Cases reported "Chordoma"

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1/17. Total spondylectomy for primary tumor of the thoracolumbar spine.

    STUDY DESIGN: Six patients with primary malignant tumor of the thoracolumbar spine who underwent total spondylectomy (TS) by en bloc resection were reviewed retrospectively. OBJECTIVES: To report surgical technique and preliminary results of TS and to evaluate its oncological curability. SETTING: japan. methods: Six patients were treated by TS by en bloc resection of the vertebral tumor. TS through a posterior approach was performed in three cases (T1 osteosarcoma, L1 osteosarcoma and L1 chordoma) and in the others through a single stage anterior and posterior combined approach (T6-8 recurrent giant cell tumor. L4 chordoma and L5 giant cell tumor). Surgical margins of the specimens were evaluated histologically. All patients were followed, and their status was evaluated by clinical and imaging studies. RESULTS: There were no complications related to surgery. Programmed sacrifice of nerve roots were performed in three cases for oncologic excision. A wide surgical margin was achieved in one case, a marginal one in four, and an intralesional margin in one. Five patients were alive without evidence of tumor and one was alive with disease at follow-up evaluation after 2.0-4.8 years. Local recurrence was found in one case of T1 osteosarcoma with an intralesional margin. CONCLUSIONS: These preliminary results suggested that TS is an effective procedure in control of local recurrence with acceptable complications.
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2/17. role of autologous blood transfusion in sacral tumor resection: patient selection and recovery after surgery and blood donation.

    We carried out sacral en-bloc resection in six patients (three with chordoma; one with pheochromocytoma; one with malignant schwannoma; and one with giant cell tumor) using preoperatively collected autologous blood, to avoid homologous blood transfusion. An average of 3200 ml was collected preoperatively, with patients receiving recombinant human erythropoietin (r-HuEPO), at a total dose of 130 000 units on average. In four patients, we were able to accomplish the surgery without homologous blood transfusion. Postoperatively, the hemoglobin level in these four patients recovered to the pre-collective level in 4.5 weeks, on average. These clinical results indicate that en-bloc sacrectomy, which requires a large volume of blood transfusion, can be accomplished with preoperatively collected autologous blood alone.
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3/17. Giant notochordal hamartoma of intraosseous origin: a newly reported benign entity to be distinguished from chordoma. Report of two cases.

    Two cases are reported of a newly described intraosseous entity of vertebral bodies deemed "giant notochordal hamartoma of intraosseous origin". This entity is commonly mistaken for chordoma and must be distinguished from it as the consequences of misinterpretation may be serious. The clinical, radiological and histologic criteria that can be used to distinguish these two entities are emphasized. Included is a proposed pathogenesis for this lesion, its probable notochordal origin, and a review of other probable cases.
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4/17. Giant mediastinal chordoma.

    A chordoma is a slow-growing tumor representing about 5% of all malignant bone tumors. Mediastinal chordoma is very rare. We report a giant thoracic chordoma in a 32-year-old woman who presented with chest pain, progressive dyspnea, and cough. Open biopsy confirmed a definitive preoperative diagnosis, and complete surgical excision of the tumor was accomplished.
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5/17. Total sacrectomy and reconstruction for sacral tumors.

    STUDY DESIGN: Report of three patients in whom the lumbosacral junctions were successfully restored by spinal instrumentations after total sacrectomies. OBJECTIVES: To describe the surgical technique of the reconstruction of the continuity between the pelvic ring and spinal column by using a transpedicular and iliac screw system. SUMMARY OF BACKGROUND DATA: Although there have been case reports about reconstruction methods after total sacrectomy, biomechanical, and technical problems still remain unresolved. methods: Total sacrectomy was carried out in three cases: two with chordomas and one with a recurrent giant cell tumor. In the first case, reconstruction was achieved with Zielke transpedicular screw and rod system and a sacral rod. The other two patients were reconstructed using a transpedicular and iliac screw system and a sacral rod for bilateral fixation of the iliac wings. In the third patient, the vertical rods were connected to transverse rod with rod connectors. RESULTS: No instrumentation failure was observed, and the continuity between the pelvic wing and spinal column was established with the instrumentation and bone grafting. Although one patient died of metastatic chordoma, the lumbosacral junction was successfully reconstructed with the instrumentation. The other two patients could stand with double crutches 13 and 2 years after surgery, respectively. CONCLUSIONS: Total sacrectomy is a feasible operation for primary malignant tumors involving the entire sacrum. Reconstruction of the union between the lumbar spine and the ilia with spinal instrumentation achieves stabilization suitable for ambulation.
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6/17. Chondroid chordoma of the L5 spinous process and lamina: a case report.

    chordoma is a rare bone tumor that originates from the remnants of the notochord. These tumors have axial distribution particularly at the upper and lower ends of the vertebral column. This paper reports a rare occurrence of a chordoma in the posterior elements of the L5 vertebra. A differential diagnosis of a benign tumor (giant cell tumor, aneurysmal bone cyst or osteoblastoma) was made initially. Other differential diagnoses included plasmacytoma and metastasis. The tumor was removed enbloc. Histopathological examination revealed the tumor mass to be chordoma. There were no clinical or radiological signs of recurrence at 21 months follow-up. Chordomas are tumors of the axial skeleton. However, they may occur in unusual sites in ectopic notochordal tissue. The case is being presented for its unusual site of occurrence.
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7/17. Spinal pelvic reconstruction after total sacrectomy for en bloc resection of a giant sacral chordoma. Technical note.

    Although radical resection prolongs the disease-free survival period, surgical management of primary sacral tumors is challenging because of their location and often large size. Moreover, in cases of lesions for which a radical resection necessitates total sacrectomy, reconstruction is required. The authors have previously described a modified Galveston technique in which a liaison between the spine and pelvis is achieved using lumbar pedicle screws and Galveston rods embedded into the ilia; additionally, a transiliac bar reestablishes the pelvic ring. Although this reconstruction technique achieves stabilization, several biomechanical limitations exist. In the present report the authors present the case of a patient who underwent spinal pelvic reconstruction after a total sacrectomy was performed to remove a giant sacral chordoma. They describe a novel spinal pelvic reconstruction technique that addresses some of the biomechanical limitations.
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8/17. Infantile clivus chordoma without clivus involvement: case report and review of the literature.

    BACKGROUND: We present a giant clival chordoma with disseminated disease but without involvement of the clivus. To our knowledge, this is the youngest child and only the second case, presenting without base of skull involvement, in paediatric literature and the fourth reported case of a chordoma in a patient with tuberous sclerosis. DISCUSSION: We discuss the subtle presentation, difficulties in diagnosis and management and also review the literature.
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9/17. Transoral transpalatal removal of a giant premesencephalic clivus chordoma.

    Due to their surgical inaccessibility and resistance to radiotherapy, clivus chordomas represent a formidable therapeutic challenge. The transoral approach to chordomas of the clivus has been usually restricted to relatively small or midsized neoplasms, located at the lower end of the clivus or at the anterior clival-cervical junction. In this report the transoral transpalatal transclival removal of a giant recurrent chordoma occupying the whole length of the clivus with considerable premesencephalic extension and brain stem compression is described. Regression of preoperative symptoms without additional postoperative morbidity could be achieved by radical transoral tumour extirpation documented by magnetic resonance imaging.
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10/17. Total sacrectomy and reconstruction for huge sacral tumors.

    The authors carried out successful total sacrectomy in three cases, two with giant cell tumors and one with a chordoma. The anterior and posterior approach is feasible for resecting huge sacral tumors en bloc, but it is important to reconstruct the continuity between the pelvic ring and spinal column using spinal instrumentation and sacral rods or AO plates. As total sacrectomy is a large-scale, time-consuming, and collaborative operation, two or three teams should be used in relays. Both pelvic and spinal surgical techniques are required. Post-operatively the patient can stand within 3 to 6 months and well-planned rehabilitation allows ambulation. In spite of the serious structural and neurologic damage caused, total sacrectomy can be rewarding procedure in terms of improved morbidity and mortality.
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