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Definitive radiation therapy for chordoma and chondrosarcoma of base of skull and cervical spine.

Proton-beam radiation therapy has been developed for the treatment of chordomas or sarcomas of bone or soft tissue that abut the central nervous system. The authors report the results of treatment of 10 patients, six with chordoma, three with chondrosarcoma, and one with a neurofibrosarcoma. Local control has been achieved for all patients (with, however, one marginal failure) with a follow-up period ranging from 2 months to 6 years. High doses of radiation, up to 76 Cobalt Gray Equivalents (CGE), have been delivered without significant morbidity. In particular, no neurological sequelae have been observed.

Adult↗

Retrospective long-term follow-up analysis in 21 patients with chordomas of various sites treated at a single institution.

Twenty-one patients with chordoma were treated at the Radiation Oncology Center, Mallinckrodt Institute of Radiology, between 1949 and 1986. Thirteen patients had sacrococcygeal tumors, five had clival tumors, two had nasopharyngeal tumors, and one had a lumbar spine tumor. Nine patients were treated with surgery alone, eight patients with subtotal resection and postoperative irradiation, and four patients with radiotherapy alone after biopsy. The 5- and 10-year actuarial survival rates were 74% and 46%, respectively. The 10-year actuarial survival rate was significantly better in patients treated with surgery alone or surgery and irradiation than in those treated with radiotherapy alone (52%, 32%, and 0%, respectively, p = 0.02). Although all patients ultimately suffered a recurrence, those with lumbosacral tumors treated with surgery and irradiation had a longer mean disease-free survival period (6.6 years) than those treated with surgery alone (4.1 years) (p = 0.08). Disease-free survival times of patients with base of the skull tumors was not significantly different between the treatment groups. Irradiation after resection of chordomas appears to increase the time to first relapse in lumbosacral tumors and should be considered after subtotal resection.

Actuarial Analysis↗

Cervical corpectomy and plate fixation for postlaminectomy kyphosis.

Between 1987 and 1991, 20 patients with symptomatic postlaminectomy kyphosis were treated with anterior decompression, bone graft, and anterior cervical plate. The patients were predominantly male (14:6) with a mean age of 58 years. The initial laminectomy was performed for either spondylosis (80%) or spinal tumor (20%). All patients had anterior compressive pathology, which was associated with instability (45%), neck pain (75%), myeloradiculopathy (90%), or severe neck deformity (30%). The mean degree of kyphosis was 38 degrees. Treatment consisted of a trial of cervical traction (75%), anterior corpectomy (95%), intersegmental decompression (5%), bone fusion (100%), and fixation with either Caspar (85%) or Synthes (15%) anterior plating at a mean of 3.8 levels. Halo fixation was used in 10% of patients. Postoperative complications included vocal cord paresis (15%), pneumonia (10%), wound dehiscence (5%), and screw pull-out (5%). At follow-up evaluation, a mean of 28 months after treatment, all patients had a solid fusion and a mean curvature improvement to 16 degrees residual kyphosis. Neurologically, 10% were cured, 55% were improved and returned to premorbid function, 30% were stable, and 5% had late progression. These data suggest that immediate fixation with anterior plating facilitates solid fusion, maintains spinal curvature, and promotes neurological improvement.

Adult↗

Posterior cervical spine fusion with tension-band wiring.

In this study the authors detail their experience with posterior tension-band wiring for stabilization of the subaxial cervical spine. Fifty-five patients underwent fusion for trauma (41 patients), degenerative disease (13 patients), and tumor (one patient). The fusion rate was 96% (50 of 52 patients) and postoperative immobilization was accomplished by means of a Philadelphia collar in the majority of cases. Tension-band wiring provides a stable construct that is simple to perform, requires fusion of a minium number of motion segments, and allows early mobilization with only a hard collar needed for support. The details of the technique, which has been modified from preliminary descriptions, are discussed.

Adolescent↗

Surgical seeding of chordomas.

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.

Adolescent↗

Suboccipital and cervical chordomas: the value of aggressive treatment at first presentation of the disease.

OBJECT: Chordoma is a locally invasive tumor with a high tendency for recurrence for which radical resection is generally recommended. To assess the benefits of aggressive treatment of chordomas, the authors compared results in patients treated aggressively at the first presentation of this disease with results in patients who were similarly treated, but after recurrence. METHODS: Among 36 patients with cervical chordomas who were treated at the authors' institution, 22 underwent primary aggressive treatment (Group A) and 14 were treated secondarily after tumor recurrence (Group B). Two cases were excluded from Group A because of unrelated early deaths and three from Group B because of insufficient pre- or postoperative data. Most tumors were located at the suboccipital level and only eight cases at a level below C-2. Radiotherapy and proton therapy were similarly conducted in both groups of patients. The actuarial survival rates were 80 and 65% at 5 and 10 years, respectively, in Group A patients and 50 and 0% at 5 and 10 years, respectively, in Group B patients (p = 0.049, log-rank test). The actuarial recurrence-free rates were 70 and 35% at 5 and 10 years, respectively, in Group A and 0% at 3 years in Group B (p < 0.0001, log-rank test). The numbers of recurrences per year were 0.15 in Group A and 0.62 in Group B (p > 0.05). All other parameters that were analyzed (patient age, delay before diagnosis, clinical symptoms, chondroid type of lesion, and histological features) did not prove to influence prognosis in a statistically significant manner. CONCLUSIONS: Aggressive therapy, combining as radical a resection as possible with radiotherapy, seems to improve the prognoses of suboccipital and cervical chordomas when applied at the patient's first presentation with the disease.

Adolescent↗

Vertebral hemangiomas associated with familial cerebral cavernous malformation: segmental disease expression. Case report.

Recently, several groups of authors have described mutations in the Krev interaction-trapped 1 [corrected] (KRIT1) gene in families in whom cerebral cavernous malformations (CCMs) are present. In a number of French kindreds harboring familial CCMs, cutaneous as well as cerebral manifestations of this autosomal-dominant disorder were demonstrated. Involvement of other tissues has been poorly described. The authors present the proband, in an affected family with a previously reported KRIT1 mutation, in whom vertebral hemangiomas in addition to cerebral and cutaneous lesions were found. One of the vertebral lesions was associated with a large cutaneous lesion. This combination of vertebral and overlying cutaneous lesions suggests segmental disease expression as the result of a second hit during development, implying loss of function as the relevant molecular pathogenic mechanism. This case illustrates that tissue involvement outside the nervous system must be considered when treating patients with familial CCMs.

Biopsy↗

Hydroxyapatite laminar spacers and titanium miniplates in cervical laminoplasty.

OBJECTIVE: The authors describe a new surgical technique for cervical laminoplasty that was performed in 25 patients. The posterior elements along with the various ligaments are removed en bloc and are stabilized in a lift-up position by placing hydroxyapatite (HA) laminar spacers and titanium miniplates and screws. The procedure and clinical results are discussed. METHODS: The posterior spinal elements, including the lamina(e), spinous process(es), and various attached ligaments, are removed en bloc by incising the lamina in its lateral aspect. Trapezoid-shaped HA spacers are placed between the cut ends of the laminae or between the laminae and lateral masses bilaterally at each level. Malleable titanium miniplates and screws are used for fixation of the spacers. The fixation of transected laminae was judged to be successful. Postoperative care included application of a soft neck collar for 1 week but no further restriction of activity. Surgery-related outcome was assessed in the 21 patients who attended more than 6 months of follow up after laminoplasty. There were 18 men and three women who ranged in age from 27 to 81 years. Cervical stenotic myelopathy was demonstrated in 15 patients who underwent decompressive and expansive laminoplasty, and spinal tumors were documented in six patients who underwent a nonexpansive laminoplasty. Postoperative and follow-up computerized tomography scans demonstrated no hardware failure. Bone formation around the spacers was observed either at 6- or at 9-month follow-up examination in all 21 patients. Fusion of the reconstructed laminae was found to be completed at 12 months in all 18 patients able to attend follow up for this duration. Spinal alignment and the range of motion of the cervical spine were well preserved. In patients with stenotic cervical myelopathy, neurological and anatomical outcome of canal expansion were satisfactory. CONCLUSIONS: This technique enables rigid laminoplasty while maintaining anatomical and biomechanical integrity of posterior elements of cervical spine. Expansive and nonexpansive laminoplasty procedures are possible.

Adult↗

Rotational and transpositional flaps for the treatment of spinal wound dehiscence and infections in patient populations with degenerative and oncological disease.

OBJECT: Wound-related complications following complex posterior spine procedures may result in the need for serial debridements and may place the instrumentation at risk. Numerous treatments have been advocated for this problem, but each has limitations. In this article the authors discuss the experience from two large teaching institutions at which rotational and transpositional flaps were used in the management of deep wound infections and dehiscences. METHODS: The authors generated a list of patients treated via posterior or posterolateral approaches for metastatic tumors or complex degenerative disorders in whom wound complications subsequently developed. Data were obtained from the medical records and reviewed retrospectively. Thirty-seven patients were treated with rotational or transpositional flaps at the two institutions during the study period. Patients underwent a mean of 1.3 procedures for the treatment of wound healing problems, and cultures were positive in 70%. In three patients (8%) this treatment failed due to protrusion of hardware through the skin or repeated dehiscence requiring reclosure. Spinal instrumentation was salvaged in 97% of the cases. CONCLUSIONS: The use of local tissue flaps is advantageous for treatment of posterior wound complications due to spine surgery. In this procedure highly vascularized tissue is used to increase healing, accelerate clearance of bacteria, and fill any dead space.

Adolescent↗

Occipitocervical fusion after resection of craniovertebral junction tumors.

OBJECT: Surgical access to tumors at the craniovertebral junction (CVJ) requires extensive bone removal. Guidelines for the use of occipitocervical fusion (OCF) after resection of CVJ tumors have been based on anecdotal evidence. The authors performed a retrospective study of factors associated with the use of OCF in 46 patients with CVJ tumors. The findings were used to develop recommendations for use of OCF in such patients. METHODS: The authors retrospectively reviewed the cases of 51 patients with CVJ tumors treated by their group between March 1991 and February 2004. Forty-six patients were available for follow up. Charts were reviewed to obtain data on demographic characteristics, presenting symptoms, and perioperative complications. Preoperative computerized tomography scans and magnetic resonance imaging studies were obtained in all patients. Occipitocervical fusion was performed in patients who had undergone a unilateral condyle resection in which 70% or more of the condyle was removed, a bilateral condyle resection with 50% removal, or C1-2 vertebral body destruction. Of the 46 patients, 16 had foramen magnum meningiomas, 17 had chordomas, one had a chondrosarcoma, two had Schwann cell tumors, two had glomus tumors, and eight had other types of tumors. Twenty-three (50%) of the 46 patients underwent OCF, including 15 of the 17 patients with chordomas (88%). None of the patients with meningiomas required fusion. Seventeen (71%) of the 24 patients presenting with neck pain preoperatively underwent OCF. CONCLUSIONS: Patients presenting with neck pain had a 71% chance of undergoing OCF. Patients with chordomas and metastatic tumors were most likely to require OCF. One patient with a 50% unilateral condylar resection returned with OC instability for which OCF was required. Based on their clinical experience and published biomechanical studies, the authors recommend that OCF be performed when 50% or more of one condyle is resected.

Adolescent↗