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Emad Aboud, Ossama Al-Mefty and M. Gazi Yaşargil

Object. Laboratory training models are essential for developing and refining surgical skills, especially for microsurgery. The closer to live surgery the model is, the greater the benefit. In this paper the authors introduce a cadaver model with unique characteristics: dynamic filling of the cerebral vasculature with colored liquid and clear fluid filling of the arachnoid cisterns. This model is distinctive and has great practical value for training in a wide range of surgical procedures.

Methods. Cadaveric heads were prepared for surgical procedures in the following manner: the carotid arteries (CAs) and vertebral arteries (VAs) in the neck were cannulated, as were the internal jugular veins (JVs) on both sides. Two tubes were introduced into the spinal canal and each one was advanced into one of the cerebellopontine angle cisterns. A CA, VA, or both were then connected to a reservoir containing light red fluid and a pressure of 80 to 120 mm Hg and a pulse rate of 60 beats/minute were established using a pump. The JV on the side currently being dissected was connected to a reservoir containing dark red fluid and kept at a pressure between 20 and 40 mm Hg. The remaining vessels were clamped in the neck. The cisternal tubes were connected to a reservoir of clear fluid that was regulated by an adjustable flow. Nine trainees have tested this model on eight specimens by practicing a variety of surgical procedures and maneuvers, including craniotomies; hemostasis; cisternal and vascular dissection; vascular anastomosis and repair; establishment of arterial bypasses; aneurysm creation, dissection, and clipping; management of an aneurysm rupture; intraparenchymal resection such as amygdalohippocampectomy; ventricular endoscopy and third ventriculostomy; cavernous sinus and skull base approaches; and resection of artificial tumors in the basal cisterns.

Conclusions. This model mimics the normal human anatomy and dynamic vascular filling found in real surgery and presents it from the training perspective, allowing a wide range of skill development and repeated practice. It provides an alternative model to laboratory animals. It is inexpensive and readily available, and has great value for the acquisition and refinement of surgical skills that are not only specific to neurosurgery, but are applicable to other surgical disciplines.

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Kenan I. Arnautović and Ossama Al-Mefty

Object. Primary jugular fossa meningomas (JFMs) are one of the rarest subgroups of meningioma, with fewer than 40 cases reported in the literature. The authors retrospectively analyzed the results of surgical treatment in their series of patients, including clinical, pathological, and complication features. The surgical approach was mandated by the pathological anatomy of the tumor as well as by the anatomy of the individual patient.

Methods. During a 6.5-year period, the authors performed nine surgeries in eight patients (seven women [88%] and one man [12%]) with JFMs. Six lesions occurred on the right side and two on the left. The most common presenting symptoms were altered hearing in five patients (62%), swallowing difficulties in four patients (50%), and a deficit of the 11th cranial nerve in three patients (38%); a combination of two or more signs or symptoms was common. The surgical approach was tailored to the local anatomy (tumor—neurovascular relationships) found in each patient; three different routes were used. Radical tumor removal was achieved in all patients; one tumor recurrence occurred after 20 months in a patient in whom the tumor had displayed atypical histological features. This woman underwent a second operation. The mean length of hospital stay was 1 week. The mean and the median follow-up period were 45 and 40 months, respectively. The most common complications were transient lower cranial nerve deficits, which resolved or were compensated for in all patients within 1 month.

Conclusions. With a careful, extensive preoperative evaluation and appropriate tailoring of the operative approach, JFMs can be radically resected with the expectation of good outcome.

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Ossama Al-Mefty, Samer Ayoubi and Esam Gaber

Object. As in patients with vestibular schwannomas, advances in surgical procedures have markedly improved outcomes in patients with trigeminal schwannomas. In this article the authors address the function of cranial nerves in a series of patients with trigeminal schwannomas that were treated with gross-total surgical removal. The authors emphasize a technique they use to remove a dumbbell-shaped tumor through the expanded Meckel cave, and discuss the advantage of the extradural zygomatic middle fossa approach for total removal of tumor and preservation or improvement of cranial nerve function.

Methods. Within an 11-year period (1989–2000), 25 patients (14 female and 11 male patients with a mean age of 44.4 years) with benign trigeminal schwannomas were surgically treated by the senior author (O.A.) with the aim of total removal of the tumor. Three patients had undergone previous surgery elsewhere. Trigeminal nerve dysfunction was present in all but two patients. Abducent nerve paresis was present in 40%. The approach in each patient was selected according to the location and size of the lesion. Nineteen tumors were dumbbell shaped and extended into both middle and posterior fossae. All 25 tumors involved the cavernous sinus. The zygomatic middle fossa approach was particularly useful and was used in 14 patients. The mean follow-up period was 33.12 months. In patients who had not undergone previous surgery, the preoperative trigeminal sensory deficit improved in 44%, facial pain decreased in 73%, and trigeminal motor deficit improved in 80%. Among patients with preoperative abducent nerve paresis, recovery was attained in 63%. Three patients (12%) experienced a persistent new or worse cranial nerve function postoperatively. Fifth nerve sensory deficit persisted in one of these patients, sensory and motor dysfunction in another, and motor trigeminal weakness in the third patient. In all patients a good surgical outcome was achieved. One patient died 2 years after treatment from an unrelated cause. In three patients the tumors recurred after an average of 22.3 months.

Conclusions. Preservation or improvement of cranial nerve function can be achieved through total removal of a trigeminal schwannoma, and skull base approaches are better suited to achieving this goal. The zygomatic middle fossa approach is particularly helpful and safe. It allows extradural tumor removal from the cavernous sinus, the infratemporal fossa, and the posterior fossa through the expanded Meckel cave.

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Benedicto O. Colli and Ossama Al-Mefty

Object. Chordomas are rare tumors that arise from the remnants of the notochord. Because of their deep location, local infiltrative nature, and involvement of surrounding bone, treatment of chordomas is a challenge. In this study the authors analyze the data and prognostic factors obtained during the follow-up period (range 1–150 months, median 38 months) in 53 patients with craniocervical junction chordomas and 10 patients with chondrosarcomas.

Methods. Several surgical approaches were used, and some tumor excisions required staged procedures. Survival was calculated according the Kaplan—Meier method. Statistical analysis was performed using Fisher exact, log rank and Kruskal—Wallis tests. Radical/subtotal resection was achieved in 77.8% of the patients. The mortality rate during the follow-up period was 14.3%. In patients harboring chondrosarcoma better 5-year recurrence-free survival (RFS) rates were demonstrated than in those with chordoma (100% and 50.7%, respectively). Histological patterns (typical or chondroid chordomas) and patient age at onset of symptoms had no effect on the RFS rates. Radical/subtotal resections were associated with better RFS rates than partial resection. Adjuvant proton-beam therapy was shown to increase the RFS rates compared with conventional radiotherapy (90.9% and 19.4%, respectively, at 4 years posttreatment). Karyotypically abnormal tumors were associated with the worst RFS rates compared with karyotypically normal lesions (44.5% and 90.3%, respectively, at 3 years). Cases of cranial nerve palsy followed by those with cerebrospinal fluid leakages were the most frequent postoperative complications. Permanent postoperative neurological deficit was observed in 28.6% of the patients.

Conclusions. A better prognosis was observed in patients with chondrosarcoma compared with those harboring chordoma. Histological pattern and patient age at symptom onset were not factors that influenced prognosis in cases of chordoma. Extensive resection and possibly adjuvant proton-beam therapy provided better prognoses for these patients.

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Kenan I. Arnautović and Ossama Al-Mefty

Object. Chordomas have a high propensity for local recurrence and progression, as well as for systemic and cerebrospinal fluid metastasis. The authors identified and analyzed a series of patients with chordomas, focusing on an underrecognized pathological entity—surgical seeding of tumor cells.

Methods. In a retrospective analysis of 82 patients with chordomas treated over a 10-year period (1990–2000), the authors found six patients (7.3%) in whom surgical seeding had occurred. In five (83%) of these patients the primary tumor was located at the clivus. In one (17%), the tumor was present at the cervical spine. There were two male (33%) and four female patients (67%) whose mean age was 34 years. The seeding sites, which were separate from the primary tumor, were located along the operative route or in the abdomen where fat was removed. The seeding was diagnosed 5 to 15 months (mean 12 months) after surgery. One seeding site was present in five patients, and 17 seeding sites were present in one patient. The involved tissues included mucosa, bone, dura, muscle, and fat. After resection, all seedings were confirmed histologically.

Conclusions. Seeding of chordomas occurs along the operative route and at distant locations where tissue is harvested. Early diagnosis and aggressive surgery are recommended. Based on the results of this study, the authors suggest that surgical techniques, postoperative radiotherapy, neuroradiological follow-up protocol, and even research on chordomas should be reevaluated.

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Benedicto O. Colli and Ossama Al-Mefty

Object

Chordomas are rare tumors that arise from the remnants of the notochord. Because of their deep location, local infiltrative nature, and involvement of surrounding bone, treatment of chordoma is a challenge. The authors analyze the data and prognostic factors obtained during the follow-up period (range 1–150 months, median 38 months) in 53 patients with craniocervical junction chordoma and 10 patients with chondrosarcoma.

Methods

Several surgical approaches were used, and some tumor excisions required staged procedures. Survival was calculated according the Kaplan–Meier method. Statistical analysis was performed using the Fisher exact and Kruskal–Wallis tests. Radical/subtotal resection was achieved in 77.8% of the patients. The mortality rate during the follow-up period was 14.3%. In patients harboring chondrosarcoma better 5-year recurrence-free survival (RFS) rates were demonstrated than in those with chordoma (100% and 50.7%, respectively). Histological patterns (typical or chondroid chordoma) and patient age at onset of symptoms had no effect on the RFS rates. Radical/subtotal resections were associated with better RFS rates than partial resection. Adjuvant proton-beam radiotherapy was shown to increase the RFS rates compared with conventional radiotherapy (90.9% and 19.4%, respectively at 4 years posttreatment). Karyotypically abnormal tumors were associated with worst RFS rates as compared with karyotypically normal lesions (44.5% and 90.3%, respectively at 3 years). Cases of cranial nerve palsy, followed by CSF leakages were the most frequent postoperative complication. Permanent postoperative neurological deficit was observed in 28.6% of the patients.

Conclusions

A better prognosis was observed in patients with chondrosarcoma compared with those harboring chordoma. Histological pattern and the patient's age at symptom onset were not factors that influenced prognosis in cases of chordoma. Extensive resection and possibly adjuvant proton-beam radiotherapy provided better prognosis for these patients.

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Jeffrey R. Sawyer, Muhammad Husain and Ossama Al-Mefty

Object

The authors conducted a study of 22 skull base chordomas.

Methods

A series of 22 skull base chordomas was analyzed with G banding. Subsequently, metaphase cells obtained from three tumors were reexamined using multicolor spectral karyotyping. Clonal chromosome aberrations were identified in 11 cases, all of which were recurrent tumors. Three tumors showed a remarkable similarity in cytogenetic features, and these features appear to characterize a recurring combination of nonrandom chromosome aberrations, including isochromosome 1q, gain of chromosome 7, and monosomy for chromosomes 3, 4, 10,13, and 18. Isochromosome 1q was identified as the sole recurring structural chromosome rearrangement in these tumors. The pattern of chromosome loss reported in the progression of lumbosacral chordoma also appears to be true of skull base chordomas with the additional findings of isochromosome 1q, gain of chromosome 7, and loss of chromosome 18.

Conclusions

Skull base chordomas characterized by isochromosome 1q and monosomy 13 provide support for the concept of the loss of putative tumor suppressor loci on 1p and 13q and aggressive tumor behavior.

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Ossama Al-Mefty

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Kenan I. Arnautoviæ and Ossama Al-Mefty

Object

Chordomas have a high propensity for local recurrence and progression, as well as for systemic and cerebrospinal fluid metastasis. The authors identified and analyzed a series of patients with chordomas, focusing on an underrecognized entity—surgical seeding.

Methods

In a retrospective analysis of 82 patients with chordomas treated over a 10-year period (1990–2000) the authors found six patients (7.3%) in whom surgical seeding had occurred. In five (83%) of these patients the primary tumor was located at the clivus. In one (17%) the tumor was present in the cervical region. There were two male (33%) and four female patients (67%) with a mean age of 34 years. The seeding sites, which were separate from the primary tumor, were located along the operative route or in the abdomen where fat was removed. The seeding was diagnosed 5 to 15 months after surgery (mean 12 months). One seeding site was present in five patients and 17 seeding sites were present in one patient. The involved tissues included mucosa, bone, dura, muscle, and fat. After resection, all seedings were documented histologically.

Conclusions

Seeding of chordomas occurs along the operative route and at distant locations where fat is harvested. Early diagnosis and aggressive surgical treatment are recommended. Based on the results of this study, it is suggested that surgical techniques, postoperative irradiation, the neuroradiological follow-up protocol, and even research on chordomas should be reevaluated.

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Uğur Türe, M. Gazi Yaşargil, Ossama Al-Mefty and Dianne C. H. Yaşargil

Object. The insula is located at the base of the sylvian fissure and is a potential site for pathological processes such as tumors and vascular malformations. Knowledge of insular anatomy and vascularization is essential to perform accurate microsurgical procedures in this region.

Methods. Arterial vascularization of the insula was studied in 20 human cadaver brains (40 hemispheres). The cerebral arteries were perfused with red latex to enhance their visibility, and they were dissected with the aid of an operating microscope.

Arteries supplying the insula numbered an average of 96 (range 77–112). Their mean diameter measured 0.23 mm (range 0.1–0.8 mm), and the origin of each artery could be traced to the middle cerebral artery (MCA), predominantly the M2 segment. In 22 hemispheres (55%), one to six insular arteries arose from the M1 segment of the MCA and supplied the region of the limen insulae. In an additional 10 hemispheres (25%), one or two insular arteries arose from the M3 segment of the MCA and supplied the region of either the superior or inferior periinsular sulcus. The insular arteries primarily supply the insular cortex, extreme capsule, and, occasionally, the claustrum and external capsule, but not the putamen, globus pallidus, or internal capsule, which are vascularized by the lateral lenticulostriate arteries (LLAs). However, an average of 9.9 (range four–14) insular arteries in each hemisphere, mostly in the posterior insular region, were similar to perforating arteries and some of these supplied the corona radiata. Larger, more prominent insular arteries (insuloopercular arteries) were also observed (an average of 3.5 per hemisphere, range one–seven). These coursed across the surface of the insula and then looped laterally, extending branches to the medial surfaces of the opercula.

Conclusions. Complete comprehension of the intricate vascularization patterns associated with the insula, as well as proficiency in insular anatomy, are prerequisites to accomplishing appropriate surgical planning and, ultimately, to completing successful exploration and removal of pathological lesions in this region.