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Background: Chordomas are rare, slow-growing, locally aggressive, malignant tumors of the spine. Chordomas are conventionally treated with surgical resection with or without radiation. There is an absence of literature documenting the natural history of a primary sacral chordoma., Case Description: A 65-year-old man presented with rectal pain, constipation, urinary and fecal incontinence, S1 radiculopathy, and a palpable rectal mass. A needle biopsy confirmed the pathologic diagnosis of sacral chordoma. The patient declined to have surgery because of the surgical risks involved. He was managed conservatively with supportive care only. The patient was routinely followed in clinic and had a subjective and objective excellent quality of life with adequate pain management. Meanwhile, his neurologic status did not deteriorate. During follow-up, some posterolateral aspects of the chordoma regressed. However, the bulk of the lesion continued to slowly progress. The patient survived for 7.5 years. He eventually succumbed to urosepsis and new-onset peritoneal metastasis., Conclusions: To our knowledge, the patient is the only documented case in the literature of an untreated biopsy-proven sacral chordoma. The patient's tumor was intended for resection, and therefore comparable with data from treated chordomas. The patient's survival is similar to the median survival in treated chordomas. The patient's survival was despite negative prognosticators, such as advanced age of the patient and high sacral location above S2., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Object: Hematogenous metastases to the sacrum can produce significant pain and lead to spinal instability. The object of this study was to evaluate the palliative benefit of surgery in patients with these metastases., Methods: The authors retrospectively reviewed all cases involving patients undergoing surgery for metastatic disease to the sacrum at a single tertiary cancer center between 1993 and 2005., Results: Twenty-five patients (21 men, 4 women) were identified as having undergone sacral surgery for hematogenous metastatic disease during the study period. Their median age was 57 years (range 25-71 years). The indications for surgery included palliation of pain (in 24 cases), need for diagnosis (in 1 case), and spinal instability (in 3 cases). The most common primary disease was renal cell carcinoma. Complications occurred in 10 patients (40%). The median overall survival was 11 months (95% CI 5.4-16.6 months). The median time from the initial diagnosis to the diagnosis of metastatic disease in the sacrum was 14 months (95% CI 0.0-29.3 months). The numerical pain scores (scale 0-10) were improved from a median of 8 preoperatively to a median of 3 postoperatively at 90 days, 6 months, and 1 year (p < 0.01). Postoperative modified Frankel grades improved in 8 cases, worsened in 3 (due to disease progression), and remained unchanged in 14 (p = 0.19). Among patients with renal cell carcinoma, the median overall survival was better in those in whom the sacrum was the sole site of metastatic disease than in those with multiple sites of metastatic disease (16 vs 9 months, respectively; p = 0.053)., Conclusions: Surgery is effective to palliate pain with acceptable morbidity in patients with metastatic disease to the sacrum. In the subgroup of patients with renal cell carcinoma, those with the sacrum as their solitary site of metastatic disease demonstrated improved survival.

Object: Sarcomas of the spine are a challenging problem due to their frequent and extensive involvement of multiple spinal segments and high recurrence rates. Gross-total resection to negative margins, with preservation of neurological function and palliation of pain, is the surgical goal and may be achieved using either intralesional resection or en bloc excision. The authors report outcomes of surgery for primary and metastatic sarcomas of the mobile spine in a large patient series., Methods: A retrospective review of patients undergoing resection for sarcomas of the mobile spine between 1993 and 2005 was undertaken. Sarcomas were classified by histology study results and as either primary or metastatic. Details of the surgical approach, levels of involvement, and operative complications were recorded. Outcome measures included neurological function, palliation of pain, local recurrence, and overall survival., Results: Eighty patients underwent 110 resections of either primary or metastatic sarcomas of the mobile spine. Twenty-nine lesions were primary sarcomas (36%) and 51 were metastatic sarcomas (64%). Intralesional resections were performed in 98 surgeries (89%) and en bloc resections were performed in 12 (11%). Median survival from surgery for all patients was 20.6 months. Median survival for patients with a primary sarcoma of the spine was 40.2 months and was 17.3 months for patients with a metastatic sarcoma. Predictors of improved survival included a chondrosarcoma histological type and a better preoperative functional status, whereas osteosarcoma and a high-grade tumor were negative influences on survival. Multivariate analysis showed that only a high-grade tumor was an independent predictor of shorter overall survival. American Spinal Injury Association scale grades were maintained or improved in 97% of patients postoperatively, and there was a significant decrease in pain scores postoperatively. No significant differences in survival or local recurrence rates between intralesional or en bloc resections for either primary or metastatic spine sarcomas were found., Conclusions: Surgery for primary or metastatic sarcoma of the spine is associated with an improvement in neurological function and palliation of pain. The results of this study show a significant difference in patient survival for primary versus metastatic spine sarcomas. The results do not show a statistically significant benefit in survival or local recurrence rates for en bloc versus intralesional resections for either metastatic or primary sarcomas of the spine, but this may be due to the small number of patients undergoing en bloc resections.

Object: The authors conducted a study to evaluate the clinical characteristics and surgical outcomes in patients with spinal schwannomas and without neurofibromatosis (NF)., Methods: The data obtained in 128 patients who underwent resection of spinal schwannomas were analyzed. All cases with neurofibromas and those with a known diagnosis of NF Type 1 or 2 were excluded. Karnofsky Performance Scale (KPS) scores were used to compare patient outcomes when examining the anatomical location and spinal level of the tumor. The neurological outcome was further assessed using the Medical Research Council (MRC) muscle testing scale., Results: Altogether, 131 schwannomas were treated in 128 patients (76 males and 52 females; mean age 47.7 years). The peak prevalence is seen between the 3rd and 6th decades. Pain was the most common presenting symptom. Gross-total resection was achieved in 127 (97.0%) of the 131 lesions. The nerve root had to be sacrificed in 34 cases and resulted in minor sensory deficits in 16 patients (12.5%) and slight motor weakness (MRC Grade 3/5) in 3 (2.3%). The KPS scores and MRC grades were significantly higher at the time of last follow-up in all patient groups (p = 0.001 and p = 0.005, respectively)., Conclusions: Spinal schwannomas may occur at any level of the spinal axis and are most commonly intradural. The most frequent clinical presentation is pain. Most spinal schwannomas in non-NF cases can be resected totally without or with minor postoperative deficits. Preoperative autonomic dysfunction does not improve significantly after surgical management.

The role of surgery in the treatment of metastatic spinal tumors causing epidural compression traditionally consisted of posterior decompression. This procedure plus radiotherapy, however, could not be demonstrated to provide any benefit over radiotherapy alone, and surgery fell into disfavor in managing metastatic vertebral tumors. The advent of newer, more sophisticated approaches, along with improved spinal instrumentation and reconstruction techniques, which allowed direct decompression of neural elements and resection of the tumor, have revived the use of surgery in these tumors. These modern spinal surgery techniques, in combination with radiotherapy, have yielded significantly superior functional outcomes and prolonged survival in symptomatic metastatic epidural compression when compared to radiotherapy alone. Management of spinal metastases is evolving, and a multitude of factors determine the indication for and the technique and goals of surgical intervention. Between 1993 and 2005, 21.1% of patients with metastatic spinal tumors evaluated at The University of Texas M.D. Anderson Cancer Center were treated surgically by the Department of Neurosurgery. The most common spinal metastasis operated upon was metastatic kidney cancer (31.5%), even though kidney cancer was only the third most common primary tumor (after lung and breast cancers) giving rise to vertebral metastases observed during the same time period at this institution. This highlights the importance of the histology of the primary cancer (among other factors) in determining the indication for surgical intervention.

Object: Metastases of multiple myeloma often occur in the cervical spine. These metastases may cause pain and associated spinal instability. The authors report the results of radiotherapy and surgical treatment for myeloma involving the cervical spine. The results of radiation therapy for multiple myeloma metastases to the cervical spine that cause clinical or radiographically documented instability have not been reported previously., Methods: A retrospective chart review of patients with multiple myeloma metastases to the cervical spine was undertaken. Between 1993 and 2005, 35 patients were treated with external-beam radiation and/or surgical stabilization at the University of Texas M. D. Anderson Cancer Center in Houston, Texas. Nineteen of 20 patients with sufficient follow-up data experienced resolution of their pain when treated with radiation without surgical intervention. Twenty-three patients had evidence of spinal instability on radiographic images; 15 of these were treated with radiation alone. Of these, 10 had sufficient follow-up data, and none showed any clinical progression of instability. Radiographic follow-up images demonstrated an arrest of further progression of instability and, in some cases, healing of pathological fractures by means of radiation alone., Conclusions: The results of this series suggest that, in selected cases, external-beam radiation for multiple myeloma metastases to the cervical spine is an effective palliative treatment, even in cases involving clinical or radiographically documented instability.

The authors report the use of percutaneous transpedicular vertebroplasty performed using polymethylmethacrylate (PMMA) in two patients. These men (53 and 57 years old) had previously undergone open surgery and posterior instrumentation to treat myelomatous compression fractures. Both patients presented with acute back pain that manifested after minor activities. Kyphotic wedge fractures were diagnosed at T-1 in one case and at L-1 in the other. Both patients were treated at other hospitals with laminectomy and instrumented fusion; multiple myeloma was diagnosed after surgery. The patients experienced severe, recalcitrant, and progressive pain; on referral, they were found to have persistent kyphosis. Multiple myelomatous lesions of the spine were seen in one case and in the other the L-1 fracture represented the only site of disease. Percutaneous vertebroplasty was performed by injecting PMMA into the anterior third of the compressed vertebral body. Both patients experienced a 50% reduction in pain immediately after treatment; 3 months later both were walking and reported minimal back pain while undergoing treatment for multiple myeloma. Three years after surgery one patient reported no back pain and no progressive instability of the spine. Four years after surgery the other patient remains pain free, ambulatory, and with overall disease remission. Percutaneous vertebroplasty provided effective analgesia in these two patients with progressive back pain despite posterior stabilization. In both cases, the anterior column was effectively stabilized. A much larger operative intervention with its attendant risks of morbidity was avoided. In addition, subsequent aggressive medical treatment was well tolerated.