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Daryl R. Fourney, Gregory N. Fuller and Ziya L. Gokaslan

✓ Extradural ependymomas of the sacrococcygeal region are very rare, with most arising from the soft tissues of the presacral area or from the regions dorsal to the sacrum. In even rarer circumstances, the tumor may arise within the sacral canal, likely as a result of ependymal cells of the extradural filum terminale. Because of bone erosion caused by extension of the tumor into the pelvis or dorsal to the sacrum, a truly intraspinal extradural ependymoma in this region has until now never been clearly demonstrated. The authors present a patient with a myxopapillary ependymoma arising from the filum terminale externa in which there was no involvement of the intradural filum or extension outside the sacral canal. A review of the literature is presented, with emphasis on the pathogenesis and clinical management of these rare tumors.

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Daryl R. Fourney, Dima Abi-Said, Frederick F. Lang, Ian E. McCutcheon and Ziya L. Gokaslan

Object. Few reports are available on the use of pedicle screw fixation for cancer-related spinal instability. The authors present their experience with pedicle screw fixation in the management of malignant spinal column tumors.

Methods. Records for patients with malignant spinal tumors who underwent pedicle screw fixation at the authors' institution between September 1994 and December 1999 were retrospectively reviewed.

Results. Ninety-five patients with malignant spinal tumors underwent 100 surgeries involving pedicle screw fixation: metastatic spinal disease was present in 81 patients, and locally invasive tumors were demonstrated in 14 patients. Indications for surgery were pain (98%) and/or neurological dysfunction (80%). A posterior (48%) or a combined anterior—posterior (52%) approach was performed depending on the extent of tumor and the patient's condition. At the mean follow up of 8.2 months, 43 patients (45%) had died; median survival, as determined by Kaplan—Meier analysis, was 14.8 months. At 1 month postsurgery, self-reported pain had improved in 87% of cases (p < 0.001), which is a finding substantiated by reductions in analgesic use, and 29 (47%) of 62 patients with preoperative neurological impairments were functionally improved (p < 0.001). Postoperative complications were associated only with preoperative radiation therapy (p = 0.002) and with preexisting serious medical conditions (p = 0.04). In two patients asymptomatic violation of the lateral wall of the pedicle was revealed on postoperative radiography. The 30-day mortality rate was 1%.

Conclusions. For selected patients with malignant spinal tumors, pedicle screw fixation after tumor resection may provide considerable pain relief and restore or preserve ambulation with acceptable rates of morbidity and mortality.

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Daryl R. Fourney, Dima Abi-Said, Laurence D. Rhines, Garrett L. Walsh, Frederick F. Lang, Ian E. McCutcheon and Ziya L. Gokaslan

Object. Thoracic or lumbar spine malignant tumors involving both the anterior and posterior columns represent a complex surgical problem. The authors review the results of treating patients with these lesions in whom surgery was performed via a simultaneous anterior—posterior approach.

Methods. The hospital records of 26 patients who underwent surgery via simultaneous combined approach for thoracic and lumbar spinal tumors at our institution from July 1994 to March 2000 were reviewed. Surgery was performed with the patients in the lateral decubitus position for the procedure. The technical details are reported.

The mean survival determined by Kaplan—Meier analysis was 43.4 months for the 15 patients with primary malignant tumors and 22.5 months for the 11 patients with metastatic spinal disease. At 1 month after surgery, 23 (96%) of 24 patients who complained of pain preoperatively reported improvements (p < 0.001, Wilcoxon signed-rank test), and eight (62%) of 13 patients with preoperative neurological deficits were functionally improved (p = 0.01). There were nine major complications, five minor complications, and no deaths within 30 days of surgery. Two patients (8%) later underwent surgery for recurrent tumor.

Conclusions. The simultaneous anterior—posterior approach is a safe and feasible alternative for the exposure tumors of the thoracic and lumbar spine that involve both the anterior and posterior columns. Advantages of the approach include direct visualization of adjacent neurovascular structures, the ability to achieve complete resection of lesions involving all three columns simultaneously (optimizing hemostasis), and the ability to perform excellent dorsal and ventral stabilization in one operative session.

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Michel Lacroix, Dima Abi-Said, Daryl R. Fourney, Ziya L. Gokaslan, Weiming Shi, Franco DeMonte, Frederick F. Lang, Ian E. McCutcheon, Samuel J. Hassenbusch, Eric Holland, Kenneth Hess, Christopher Michael, Daniel Miller and Raymond Sawaya

Object. The extent of tumor resection that should be undertaken in patients with glioblastoma multiforme (GBM) remains controversial. The purpose of this study was to identify significant independent predictors of survival in these patients and to determine whether the extent of resection was associated with increased survival time.

Methods. The authors retrospectively analyzed 416 consecutive patients with histologically proven GBM who underwent tumor resection at the authors' institution between June 1993 and June 1999. Volumetric data and other tumor characteristics identified on magnetic resonance (MR) imaging were collected prospectively.

Conclusions. Five independent predictors of survival were identified: age, Karnofsky Performance Scale (KPS) score, extent of resection, and the degree of necrosis and enhancement on preoperative MR imaging studies. A significant survival advantage was associated with resection of 98% or more of the tumor volume (median survival 13 months, 95% confidence interval [CI] 11.4–14.6 months), compared with 8.8 months (95% CI 7.4–10.2 months; p < 0.0001) for resections of less than 98%. Using an outcome scale ranging from 0 to 5 based on age, KPS score, and tumor necrosis on MR imaging, we observed significantly longer survival in patients with lower scores (1–3) who underwent aggressive resections, and a trend toward slightly longer survival was found in patients with higher scores (4–5). Gross-total tumor resection is associated with longer survival in patients with GBM, especially when other predictive variables are favorable.

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Total cervical spondylectomy for primary osteogenic sarcoma

Case report and description of operative technique

Zvi R. Cohen, Daryl R. Fourney, Rex A. Marco, Laurence D. Rhines and Ziya L. Gokaslan

✓ The authors describe a technique for total spondylectomy for lesions involving the cervical spine. The method involves separately staged anterior and posterior approaches and befits the unique anatomy of the cervical spine. The procedure is described in detail, with the aid of radiographs, intraoperative photographs, and illustrations. Unlike in the thoracic and lumbar spine—for which methods of total en bloc spondylectomy have previously been described—a strictly en bloc resection is not possible in the cervical spine because of the need to preserve the vertebral arteries and the nerve roots supplying the upper limbs. Although the resection described in this case is by definition intralesional, it is oncologically sound, given the development of effective neoadjuvent chemotherapeutic regimens for osteosarcoma.

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Daryl R. Fourney, Donald F. Schomer, Remi Nader, Jennifer Chlan-Fourney, Dima Suki, Kamran Ahrar, Laurence D. Rhines and Ziya L. Gokaslan

Object. The current North American experience with minimally invasive vertebro- and kyphoplasty is largely limited to the treatment of benign osteoporotic compression fractures. The objective of this study was to assess the safety and efficacy of these procedures for painful vertebral body (VB) fractures in cancer patients.

Methods. The authors reviewed a consecutive group of cancer patients (21 with myeloma and 35 with other primary malignancies) undergoing vertebro- and kyphoplasty at their institution. Ninety-seven (65 vertebro- and 32 kyphoplasty) procedures were performed in 56 patients during 58 treatment sessions. The mean patient age was 62 years (± 13 years [standard deviation]) and the median duration of symptoms was 3.2 months. All patients suffered intractable spinal pain secondary to VB fractures.

Patients noted marked or complete pain relief after 49 procedures (84%), and no change after five procedures (9%); early postoperative Visual Analog Scale (VAS) pain scores were unavailable in four patients (7%). No patient was worse after treatment. Reductions in VAS pain scores remained significant up to 1 year (p = 0.02, Wilcoxon signed-rank test). Analgesic consumption was reduced at 1 month (p = 0.03, Wilcoxon signed-rank test). Median follow-up length was 4.5 months (range 1 day–19.7 months). Asymptomatic cement leakage occurred during vertebroplasty at six (9.2%) of 65 levels; no cement extravasation was seen during kyphoplasty. There were no deaths or complications related to the procedures. The mean percentage of restored VB height by kyphoplasty was 42 ± 21%.

Conclusions. Percutaneous vertebro- and kyphoplasty provided significant pain relief in a high percentage of patients, and this appeared durable over time. The absence of cement leakage—related complications may reflect the use of 1) high-viscosity cement; 2) kyphoplasty in selected cases; and 3) relatively small 3volume injection. Precise indications for these techniques are evolving; however, they are safe and feasible in well-selected patients with refractory spinal pain due to myeloma bone disease or metastases.

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Daryl R. Fourney and Ziya L. Gokaslan

In addition to tumor resection, a major goal of spine surgery involving tumors is the preservation or achievement of spinal stability. The criteria defining stability, originally developed for use in trauma, are not directly applicable in the setting of neoplasia. The authors discuss the most common patterns of tumor-related instability and deformity at all levels of the spinal column and review the surgical options for treatment.

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Daryl R. Fourney, Julie E. York, Zvi R. Cohen, Dima Suki, Laurence D. Rhines and Ziya L. Gokaslan

Object. The treatment of atlantoaxial spinal metastases is complicated by the region's unique biomechanical and anatomical characteristics. Patients most frequently present with pain secondary to instability; neurological deficits are rare. Recently, some authors have performed anterior approaches (transoral or extraoral) for resection of upper cervical metastases. The authors review their experience with a surgical strategy that emphasizes posterior stabilization of the spine and avoidance of poorly tolerated external orthoses such as the rigid cervical collar or halo vest.

Methods. The authors performed a retrospective review of 19 consecutively treated patients with C-1 or C-2 metastases who underwent surgery at The University of Texas M. D. Anderson Cancer Center between 1994 and 2001.

Visual analog pain scores were reduced at 1 and 3 months (p < 0.005, Wilcoxon signed-rank test); however, evaluation of pain at 6 months and 1 year was limited by the remaining number of surviving patients. Analgesic medication consumption was unchanged. There were no cases of neurological decline or sudden death secondary to residual or recurrent atlantoaxial disease during the follow-up period. One patient underwent revision of hardware at 11 months. The mean follow-up period was 8 months (range 1–32 months). Median survival determined by Kaplan—Meier analysis was 6.1 months (95% confidence interval 2.99–9.21).

Conclusions. Occipitocervical stabilization provided durable pain relief and preservation of ambulatory status over the remaining life span of patients. Because of the palliative goals of surgery, the authors have not found an indication for anterior-approach tumor resection in these patients. Successful stabilization obviates the need for an external orthosis.

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Daryl R. Fourney and Ziya L. Gokaslan

Sacral chordomas are relatively rare, locally invasive, malignant neoplasms. Although metastasis is infrequent at presentation, the prognosis for patients with chordoma of the sacrum is reported to be poor and attributable in most cases to intralesional resection. The value of adjuvant treatment is uncertain, and resection remains the primary mode of treatment. Chordomas are difficult to excise completely, but recent improvements in imaging and surgical techniques have allowed surgeons to perform more frequently en bloc sacral resections with wide surgical margins. The technical challenges of such operations, and the functional costs for the patient (with respect to anorectal and urogenital dysfunction) are significantly increased when the tumor involves high sacral levels. The authors review the clinical presentation and natural history of sacral chordoma and discuss the current treatment techniques and outcomes.

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Giant cell ependymoma of the spinal cord

Case report and review of the literature

Daryl R. Fourney, Abdolreza Siadati, Janet M. Bruner, Ziya L. Gokaslan and Laurence D. Rhines

✓ Several rare histological variants of ependymoma have been described. The authors report on a patient in whom cervical spinal cord astrocytoma was originally diagnosed after evaluation of a limited biopsy specimen. More abundant tissue obtained during gross-total resection included areas of well-differentiated ependymoma. The histological features of the tumor were extremely unusual, with a major component of pleomorphic giant cells. Its histological, immunohistochemical, and electron microscopic features, however, were consistent with ependymoma. Only two cases of terminal filum and two of supratentorial giant cell variant of ependymoma have been reported. To the authors' knowledge, this represents the first case of giant cell ependymoma of the spinal cord. The clinical significance is the potential for misdiagnosis with anaplastic (gemistocytic) astrocytoma, especially in cases in whom limited biopsy samples have been obtained.