Endonasal endoscopic resection of esthesioneuroblastoma: the Johns Hopkins Hospital experience and review of the literature

Neurosurg Rev. DOI 10.1007/s10143-011-0329-2

Esthesioneuroblastoma is an uncommon malignant tumor originating in the upper nasal cavity. The surgical treatment for this tumor has traditionally been via an open craniofacial resection. Over the past decade, there has been tremendous development in endoscopic techniques.

In this report, we performed a retrospective analysis of patients with esthesioneuroblastomas treated with a purely endonasal endoscopic approach and resection at the Johns Hopkins Hospital between January 2005 and April 2010.

A total of eight patients with esthesioneuroblastoma, five men and three women, were identified. Six patients were treated for primary disease, and two were treated for tumor recurrence. The modified Kadish staging was A in one patient (12.5%), B in two patients (25%), C in four patients (50%), and D in one patient (12.5%). All patients had a complete resection with negative intraoperative margins. One patient had intraoperative hypertension; there were no perioperative complications. With a mean follow-up of over 27 months, all patients are without evidence of disease. In addition, we reviewed the literature and identified several overlapping case series of patients with esthesioneuroblastoma treated via a purely endoscopic technique.

Our series adds to the growing experience of expanded endonasal endoscopic surgery in the treatment of skull base tumors including esthesioneuroblastoma. Longer follow-up on a larger number of patients is required to further demonstrate the utility of endoscopic approaches in the management of this malignancy.

Treatment policy for petroclival meningioma based on tumor size: aiming radical removal in small tumors for obtaining cure without morbidity

Neurosurg Rev (2011) 34:327–335. DOI 10.1007/s10143-011-0308-7

Aggressive tumor removal is not always the best treatment for petroclival meningioma (PCM). However, radical removal actually provides the cure with minimal morbidity.

We evaluated the relation of surgical results and tumor size in the PCM removal to clarify the treatment policy for PCM. This study comprised 32 consecutive patients with newly-diagnosed PCM who underwent tumor removal; tumor size was small (< 3 cm) in 12 patients and large (≥3 cm) in 20. Tumor removal was classified into radical (Simpson’s grade I/II) and non-radical (Simpson’s grade III/IV). Removal of small PCM was 11 radical and one non-radical; no surgical morbidity/mortality occurred and postoperative regular follow-up using magnetic resonance imaging showed no recurrence in the period of 66± 45 months. Removal of large PCM was eight radical and 12 non-radical; despite no mortality, the incidence of permanent cranial nerve deficits and major neurological deficits newly developed postoperatively was 35% and 25%, respectively. Radical removal was significantly more frequent in small PCMs than in large PCMs. Permanent cranial nerve deficits newly developed postoperatively and poor outcome (Karnofsky score ≤80) were significantly more frequent in large PCMs than in small PCMs. Radical removal of small PCM is achieved with minimal morbidity and results in the cure. Notwithstanding high morbidity, aggressive removal of large PCM does not achieve a high rate of radical removal.

To find and remove PCM radically while it is small is the only way to cure the disease with minimal morbidity.

Gamma Knife surgery for parasellar meningiomas: long-term results including complications, predictive factors, and progression-free survival

J Neurosurg 114:1571–1577, 2011. DOI: 10.3171/2011.1.JNS091939

Stereotactic radiosurgery serves as an important primary and adjuvant treatment option for patients with many types of intracranial meningiomas. This is particularly true for patients with parasellar meningiomas. In this study, the authors evaluated the outcomes of Gamma Knife surgery (GKS) used to treat parasellar meningiomas.

Methods. The study is a retrospective review of the outcomes in 138 patients with meningiomas treated at the University of Virginia from 1989 to 2006; all patients had a minimum follow-up of 24 months. There were 31 men and 107 women whose mean age was 54 years (range 19–85 years). Eighty-four patients had previously undergone resection. The mean pre-GKS tumor volume was 7.5 ml (range 0.2–54.8 ml). Clinical and radiographic evaluations were performed, and factors related to favorable outcomes in each case were assessed.

Results. The mean follow-up duration was 84 months (median 75.5 months, range 24–216 months). In 118 patients (86%), the tumor volume was unchanged or had decreased at last follow-up. Kaplan-Meier analysis demonstrated radiographic progression-free survival at 5 and 10 years to be 95.4% and 69%, respectively. Fourteen patients (10%) developed new cranial nerve palsies following GKS. Factors associated with tumor control included younger age, a higher isodose, and smaller tumor volume. A longer follow-up duration was associated with either a decrease or increase in tumor volume. Fourteen patients (10%) experienced new or worsening cranial nerve deficits after treatment. Factors associated with this occurrence were larger pretreatment tumor volume, lower peripheral radiation dose, lower maximum dose, tumor progression, and longer follow-up.

Conclusions. Gamma Knife surgery offers an acceptable rate of tumor control for parasellar meningiomas and accomplishes this with a low incidence of neurological deficits. Radiological control after radiosurgery is more likely in those patients with a smaller tumor volume and a higher prescription dose.

Gamma Knife surgery of meningiomas located in the posterior fossa: factors predictive of outcome and remission

J Neurosurg 114:1399–1409, 2011. DOI: 10.3171/2010.11.JNS101193

Although numerous studies have analyzed the role of stereotactic radiosurgery for intracranial meningiomas, few studies have assessed outcomes of posterior fossa meningiomas after stereotactic radiosurgery. In this study, the authors evaluate the outcomes of posterior fossa meningiomas treated with Gamma Knife surgery (GKS). The authors also assess factors predictive of new postoperative neurological deficits and tumor progression.

Methods. A retrospective review was performed of a prospectively compiled database documenting the outcomes of 152 patients with posterior fossa meningiomas treated at the University of Virginia from 1990 to 2006. All patients had a minimum follow-up of 24 months. There were 30 males and 122 females, with a median age of 58 years (range 12–82 years). Seventy-five patients were treated with radiosurgery initially, and 77 patients were treated with GKS after resection. Patients were assessed clinically and radiographically at routine intervals following GKS. Factors predictive of new neurological deficit following GKS were assessed via univariate and multivariate analysis, and Kaplan-Meier analysis and Cox multivariate regression analysis were used to assess factors predictive of tumor progression.

Results. Patients had meningiomas centered over the tentorium (35 patients, 23%), cerebellopontine angle (43 patients, 28%), petroclival region (28 patients, 18%), petrous region (6 patients, 4%), and clivus (40 patients, 26%). The median follow-up was 7 years (range 2–16 years). The mean preradiosurgical tumor volume was 5.7 cm3 (range 0.3–33 cm3), and mean postradiosurgical tumor volume was 4.9 cm3 (range 0.1–33 cm3). At last follow-up, 55 patients (36%) displayed no change in tumor volume, 78 (51%) displayed a decrease in volume, and 19 (13%) displayed an increase in volume. Kaplan-Meier analysis demonstrated radiographic progression-free survival at 3, 5, and 10 years to be 98%, 96%, and 78%, respectively. In Cox multivariable analysis, pre-GKS covariates associated with tumor progression included age greater than 65 years (hazard ratio [HR] 3.24, 95% CI 1.12–9.37; p = 0.03) and a low dose to the tumor margin (HR 0.76, 95% CI 0.60–0.97; p = 0.03), and post-GKS covariates included shunt-dependent hydrocephalus (HR 25.0, 95% CI 3.72–100.0; p = 0.001). At last clinical follow-up, 139 patients (91%) demonstrated no change or improvement in their neurological condition, and 13 patients showed symptom deterioration (9%). In multivariate analysis, the only factors predictive of new or worsening symptoms were clival or petrous location (OR 4.0, 95% CI 1.1–13.7; p = 0.03).

Conclusions. Gamma Knife surgery offers an acceptable rate of tumor control for posterior fossa meningiomas and accomplishes this with a low incidence of neurological deficits. In patients selected for GKS, tumor progression is associated with age greater than 65 years and decreasing dose to the tumor margin. Clival- or petrous-based locations are predictive of an increased risk of new or worsening neurological deficit following GKS.

Sphenoorbital meningioma: surgical technique and outcome

J Neurosurg 114:1241–1249, 2011.DOI: 10.3171/2010.10.JNS101128

The aim of this study was to describe the surgical technique used for removal of sphenoorbital meningiomas in the authors’ practice and to review the operative outcome.

Methods. Review of the senior author’s practice between 1994 and 2009 revealed 39 patients (mean age 48 years) eligible for this study. Clinical presentation, surgical technique, postoperative outcome, and follow-up data are presented. Surgical technique is detailed, with an emphasis on aggressive removal consisting of drilling of the hypertrophied sphenoid bone, orbital wall, and anterior clinoid process, followed by tumor removal and a wide resection of the involved dura. A simple dural closure without reconstruction of the orbital roof or the lateral wall of the orbit is also described.

Results. Gross-total resection was achieved in 15 cases (38.5%), near-total resection with small residual in the cavernous sinus or periorbita in 20 cases (51.3%), and subtotal resection in 4 cases (10.3%). Postoperative complications included trigeminal hypesthesia in 9 patients, oculomotor palsy in 3 patients, and seizure in 2 patients. Seven patients had recurrence within the mean follow-up period of 40.7 months. Preoperative visual deficits were present in 21 patients (53.8%). Of these, 14 (66.7%) experienced visual recovery to normal levels postoperatively. Statistical analyses revealed preoperative severe visual deficit and sphenoid bone hypertrophy as an independent risk factor and an independent favorable factor, respectively, for a favorable visual outcome. Proptosis was resolved (≤ 2 mm) in 73.5% of the authors’ patients. No patient had postoperative enophthalmos.

Conclusions. In the authors’ practice, surgery for sphenoorbital meningiomas consists of resection of the orbital/ sphenoid intraosseous, intraorbital, and intradural tumor components. The authors believe that aggressive removal of the orbital/sphenoid intraosseous tumor is critical for a favorable visual outcome and tumor control. Furthermore, satisfactory cosmetic results can be achieved with simple reconstruction techniques as described.

Trigeminal schwannomas: experience with 57 cases and a review of the literature

Neurosurg Rev (2011) 34:159–171. DOI 10.1007/s10143-010-0289-y

Trigeminal schwannoma is a mostly benign tumor that can be cured by complete resection. Over the last few decades, several pioneers have developed surgical approaches enabling the total removal of such tumors.

We analyzed 57 patients who underwent radical surgery, including 45 patients who underwent skull base surgery as their initial treatment, for removal of trigeminal schwannomas. Here, we report the surgical management of these cases. Since 1990, all such patients have been treated using three main types of middle fossa skull base approaches, which minimize the exposure of the brain: the anterior transpetrosal approach, subtemporal interdural approach (Dolenc), or a combination of these approaches.

Before 1990, total tumor removal was achieved in only three of eight patients (38%). After 1990, the tumors were totally removed in 43 patients (90%) and were nearly completely removed in an additional three patients (6%). Among the patients who underwent skull base surgery as their initial treatment, a complete resection was achieved in 93% (42/45 patients) of the cases. However, total surgical removal after surgery and Gamma knife surgery was very difficult because of dense adhesions to the brain stem and cranial nerves. No surgery-related mortalities occurred in this series, and the individual KPS scores were more than 90% among the patients who underwent skull base surgery. No recurrences requiring additional surgery have occurred after an average follow-up period of 4.9 years.

Most of the trigeminal schwannomas could be removed totally and safely during a single operation after the introduction of skull base surgery. Therefore, radiosurgery should not be applied as the treatment of first choice for younger patients. A correct anatomical knowledge is critical for minimizing brain exposure and avoiding surgical complications.

Skull base tumor model. Laboratory investigation

J Neurosurg 113:1106–1111, 2010. DOI: 10.3171/2010.3.JNS09513

Resident duty-hours restrictions have now been instituted in many countries worldwide. Shortened training times and increased public scrutiny of surgical competency have led to a move away from the traditional apprenticeship model of training. The development of educational models for brain anatomy is a fascinating innovation allowing neurosurgeons to train without the need to practice on real patients and it may be a solution to achieve competency within a shortened training period. The authors describe the use of Stratathane resin ST-504 polymer (SRSP), which is inserted at different intracranial locations to closely mimic meningiomas and other pathological entities of the skull base, in a cadaveric model, for use in neurosurgical training.

Methods. Silicone-injected and pressurized cadaveric heads were used for studying the SRSP model. The SRSP presents unique intrinsic metamorphic characteristics: liquid at first, it expands and foams when injected into the desired area of the brain, forming a solid tumorlike structure. The authors injected SRSP via different passages that did not influence routes used for the surgical approach for resection of the simulated lesion. For example, SRSP injection routes included endonasal transsphenoidal or transoral approaches if lesions were to be removed through standard skull base approach, or, alternatively, SRSP was injected via a cranial approach if the removal was planned to be via the transsphenoidal or transoral route. The model was set in place in 3 countries (US, Italy, and The Netherlands), and a pool of 13 physicians from 4 different institutions (all surgeons and surgeons in training) participated in evaluating it and provided feedback.

Results. All 13 evaluating physicians had overall positive impressions of the model. The overall score on 9 components evaluated—including comparison between the tumor model and real tumor cases, perioperative requirements, general impression, and applicability—was 88% (100% being the best possible achievable score where the evaluator strongly agreed with the proposed factor). Individual components had scores at or above 80% (except for 1). The only score that was below 80% was related to radiographic visibility of the model for adequate surgical planning (score of 74%). The highest score was given to usefulness in neurosurgical training (98%).

Conclusions. The skull base tumor model is an effective tool to provide more practice in preoperative planning and technical skills.

The endoscopic, endonasal, transmaxillary transpterygoid approach to the pterygopalatine fossa, infratemporal fossa, petrous apex, and the Meckel cave

J Neurosurg 113:967–974, 2010. DOI: 10.3171/2009.10.JNS09157

In this paper the authors’ goal was to present their clinical experience with lesions of the pterygopalatine fossa, infratemporal fossa, lateral sphenoid sinus, cavernous sinus, petrous apex, and Meckel cave using simple and extended endoscopic transpterygoid approaches to the lateral skull base.

Methods. Simple and expanded endoscopic transpterygoid approaches were performed in a series of 13 patients with varying pathology that included lateral sphenoid sinus encephaloceles, benign and malignant sinonasal tumors, and lesions of neural origin.

Results. A gross-total resection was achieved in 5 of 9 patients, while a subtotal resection for tissue diagnosis and cytoreduction prior to further adjuvant treatment was performed in the remaining patients. Sphenoid sinus encephaloceles were successfully repaired via a transpterygoid approach in all 4 patients. The skull base defect was reconstructed using a multilayered closure. One patient developed a postoperative CSF leak, which was successfully treated conservatively. The mean follow-up time was 16 months. Five patients complained of recurrent sinusitis. One patient experienced xerophthalmia and palate numbness. Three patients had died by the time of this report. Two patients died of unrelated causes. The third patient died of progression of an aggressive pterygopalatine osteosarcoma despite undergoing cytoreductive surgery and adjuvant chemotherapy.

Conclusions. An endoscopic transpterygoid approach is a minimally invasive endoscopic approach for lesions located or extending to the pterygopalatine fossa, infratemporal fossa, petrous apex, Meckel cave, and other regions of the paramedian skull base.

Trigeminal schwannomas: experience with 57 cases and a review of the literature

Neurosurg Rev. DOI 10.1007/s10143-010-0289-y

Trigeminal schwannoma is a mostly benign tumor that can be cured by complete resection. Over the last few decades, several pioneers have developed surgical approaches enabling the total removal of such tumors.

We analyzed 57 patients who underwent radical surgery, including 45 patients who underwent skull base surgery as their initial treatment, for removal of trigeminal schwannomas. Here, we report the surgical management of these cases. Since 1990, all such patients have been treated using three main types of middle fossa skull base approaches, which minimize the exposure of the brain: the anterior transpetrosal approach, subtemporal interdural approach (Dolenc), or a combination of these approaches.

Before 1990, total tumor removal was achieved in only three of eight patients (38%). After 1990, the tumors were totally removed in 43 patients (90%) and were nearly completely removed in an additional three patients (6%). Among the patients who underwent skull base surgery as their initial treatment, a complete resection was achieved in 93% (42/45 patients) of the cases. However, total surgical removal after surgery and Gamma knife surgery was very difficult because of dense adhesions to the brain stem and cranial nerves. No surgery-related mortalities occurred in this series, and the individual KPS scores were more than 90% among the patients who underwent skull base surgery. No recurrences requiring additional surgery have occurred after an average follow-up period of 4.9 years.

Most of the trigeminal schwannomas could be removed totally and safely during a single operation after the introduction of skull base surgery. Therefore, radiosurgery should not be applied as the treatment of first choice for younger patients. A correct anatomical knowledge is critical for minimizing brain exposure and avoiding surgical complications.

Trigeminal neurinomas with extracranial extension: analysis of 28 surgically treated cases

J Neurosurg 113:1079–1084, 2010.DOI: 10.3171/2009.10.JNS091149

The object of this paper was to review the authors’ experience with 28 cases of trigeminal neurinomas having an extracranial extension.

Methods. The authors analyzed 28 cases of trigeminal neurinoma in which there was an extracranial extension of the tumor. All patients were treated in their department between the years 1989 and 2009.

Results. There was tumor extension along the ophthalmic division of the nerve in 4 cases, along the maxillary division in 5, and along the mandibular division in 13. In 6 tumors there was diffuse extracranial extension and the exact extracranial division of nerve involvement could not be ascertained. In 10 cases, the tumor had a multicompartmental location—in the posterior fossa, the middle fossa, and the extracranial compartment. Tingling paraesthesiae, numbness, and diffuse pain in the distribution of the trigeminal nerve were common symptoms and were present in 90% of patients. The extracranial component had a well-defined perineural/meningeal membrane cover that was continuous with the middle fossa dura mater and isolated the tumor tissue from the adjoining critical structures. In 7 out of 10 cases, even the posterior fossa component of the tumor was entirely “interdural” (within the confines of the dura). The maximum dimension of the tumor was > 4 cm in 22 cases. A limited “transcranial” approach with (12 cases) or without (16 cases) zygomatic osteotomy was found suitable for resection of these tumors. In 4 cases a lateral orbitotomy was performed. Total tumor resection was performed in 20 cases and partial resection in 8. The duration of follow-up ranged from 6 months to 19 years. Two patients required additional surgery for symptomatic recurrence.

Conclusions. Extracranial extensions of trigeminal neurinomas have a well-defined meningeal covering. In most cases resection was performed via a minimally invasive cranial avenue (a “reverse skull base approach”). Radical resection was associated with an excellent long-term outcome.

Prognostic factors for long-term outcome of patients with surgical resection of skull base chordomas—106 cases review in one institution

Neurosurg Rev (2010) 33:451–456. DOI 10.1007/s10143-010-0273-6

Skull base chordoma are still challenging. Between May 1993 and June 2005, 106 consecutive patients with skull base chordoma underwent surgical removal at Skull Base Division of Neurosurgery, Beijing Tiantan Hospital, China. Retrospective analysis included medical charts and images.

The age of the patients ranged from 7 to 65 years old, with an average age of 35.6 years. Sixty patients were male; the other 46 were female (1.3:1). Follow up data were available in 79 cases ranging from 10 to 158 months (average 63.9 months) after operation.

The prognostic factors for recurrence and survival were analyzed with Kaplan‐Meier, Cox regression and t‐test. Overall, 1, 3, 5 and 10 years survival rates were 87.2%, 79.4%, 67.6% and 59.5% respectively. One, 3, 5 and 10 year recurrent rates were 19.1%, 34.7%, 52.9% and 88.3%, respectively.

The long term outcome of the skull base chordomas is poor. The previous radiotherapy or surgery, dedifferentiated pathology, and less tumor resection are risk factors for longterm survival and recurrence (p<0.05). Although there is no statistic significant role of tumor adherent to vital structure for outcome (p=0.051), it can not exclude its importance for favorable outcome.

Gender, age, tumor size and staging are not independent risk factors for outcome. Surgical technique leading to radical tumor resection with less morbidity is advocatory and beneficial for patients with skull base chordoma with long term outcome, if the tumor could be exposed and resected completely, the recurrence rate was very low for most benign chordomas.

The first 50s: can we achieve acceptable results in vestibular schwannoma surgery from the beginning?

Acta Neurochir (2010) 152:1359–1365. DOI 10.1007/s00701-010-0672-z

Vestibular schwannoma surgery requires a profound knowledge of anatomy and long-standing experience of surgical skull base techniques, as patients nowadays requests high-quality results from any surgeon. This educes a dilemma for the young neurosurgeon as she/he is at the beginning of a learning curve. The presented series should prove if surgical results of young skull base surgeons are comparable respecting carefully planned educational steps.

Methods: The first 50 vestibular schwannomas of the first author were retrospectively evaluated concerning morbidity and mortality with an emphasis on functional cranial nerve preservation. The results were embedded in a timeline of educational steps starting with the internship in 1999.

Results: Fifty vestibular schwannomas were consecutively operated from July 2007 to January 2010. According to the Hannover Classification, 14% were rated as T1, 18% as T2, 46% as T3, and 21% as T4. The overall facial nerve preservation rate was 96%. Seventy-nine percent of patients with T1–T3 tumours had no facial palsy at all and 15% had an excellent recovery of an initial palsy grade 3 according to the House & Brackman scale within the first 3 months after surgery. Hearing preservation in T1/2 schwannomas was achieved in 66%, in patients with T3 tumours in 56%, and in large T4 tumours in 25%. Three patients suffered a cerebrospinal fluid fistula (6%), and one patient died during the perioperative period due to cardiopulmonary problems (2%).

Conclusions: The results demonstrate that with careful established educational plans in skull base surgery, excellent clinical and functional results can be achieved even by young neurosurgeons.

The role of platybasia in trigeminal neuralgia

REV NEUROL 2009; 49: 630-2

Introduction. The vascular compression of a nerve is known to be the most important cause of idiopathic trigeminal neuralgia (ITN) by most authors. However, several cases of skull base deformities in patients with ITN have been reported in our bibliographic review. Aim. To compare the existence of platybasia between a group of patients with ITN and a randomized control group.

Subjects and methods. Twenty-five patients with ITN and twenty-five control subjects were X-rayed and their basal angles were measured and compared.

Results. The basal angle measured to investigate the existence of platybasia is significantly greater in the ITN group than in the control group. Platybasia was found in 10 patients with ITN. However, only two cases of platybasia were reported in the control group. Therefore in our study we have found that the incidence of platybasia in patients with ITN is significantly increased.

Conclusion. Our data demonstrated that platybasia has an important role in the pathogenesis of the ITN by altering the normal anatomy of the nerves and vessels within the posterior fossa

ABC Surgical Risk Scale for skull base meningioma

J Neurosurg 111:1053–1061, 2009.DOI: 10.3171/2007.11.17446

Surgery for skull base meningiomas (SBMs) can lead to complications because these lesions are difficult to approach and can involve cranial nerves and arteries. The authors propose a scoring system to evaluate the relative risks and benefits of surgical treatment of SBMs.

Methods. The authors used a 2-step process to construct their scale. First, they derived significant predictive variables from retrospective data on 132 SBM cases treated surgically (primary surgeries only) between May 2000 and December 2005. Next, they validated the predictive accuracy of their scoring system in 60 consecutive cases treated surgically between January 1995 and April 2000, including both primary and repeated surgeries. Finally, they investigated the effect of the surgery on the patients’ preoperative symptoms for consecutive cases treated surgically between January 1995 and December 2005, including both primary surgeries and retreatments.

Results. Five items that predicted surgical risk were identified: 1) tumor attachment size; 2) arterial involvement; 3) brainstem contact; 4) central cavity location; and 5) cranial nerve group involvement. The authors named their scoring system the ABC Surgical Risk Scale, after the initial letters of these items. Each factor was assigned a score of 0–2 points, and an additional point was added for previous surgical treatment or for radiation, giving a possible total score of 12 points. On average, the scoring system allocated 2 points for gross-total resections, 6.1 points for near-total resections, and 9 points for subtotal resections, with significant differences between groups. For cases scoring ≥ 8 points, the percentage of cases showing neurological deterioration postoperatively exceeded the percentage showing improvement.

Conclusions. The authors conclude that this scoring system can be used to predict the extent of tumor removal and that the scores reflect the surgical risk.