A total of 16 patients in our clinic (six women, ten men; mean age 54.87 years, range 38-78 years) were diagnosed as having a sacrococcygeal chordoma. Pain was the presenting symptom in all patients. In five patients, the chordoma was inoperable. A total of 11 patients were followed-up for a mean period of 64.8 months (range 7-152 months). Five patients were lost to follow-up (3 in the operable group and two in the inoperable group). The three remaining inoperable patients received radiation therapy. The eight remaining operable patients underwent a total of 12 operations (four anterior and posterior, eight posterior only). Five of these patients received adjuvant radiotherapy and two patients received both radiotherapy and chemotherapy. In follow-up, eight patients had evidence of disease and one patient remained disease-free. Problems encountered during therapy and follow-up included urinary incontinence (72%), rectal incontinence (36%), wound infection (36%), and lower extremity muscle weakness (36%). Two patients died from metastases to the lung. Of the remaining nine patients, eight were ambulatory, with seven needing support to walk. One patient was unable to walk at all due to lower extremity muscle weakness. (+info)
Skull base chordomas: management and results.
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Growth patterns of skull base chordomas are related to important neurovascular structures. Local invasiveness results in "clinically malignant" behavior. A high rate of transient neurological deficits occurs following radical surgery. At our institution, the principle of radical removal is not followed at any price. This study compared the results of our management with recent series. Eleven patients, five females and six males aged 24-65 years (mean 41 years), underwent removal of skull base chordoma with size one <3 cm, five 3-5 cm, and five >5 cm. Mostly, standard operative approaches were chosen. All patients underwent postoperative radiotherapy. Resection was subtotal/partial in seven patients and total in four with no mortality. Neurological deterioration occurred due to transient cranial nerve deficits in six patients. Temporary surgical morbidity (including cranial nerve deficits) was observed in seven patients. Median Karnofsky performance status score improved compared to preoperative (80), early postoperative (70), and latest assessment (90) (median 36 months). Five patients underwent reoperation due to tumor recurrence after 4-48 months (mean 24 months). Most patients undergoing removal of skull base chordomas suffer from transient neurological deficits which are mainly nonsignificant as the patients return to preoperative functional status. The apparently high rate of incomplete tumor resection (64%) reflects the infiltrative behavior and relationship with neurovascular structures. The operative strategy should not be excessively aggressive at any price, but rather take into account the options of radiotherapy and observation of residual tumor. (+info)
Familial chordoma. A report of two cases.
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We have treated 175 patients with a chordoma over a ten-year period. Only two had a family history of the condition and we describe these in this paper. In one patient the tumour was at the craniocervical junction and in the other the lesion affected the sacrum. We have undertaken a literature review of familial chordoma and have identified chromosomal abnormalities associated with the condition. (+info)
Preadolescent presentation of a lumbar chordoma: results of vertebrectomy and fibula strut graft reconstruction at 8 years.
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Chordoma is a tumour of notochordal origin which usually involves the sacrum or skull base presenting in adulthood. Chordoma in a mobile spinal segment is infrequent and the authors report an extremely rare presentation of L3 chordoma in a child aged 7 years. Although a benign tumour, mobile segment chordoma is more locally aggressive, more likely to metastasise and has a poorer 5 year survival than sacral and clival lesions. Wide surgical excision and reconstruction is the treatment of choice in vertebral chordoma. This case was treated with staged vertebrectomy and fibular strut graft reconstruction and the results of clinical and radiological follow up at 8 years are presented. (+info)
Combined radiotherapy and razoxane in the treatment of chondrosarcomas and chordomas.
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BACKGROUND: Chondrosarcomas and chordomas are reported to have low radio-sensitivity. Therefore, a study was undertaken to explore the radioresponsiveness of these tumours using the sensitising agent razoxane. PATIENTS AND METHODS: Thirteen chondrosarcomas and five chordomas were irradiated with high-energy photons and razoxane in the period from 1984 to 2003. The median tumour dose was 60 Gy in the chondrosarcomas and 63 Gy in chordomas. Razoxane tablets were given at a dose of 125 mg twice daily starting 5 days before the first irradiation. The drug was continued on radiation days. RESULTS: Eight out of the 13 chondrosarcomas had unresectable or recurrent measurable disease. There were one complete and five partial responses, while two tumours remained unchanged (response rate 75%). The median duration of response was 22 months. Three out of four patients without clear surgical margins and one patient with clear margins had locally controlled disease. Overall, local control was achieved in seven out of twelve patients who were not radically resected. All five patients with chordomas survived 5 years and remained locally controlled at that time. Among four measurable tumours, two complete and one partial regression were noted. Razoxane was well tolerated; the dose limiting toxicity was leukopenia. CONCLUSION: Photon irradiation together with razoxane induces major responses in a majority of patients with chondrosarcomas and chordomas. This combination therapy seems to be more effective than photon irradiation alone. (+info)
Primary bone tumours of the spine: a 42-year survey from the Leeds Regional Bone Tumour Registry.
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We conducted a review of the Leeds Regional Bone Tumour Registry for primary bone tumours of the spine since establishment in 1958 until year 2000. To analyse the incidence of primary tumours of the spine and to record the site of occurrence, sex distribution, survival and pathology of these tumours. Primary tumours of the spine are particularly rare, accounting for between 4 and 13% of published series of primary bone tumours. The Leeds Bone Tumour Registry was reviewed and a total of 2,750 cases of bone tumours and tumour-like cases were analysed. Consultants in orthopaedic surgery, neurosurgery, oncology and pathology in North and West Yorkshire and Humberside contribute to the Registry. Primary bone tumours of the osseous spine constitute only 126 of the 2,750 cases (4.6%). Chordoma was the most frequent tumour in the cervical and sacral regions, while the most common diagnosis overall was multiple myeloma and plasmacytoma. Osteosarcoma ranked third. The mean age of presentation was 42 years and pain was the most common presenting symptom, occurring in 95% of malignant and 76% of benign tumours. Neurological involvement occurred in 52% of malignant tumours and usually meant a poor prognosis. The establishment of Bone Tumour Registries is the only way that sufficient data on large numbers of these rare tumours can be accumulated to provide a valuable and otherwise unavailable source of information for research, education and clinical follow-up. (+info)
Sacral chordoma: a case report with radiographic and histologic correlation and a review of the literature.
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A case of sacral chordoma clinically simulating pilonidal 'cyst' in a 47-year-old male is presented. The clinical presentation with radiographic and histologic features of this entity with post-treatment clinical follow up is presented with a review of the literature. (+info)
An intradural skull base chordoma presenting with acute intratumoral hemorrhage.
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We present a rare case of skull base chordoma of extraosseous intradural type that presented as acute intratumoral hemorrhage. Surgical removal of the tumor was accomplished using a skull base approach. (+info)