Donald C. Wright

Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients.

The management of chordomas and chondrosarcomas involving the cranial base remains controversial. The options for therapy include biopsy, partial resection, radical resection, and various forms of radiotherapy. In this article, we analyze the outcome of 60 patients with cranial base chordoma or chondrosarcoma treated with extensive surgical resection between 1984 and 1993. Forty-six patients had chordomas, and 14 had low-grade chondrosarcomas; 50% of these patients had been treated previously. Preoperative studies included computed tomography, magnetic resonance imaging, cerebral angiography, and balloon occlusion test of the internal carotid artery, as indicated. Magnetic resonance imaging was performed on all patients during follow-up. The surgical approaches used for tumor resection were predominantly the following: subtemporal, transzygomatic, transcavernous, and transpetrous apex; subtemporal and infratemporal; extended frontal; and extreme lateral transcondylar. Staged operations with a combination of approaches were used when necessary (52% of cases) to remove a tumor more completely. Statistical analysis was done by the chi 2 test and correlation matrix. Sixty-seven percent of the patients had total or near-total resection. Twenty percent of the patients received postoperative radiotherapy. Eleven patients died during the postoperative follow-up period, nine with chordomas and two with chondrosarcomas. Three patients died because of systemic complications within 3 months after surgery, five died because of tumor recurrence, one died from unrelated causes, and two died from late complications of radiotherapy. The recurrence-free survival rate for all tumors was 80% at 3 years and 76% at 5 years. Chondrosarcomas had a better prognosis than chordomas (recurrence-free survival rates, 90% at 5 years and 65% at 5 years, respectively; P = 0.09). Patients who had undergone previous surgery had a greater risk of recurrence (5-year recurrence-free survival rate, 64%) than did patients who had not undergone previous surgery (5-year recurrence-free survival rate, 93%; P < 0.05). Patients with total or near-total resection had a better 5-year recurrence-free survival rate (84%) than did patients with partial or subtotal resection (64%) (P < 0.05). Postoperative leakage of cerebrospinal fluid was the most frequent complication (30% of patients) and was found to increase the risk of permanent disability. Patients who had undergone previous radiotherapy had a greater risk of death in the postoperative period (within 3 months of their operations) and during follow-up. However, total or near-total resection did not increase the rate of postoperative disability.(ABSTRACT TRUNCATED AT 400 WORDS)

Acoustic Neuromas: Results of Current Surgical Management

OBJECTIVE In this article, we review the surgical outcomes of 179 patients with acoustic neuromas. METHODS Most of the tumors (84%) were operated on using a retrosigmoid, transmeatal approach. A transpetrosal, retrosigmoid approach was used in 10% of the patients, most of whom had large tumors. The translabyrinthine (4%) and transmastoid, transpetrosal, partial labyrinthectomy approaches (2%) were used selectively. The operative approaches are discussed. Tumors were categorized according to their cerebellopontine angle dimensions as small (< 2 cm), medium (2.0-3.9 cm), and large (> or = 4 cm). RESULTS House-Brackmann evaluation of postoperative facial nerve function revealed excellent results (Grade I or II) in 96% of small tumors, 74% of medium tumors, and 38% of large tumors. A fair postoperative function (Grade III or IV) was achieved in 4% of small tumors, 26% of medium tumors, and 58% of large tumors. Functional hearing preservation, defined as Gardner-Robertson Class I or II, was achieved in 48% of small tumors and 25% of medium tumors. Hearing was not preserved in any of the three patients with large tumors in whom hearing preservation was attempted. Treatment complications consisted mainly of cerebrospinal fluid leakage (15% of the patients). The majority of the patients who experienced cerebrospinal fluid leakage were treated successfully with lumbar spinal drainage; only four patients (2% of the total group) required subsequent surgery for correction of cerebrospinal fluid leakage. There were two deaths (1%) in this series. One death occurred as the result of myocardial infarction and the other as the result of severe obstructive lung disease. One patient sustained disability because of cerebellar and brain stem injury. Complete tumor resection was accomplished in 99% of the patients, and there was no evidence of recurrence in this group. Only 1 of the 179 patients underwent incomplete tumor resection; he required subsequent surgery for symptomatic tumor regrowth. Our patient follow-up had a mean duration of 70 months and a median of 65 months (range, 3-171 mo). CONCLUSION Our results are similar to those of other large microsurgical series of acoustic neuromas. Unless a patient has major medical problems, microsurgery by an experienced team of surgeons is preferred over radiosurgery.

Long-term Follow-up of Patients with Meningiomas Involving the Cavernous Sinus: Recurrence, Progression, and Quality of Life

OBJECTIVE In this report, we evaluate the recurrence- and progression-free survival of patients with meningiomas involving the cavernous sinus. METHODS The rates of tumor recurrence and progression in 119 patients with meningiomas involving the cavernous sinus treated between 1983 and 1993 were analyzed. RESULTS The mean follow-up period was 33.8 months. There were seven recurrences of completely resected tumors (7 of 73, 10%) and seven progressions of incompletely resected tumors (7 of 46 15%). Life table analysis of recurrence- and progression-free survival was performed. The recurrence-free survival rate was 94% at 3 years and 81% at 5 years. In contrast, the progression-free survival rate was 87% at 3 years and 62% at 5 years (P = 0.0456). There were two patterns of recurrence or progression. The first group of tumors had an aggressive biological behavior with growth at multiple areas of resection; the second group showed growth at or near the margins of resection. Functional status continued to improve slowly during the follow-up period; however, there was no statistically significant difference between the Karnofsky scores obtained 3 to 12 months postoperatively and the follow-up Karnofsky scores (obtained > 1 yr postoperatively). CONCLUSION Surgery for meningiomas involving the cavernous sinus seems to provide excellent tumor control. Our experience suggests that for the majority of these tumors, complete tumor resection can be performed with acceptable risks of morbidity and mortality and seems to increase the duration of recurrence-free survival. The long-term quality of life of surviving patients is satisfactory, with the great majority of the patients being independent.

Patient Outcome at Long-term Follow-up after Aggressive Microsurgical Resection of Cranial Base Chondrosarcomas

OBJECTIVE:To evaluate patient clinical outcome and survival at long-term follow-up after aggressive microsurgical resection of chondrosarcomas of the cranial base. METHODS:Over a 20-year period, 47 patients underwent 72 operative procedures for resection of cranial base chondrosarcomas. Thirty-three patients were previously untreated, whereas 14 patients previously had undergone surgery or radiation. Twenty-three patients had a single operation and 24 underwent staged (more than one) operations because of extensive disease. Patients who underwent subtotal resection also underwent radiotherapy or radiosurgery. Patients were evaluated at follow-up clinically and by imaging studies. RESULTS:Gross total resection was accomplished in 29 (61.7%) patients, and subtotal resection was accomplished in 18 patients (38.3%). The resection was better in patients who underwent a primary operation (gross total resection, 68.8 versus 46.7%) rather than a reoperation. Patients who underwent incomplete resection underwent postoperative radiotherapy, which included proton beam radiotherapy (15.6%), radiosurgery (68%), and fractionated radiation (15.6%). There were no operative deaths. Postoperative complications (cerebrospinal fluid leakage, quadriparesis, infections, cranial nerve palsies, etc.) were observed in 10 patients (18%). The follow-up ranged from 2 to 255 months, with an average of 86 months. At the conclusion of study, 36 (76.6%) patients were alive, and 21 (44.7%) patients were alive without disease. Recurrence-free survival was 32% at 10 years in all patients, 42.3% in primary patients and 13.8% in those who underwent reoperation. The Karnofsky performance score was 82.4 ± 9.8 before surgery, 85 ± 12.5 at 1 year after surgery, and 85.3 ± 5.8 at the latest follow-up. Two patients died as a result of radiotherapy complications (malignancy, radiation necrosis). CONCLUSION:Cranial base chondrosarcomas can be managed well by complete surgical resection or by a combination of surgery and radiotherapy. The study cannot comment about the efficacy of radiotherapy. Approximately half of the patients survived without recurrence at long-term follow-up (>132 mo). The functional status of the surviving patients was excellent at follow-up.

Posterior fossa meningiomas: surgical experience in 161 cases.

BACKGROUND We report the clinical, radiological, and surgical findings of patients with posterior fossa meningiomas surgically treated at our institution over the last 6 years. METHODS We reviewed 161 consecutive cases of posterior fossa meningiomas operated on between April 1993 and April 1999 at The George Washington University Medical Center. RESULTS There were 128 female and 33 male patients (mean age 47 years, range of 10-81 years). Meningiomas were classified as petroclival (110 cases), foramen magnum (21 cases), cerebellar hemispheric, lateral tentorial (14 cases), cerebellopontine angle (9 cases), and jugular foramen (7 cases). Mean tumor equivalent diameter (TED) = (D1xD2xDE)(1/3) was 3.1 cm (range of 0.53-8.95). Head pain (50% of cases) and disturbance of gait (44%) were the most common presenting symptoms, and cranial neuropathies the most common neurological signs on admission. Mean preoperative performance status (Karnofsky scale) was 80.2 (range 40-100). Surgical approaches to these tumors included partial labyrinthectomy petrous apicectomy, fronto-temporal/fronto-temporal orbitozygomatic osteotomy, retrosigmoidal, extreme lateral, transpetrosal, and combined. In 38 cases a staged procedure was performed. Gross-total resection was achieved in 57% of patients, and subtotal/partial in 43%. Surgical mortality was 2.5% and complications were encountered in 41% of patients. Postoperative CSF leak occurred in 22 cases (13.6%). The mean follow-up was 19 months, ranging from 0.2 to 63.6, and the mean performance status of patients with a follow-up of at least 12 months was 77 (range of 40-100). Recurrence or progression of disease was found in 13.7% of cases (follow-up 2 years or more). CONCLUSION Our experience suggests that although posterior fossa meningiomas represent a continuing challenge for contemporary neurosurgeons, such tumors may be completely or subtotally removed with low rate of mortality and acceptable morbidity, allowing most of these patients to achieve a good outcome in a long-term follow-up.

Cranial chordomas: clinical presentation and results of operative and radiation therapy in twenty-six patients

The clinical presentation and the results of operative and radiation therapy in a series of 26 patients with cranial chordomas seen at the University of California, San Francisco, between 1940 and 1984 are reviewed. There were 14 men and 12 women, with a mean age of 39.6 years. Six patients had chondroid chordomas. The most common presenting symptoms were headache and diplopia, and the most common presenting sign was extraocular palsy. Fifty-three operations directed at removal of the tumor were performed. Twenty-three patients received various forms of radiation therapy postoperatively, including conventional external beam therapy, heavy charged particles, and interstitial implants. The average length of follow-up is 5.6 years. Eleven of 26 patients have died; the mean duration of survival in this group, excluding 1 perioperative death, was 4 years and 2 months. Although the average survival time of 6 years and 4 months was the same in patients with typical chordomas (excluding the perioperative death and 1 patient lost to follow-up) and in those with the chondroid variant, all of the latter are still alive, whereas more than half of the patients with typical chordoma have died.

The results of surgery for benign tumors of the cavernous sinus.

CAVERNOUS SINUS SURGERY has been performed increasingly in the last 2 decades because of new knowledge and technologies. With increasing international expertise in cavernous sinus surgery, the results must be analyzed critically to search for accurate prognosticators of outcome. We performed a retrospective review of 124 patients (40 male, 84 female; mean age, 45 years) who underwent cavernous sinus surgery for benign tumors from 1983 to 1992. Sixty-five percent had tumors encasing the internal carotid artery. Mean follow-up was 29 months (median, 26 mo). Gross total or near-total resection was possible in 80%. Patients with neurilemomas, angiofibromas, epidermoids, chondroblastomas, and hemangiomas were more likely to have total or near-total resection (100% versus 75%, P < 0.025). Disabling complications (five cerebral infarctions, two meningitis, and one hydrocephalus with chiasmal prolapse) occurred only in patients with meningiomas or pituitary adenomas. On follow-up, excellent/good binocular vision was achieved in 53% of patients entering surgery with excellent/good function versus 25% who entered surgery with fair/poor binocular vision (P < 0.025). Ninety-three percent of patients had a Karnofsky score > or = 70 on follow-up. There were a total of 12 recurrences (10%), 6 in patients with meningiomas, 2 in patients with angiofibromas, 2 in patients with craniopharyngiomas, 1 in a patient with a pituitary adenoma, and 1 in a patient with an osteoblastoma. Patients with tumor growth or neurological symptoms indicative of progressive cavernous sinus involvement should undergo cavernous sinus exploration. This surgery has acceptable morbidity and mortality and, if the tumor can be removed easily, the surgeon should try to perform radical tumor resection. To avoid major complications, the surgeon must exercise utmost care to preserve the neurovascular structures of the cavernous sinus, with special attention to tumors that extend into the petroclival region. Better results from surgery can be expected in those patients with neurilemomas, hemangiomas, or epidermoids than in patients with meningiomas, craniopharyngiomas, or pituitary adenomas. Good functional outcome can be expected, particularly if the patients preoperative clinical status is good. Particular attention must be paid to the reconstruction of anatomic barriers in order to prevent cerebrospinal fluid leakage and subsequent meningitis.

Partial labyrinthectomy petrous apicectomy approach to neoplastic and vascular lesions of the petroclival area

OBJECTIVE: To study the value of an improvement of the presigmoid petrosal approach to the petroclival area by the addition of partial labyrinthectomy and petrous apicectomy and to document hearing and other results. METHODS: Thirty-six consecutive patients treated by this technique during a 2-year period were studied prospectively. The lesions treated included 33 petroclival neoplasms (25 meningiomas, 5 chordomas, 1 chondrosarcoma, 1 trigeminal schwannoma, and 1 epidermoid cyst) and 3 vertebrobasilar aneurysms. The patients underwent clinical, radiological, and neuro-otological examinations. RESULTS: There was no perioperative mortality. Cranial nerve deficits involving Cranial Nerves III, IV, V, and VI occurred in 17 patients (47%) postoperatively. Cerebrospinal fluid leak occurred in 12 patients (33%). Four of these patients were treated by lumbar drainage, two patients were treated by lumboperitoneal shunt, and six patients required reoperation and repacking of the middle ear. Hydrocephalus occurred in five patients (13.9%). There was one case of meningitis and another of systemic sepsis. All 36 patients underwent postoperative audiometric evaluation. When serviceable hearing was present preoperatively (Gardner-Robertson Grades I or II), it was determined to be preserved at postoperative follow-up in 81% of the patients (26 of 32 patients). CONCLUSION: The partial labyrinthectomy petrous apicectomy approach provided improved access to neoplasms of the clivus and petrous apex and the posterior cavernous sinus area and to vertebrobasilar aneurysms in the midclival area. This improvement in access permits more controlled and thorough treatment of these lesions, with reduced brain retraction and acceptable morbidity with respect to auditory function.

Venous and Arterial Bypass Grafts for Difficult Tumors, Aneurysms, and Occlusive Vascular Lesions: Evolution of Surgical Treatment and Improved Graft Results

OBJECTIVE: In the treatment of patients with cranial base tumors, unclippable aneurysms, or medically intractable ischemia, it may be necessary to use high-flow bypass grafts. The indications, surgical techniques and complications are discussed. METHODS: During a 10-year period, 99 saphenous vein grafts and 3 radial artery grafts were performed for 101 patients, i.e., 72 with neoplasms, 23 with aneurysms, and 6 with ischemia. Clinical follow-up monitoring of the patients was by direct examination or telephone interview, with a mean follow-up period of 41.2 months (range, 5-147 mo). Radiological follow-up monitoring was by magnetic resonance imaging, magnetic resonance angiography, or three-dimensional computed tomographic angiography, with a mean follow-up period of 32 months (range, 1-120 mo). During the follow-up period, there was one late graft occlusion and one graft stenosis. RESULTS: The use of intraoperative angiography improved the patency rate from 90 to 98% and reduced the incidence of perioperative stroke from 13 to 9.5%. Ninety-two percent of the patients were in excellent or good neurological condition at the time of discharge from the hospital, compared with 95% before surgery. The perioperative mortality rate was 2%. Other complications included three intracranial hematomas, rupture of a vein graft in a patient with Marfans syndrome, and five tumor resection-related problems. The long-term survival rates for patients who received grafts were excellent for patients with benign tumors, fair to poor for patients with malignant tumors, good for patients with aneurysms, and excellent for patients with ischemia. CONCLUSION: The results of saphenous vein and radial artery grafting have been greatly improved by the use of intraoperative angiography, improvements in surgical techniques, and improved perioperative treatment.

Pharmacology and phase I/II study of continuous intravenous infusions of iododeoxyuridine and hyperfractionated radiotherapy in patients with glioblastoma multiforme.

Forty-seven adult patients with glioblastoma multiforme (GBM) were treated in a phase I/II study combining continuous intravenous (IV) infusions of iododeoxyuridine (IdUrd) and hyperfractionated radiation therapy. IdUrd was administered as a continuous infusion (24 h/d) for two separate 14-day infusion periods. The dose of IdUrd was escalated from 500 to 1,200 mg/m2/d. The initial wide-field tumor volume was treated to 45 Gy at 1.5 Gy fractions twice daily over 3 weeks. Following a planned 2-week break, a reduced-field boost of 25 Gy was delivered using 1.25 Gy fractions twice daily over 2 weeks (total dose, 70 Gy over 9 weeks). The IdUrd infusion preceded both the wide-field and reduced-field irradiation by 1 week. All treatment was performed on an outpatient basis. Dose-limiting systemic toxicity to the bone marrow (primarily thrombocytopenia) and gastrointestinal (GI) tract (both stomatitis and diarrhea) established the maximum tolerable dose (MTD) at 1,000 mg/m2/d for a 14-day infusion. Significant local toxicity (within the radiation field) was not seen. The kinetics of IdUrd were linear with dose escalation and reached steady-state plasma concentrations of 1.3 to 3.4 mumol/L. The total body clearance of IdUrd was .82 L/min/m2. The primary metabolite, 5-iodouracil (IUra) approached steady state by day 6 of the infusion when plasma levels were 60 times higher than IdUrd. Plasma levels of uracil and thymine, but not thymidine, were elevated throughout the infusion. With a minimum follow-up of 1 year, ten patients remain alive, while 33 patients died of progressive disease, and four patients died of other causes (including one treatment-related death). The median survival for all 47 patient and for the 40 patients receiving the MTD was 45 and 47 weeks, respectively, with 12% and 14% survivals at 24 months. Using a Cox regression analysis, age (less than or equal to 50 years v greater than 50 years) and pretreatment performance status (PS) (Eastern Cooperative Oncology Group [ECOG]-PS 0 to 1 v PS 2 to 3) were independent, statistically significant (P2 less than .05) predictors of survival, with the ECOG status being a better predictor. Patients with a PS 0 to 1 (28 patients) had a median survival of 64 weeks with 21% survival at 24 months, compared with a median survival of 29 weeks and 0% survival at 12 months in the 19 patients with PS 2 to 3. The overall and subgroup survival data are at least comparable to other combined modality treatment approaches in patients with GBM.