13 patients) and/or lumbar drain placement (9 patients). This leak rate was decreased to 10.6% in recent years after the introduction of the vascularized nasoseptal flap. Five cases (7.8%) of meningitis were found and treated with antibiotics without further complications. Postoperative hydrocephalus occurred in 7 patients (12.7%) and was treated with ventriculoperitoneal shunt placement. Five patients experienced transient cranial nerve palsies. There was no operative mortality.

Conclusions. With the goal of gross-total or maximum possible safe resection, EES can be used for the treatment of every craniopharyngioma, regardless of its location, size, and extension (excluding purely intraventricular tumors), and can provide acceptable results comparable to those for traditional craniotomies. Endoscopic endonasal surgery is not limited to adults and actually shows higher resection rates in the pediatric population.

Displacement of mammillary bodies by craniopharyngiomas involving the third ventricle

Displacement of mammillary bodies by craniopharyngiomas involving the third ventricle- surgical-MRI correlation and use in topographical diagnosis

J Neurosurg 119:381–405, 2013

Accurate diagnosis of the topographical relationships of craniopharyngiomas (CPs) involving the third ventricle and/or hypothalamus remains a challenging issue that critically influences the prediction of risks associated with their radical surgical removal. This study evaluates the diagnostic accuracy of MRI to define the precise topographical relationships between intraventricular CPs, the third ventricle, and the hypothalamus.

Methods. An extensive retrospective review of well-described CPs reported in the MRI era between 1990 and 2009 yielded 875 lesions largely or wholly involving the third ventricle. Craniopharyngiomas with midsagittal and coronal preoperative and postoperative MRI studies, in addition to detailed descriptions of clinical and surgical findings, were selected from this database (n = 130). The position of the CP and the morphological distortions caused by the tumor on the sella turcica, suprasellar cistern, optic chiasm, pituitary stalk, and third ventricle floor, including the infundibulum, tuber cinereum, and mammillary bodies (MBs), were analyzed on both preoperative and postoperative MRI studies. These changes were correlated with the definitive CP topography and type of third ventricle involvement by the lesion, as confirmed surgically.

Results. The mammillary body angle (MBA) is the angle formed by the intersection of a plane tangential to the base of the MBs and a plane parallel to the floor of the fourth ventricle in midsagittal MRI studies. Measurement of the MBA represented a reliable neuroradiological sign that could be used to discriminate the type of intraventricular involvement by the CP in 83% of cases in this series (n = 109). An acute MBA (< 60°) was indicative of a primary tuberal-intraventricular topography, whereas an obtuse MBA (> 90°) denoted a primary suprasellar CP position, causing either an invagination of the third ventricle (pseudointraventricular lesion) or its invasion (secondarily intraventricular lesion; p < 0.01). A multivariate model including a combination of 5 variables (the MBA, position of the hypothalamus, presence of hydrocephalus, psychiatric symptoms, and patient age) allowed an accurate definition of the CP topography preoperatively in 74%–90% of lesions, depending on the specific type of relationship between the tumor and third ventricle.

Conclusions. The type of mammillary body displacement caused by CPs represents a valuable clue for ascertaining the topographical relationships between these lesions and the third ventricle on preoperative MRI studies. The MBA provides a useful sign to preoperatively differentiate a primary intraventricular CP originating at the infundibulotuberal area from a primary suprasellar CP, which either invaginated or secondarily invaded the third ventricle.

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.