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1. Jiang L, Liu ZJ, Liu XG, Ma QJ, Wei F, Lv Y, Dang GT: Upper cervical spine chordoma of C2-C3. Eur Spine J; 2009 Mar;18(3):293-298; discussion 298-300
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Upper cervical spine chordoma of C2-C3.
  • Chordoma is a rare low-grade malignant neoplasm derived from the remnants of the embryonic notochord.
  • This locally invasive neoplasm is subject to recurrence after treatment.
  • The median survival time is estimated to be 6.3 years.
  • Various treatment approaches have been attempted, including radical excision, radiotherapy and chemotherapy.
  • Treatment outcome is significantly influenced by the size and site of the chordoma.
  • Recently, Imatinib, a molecular-targeted agent, has been shown to have antitumor activity in chordoma.
  • Surgical treatment is still the primary choice for chordoma.
  • We present and discuss the therapeutic challenges of a young female with large retropharyngeal chordoma who presented to our institution after conventional photon beam radiotherapy.
  • The left vertebral artery (VA) was encapsulated and displaced.
  • [MeSH-major] Cervical Vertebrae / surgery. Chordoma / surgery. Spinal Neoplasms / surgery
  • [MeSH-minor] Adult. Decompression, Surgical / methods. Deglutition Disorders / etiology. Female. Humans. Internal Fixators. Laminectomy / methods. Magnetic Resonance Imaging. Neck Pain / etiology. Neoplasm Invasiveness. Neoplasm Recurrence, Local / prevention & control. Neurosurgical Procedures / instrumentation. Neurosurgical Procedures / methods. Radiotherapy / methods. Radiotherapy / standards. Spinal Fusion / instrumentation. Spinal Fusion / methods. Tomography, X-Ray Computed. Treatment Failure. Treatment Outcome

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  • (PMID = 19221808.001).
  • [ISSN] 1432-0932
  • [Journal-full-title] European spine journal : official publication of the European Spine Society, the European Spinal Deformity Society, and the European Section of the Cervical Spine Research Society
  • [ISO-abbreviation] Eur Spine J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
  • [Other-IDs] NLM/ PMC2899420
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2. Guo W, Xu W, Yang R: [Surgical management of sacrococcygeal chordomas]. Beijing Da Xue Xue Bao; 2003 Apr 18;35(2):159-62

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Surgical management of sacrococcygeal chordomas].
  • OBJECTIVE: To analyze the surgical treatment results of 52 patients with sacral chordoma.
  • METHODS: This retrospective study included 52 cases of sacrococcygeal chordoma surgically treated from December 1996 to July 2001 at the Department of Orthopaedic Surgery, Peking University People's Hospital, The ages of patients ranged from 18 to 80 years (mean 57 years), including of 35 males and 17 females.
  • Nineteen patients had received surgical management at least once and 9 of them had received radiation therapy, whereas the other 33 patients had no surgery before they came to our department.
  • RESULTS: Based on a recent follow-up, 4 patients had died, and 3 of them died of metastatic chordoma.
  • The average follow-up time of the 35 disease-free patients was 42 months.
  • CONCLUSION: Complete resection of tumor (radical surgery when possible) is the most effective way to manage sacrococcygeal chordomas.
  • Postoperative adjuvant radiation therapy can reduce the tumor recurrence rate, but it also can cause troubles that would hinder further surgical managements.
  • One patient who developed metastases in lungs received chemotherapy, and good curative effect had been observed.
  • [MeSH-major] Chordoma / surgery. Spinal Neoplasms / surgery
  • [MeSH-minor] Adolescent. Adult. Aged. Aged, 80 and over. Female. Health Services. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Orthopedic Procedures. Postoperative Complications. Retrospective Studies. Sacrum / pathology. Spinal Nerve Roots. Young Adult

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  • (PMID = 12920833.001).
  • [ISSN] 1671-167X
  • [Journal-full-title] Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences
  • [ISO-abbreviation] Beijing Da Xue Xue Bao
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
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3. Atalar H, Selek H, Yildiz Y, Sağlik Y: Management of sacrococcygeal chordomas. Int Orthop; 2006 Dec;30(6):514-8

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Management of sacrococcygeal chordomas.
  • 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.
  • In five patients, the chordoma was inoperable.
  • The three remaining inoperable patients received radiation therapy.
  • Five of these patients received adjuvant radiotherapy and two patients received both radiotherapy and chemotherapy.
  • Problems encountered during therapy and follow-up included urinary incontinence (72%), rectal incontinence (36%), wound infection (36%), and lower extremity muscle weakness (36%).
  • [MeSH-major] Chordoma / surgery. Sacrum / pathology. Sacrum / surgery. Spinal Neoplasms / surgery
  • [MeSH-minor] Adult. Aged. Female. Follow-Up Studies. Humans. Male. Middle Aged. Neoplasm Recurrence, Local. Radiotherapy, Adjuvant. Treatment Outcome

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  • (PMID = 16552577.001).
  • [ISSN] 0341-2695
  • [Journal-full-title] International orthopaedics
  • [ISO-abbreviation] Int Orthop
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Other-IDs] NLM/ PMC3172743
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4. Montella L, Addeo R, Faiola V, Cennamo G, Guarrasi R, Capasso E, Mamone R, Caraglia M, Del Prete S: Zoledronic acid in metastatic chondrosarcoma and advanced sacrum chordoma: two case reports. J Exp Clin Cancer Res; 2009;28:7
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  • [Title] Zoledronic acid in metastatic chondrosarcoma and advanced sacrum chordoma: two case reports.
  • INTRODUCTION: Chondrosarcomas and chordomas are usually chemoresistant bone tumors and may have a poor prognosis when advanced.
  • PATIENTS AND METHODS: Zoledronic acid was used in a 63-year-old man with metastatic chondrosarcoma and in a 66-year-old woman with a diagnosis of sacrum chordoma both reporting severe pain related to tumor.
  • RESULTS: In the first case, zoledronic acid was able to maintain pain control despite disease progression following chemotherapy, in the other case, zoledronic acid only produced significant clinical benefit.
  • CONCLUSION: Control of pain associated with bone tumors such as chondrosarcoma and chondroma may significantly improve from use of zoledronic acid, independently from tumor response to other treatments.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Chondroma / drug therapy. Chondrosarcoma / drug therapy. Chondrosarcoma / secondary. Diphosphonates / therapeutic use. Imidazoles / therapeutic use. Sacrum / pathology. Spinal Neoplasms / drug therapy
  • [MeSH-minor] Aged. Female. Humans. Male. Middle Aged. Neoplasm Staging. Tomography, X-Ray Computed

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  • (PMID = 19144109.001).
  • [ISSN] 1756-9966
  • [Journal-full-title] Journal of experimental & clinical cancer research : CR
  • [ISO-abbreviation] J. Exp. Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Italy
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Diphosphonates; 0 / Imidazoles; 6XC1PAD3KF / zoledronic acid
  • [Other-IDs] NLM/ PMC2631458
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5. Sciubba DM, Chi JH, Rhines LD, Gokaslan ZL: Chordoma of the spinal column. Neurosurg Clin N Am; 2008 Jan;19(1):5-15
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Chordoma of the spinal column.
  • Chordomas are the most common primary malignant tumor of the mobile spine and of the sacrum.
  • Although considered generally resistant to radiation therapy and chemotherapy, recent advances in photon and proton radiation therapy and use of monoclonal antibodies may provide improved outcomes for poor surgical candidates and for tumors that recur after surgery.
  • [MeSH-major] Chordoma / pathology. Chordoma / surgery. Spinal Neoplasms / pathology. Spinal Neoplasms / surgery. Spine / pathology. Spine / surgery

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  • (PMID = 18156043.001).
  • [ISSN] 1558-1349
  • [Journal-full-title] Neurosurgery clinics of North America
  • [ISO-abbreviation] Neurosurg. Clin. N. Am.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 51
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6. Muro K, Das S, Raizer JJ: Chordomas of the craniospinal axis: multimodality surgical, radiation and medical management strategies. Expert Rev Neurother; 2007 Oct;7(10):1295-312
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Chordomas of the craniospinal axis: multimodality surgical, radiation and medical management strategies.
  • Chordomas are neoplasms of the primitive notochord remnants and are characterized by slow growth kinetics, locally aggressive behavior and resistance to conventional therapeutic options.
  • Chemotherapy plays a limited role and currently remains an option at tumor recurrence, although increasing knowledge of the molecular biology of chordomas may lead to targeted therapeutic strategies.
  • In this review, the current multimodality treatment strategy for chordomas will be discussed and future directions will be highlighted.
  • [MeSH-major] Chordoma / radiotherapy. Chordoma / surgery
  • [MeSH-minor] Animals. Combined Modality Therapy. Humans. Skull Neoplasms / diagnosis. Skull Neoplasms / drug therapy. Skull Neoplasms / radiotherapy. Skull Neoplasms / surgery. Spinal Neoplasms / diagnosis. Spinal Neoplasms / drug therapy. Spinal Neoplasms / radiotherapy. Spinal Neoplasms / surgery

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  • (PMID = 17939768.001).
  • [ISSN] 1744-8360
  • [Journal-full-title] Expert review of neurotherapeutics
  • [ISO-abbreviation] Expert Rev Neurother
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Number-of-references] 125
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7. Guiu S, Guiu B, Feutray S, Chauffert B: Direct intratumoral chemotherapy with carboplatin and epinephrine in a recurrent cervical chordoma: case report. Neurosurgery; 2009 Sep;65(3):E629-30; discussion E630
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Direct intratumoral chemotherapy with carboplatin and epinephrine in a recurrent cervical chordoma: case report.
  • OBJECTIVE: Chordomas are rare primary bone tumors for which surgery is classically the first-line treatment.
  • Because radiation therapy and systemic chemotherapy show limited effectiveness, we report the use of direct intratumoral chemotherapy (IC) to treat recurrent chordoma.
  • CLINICAL PRESENTATION: A 46-year-old man presented with a recurrent cervical chordoma after surgery and radiation therapy.
  • This recurrence manifested as C4-C5 spinal cord compression.
  • Eleven intratumoral treatments were performed during an 18-month period.
  • CONCLUSION: A marked clinical response with regression of the spinal cord compression was observed, without specific toxicity.
  • Thus, in patients with this rare tumor, intratumoral chemotherapy may be a valid treatment option when surgery and radiation therapy fail.
  • Furthermore, intratumoral chemotherapy in combination with surgical treatment should be considered to improve the local control rate.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Carboplatin / therapeutic use. Chordoma / drug therapy. Epinephrine / therapeutic use. Spinal Neoplasms / drug therapy. Sympathomimetics / therapeutic use
  • [MeSH-minor] Combined Modality Therapy. Follow-Up Studies. Humans. Magnetic Resonance Imaging / methods. Male. Middle Aged. Neoplasm Recurrence, Local / diagnosis. Neoplasm Recurrence, Local / drug therapy

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  • (PMID = 19687674.001).
  • [ISSN] 1524-4040
  • [Journal-full-title] Neurosurgery
  • [ISO-abbreviation] Neurosurgery
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Sympathomimetics; BG3F62OND5 / Carboplatin; YKH834O4BH / Epinephrine
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8. Cerda-Olmedo G, De Andrés J, Moliner S: Management of progressive pain in a patient with intramedullary chordoma of the spine. Clin J Pain; 2002 Mar-Apr;18(2):128-31
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Management of progressive pain in a patient with intramedullary chordoma of the spine.
  • OBJECTIVES: The case here presented adequately reflects the difficulties involved in the treatment of pain in patients where the neuropathic component of pain predominates, and shows the different therapeutic steps that may be taken-from surgery and radiotherapy, to the administration of different drugs via the spinal route, to, finally, the presently little-used option of a direct intraventricular access.
  • CONCLUSIONS: Spinal tumors are infrequent, but pose great difficulties for the management and control of the pain they cause.
  • The utility of the spinal route as an early approach for the provision of adequate analgesia seems clear.
  • However, it also appears to lose efficacy with time, and dose incrementing and/or the addition of drugs that enhance the analgesic action of morphine are not always effective.
  • In such selected cases, the intraventricular route may constitute a useful alternative, allowing improved symptoms control with lower morphine doses, and the use of the system previously implanted for intrathecal spinal infusion.
  • [MeSH-major] Bupivacaine / administration & dosage. Chordoma / complications. Morphine / administration & dosage. Pain, Intractable / drug therapy. Spinal Cord Neoplasms / complications
  • [MeSH-minor] Analgesics / administration & dosage. Analgesics / therapeutic use. Anesthetics, Local / administration & dosage. Anesthetics, Local / therapeutic use. Catheters, Indwelling. Clonidine / administration & dosage. Disease Progression. Humans. Injections, Spinal / methods. Lumbosacral Region / surgery. Magnetic Resonance Imaging. Male. Middle Aged

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  • (PMID = 11882777.001).
  • [ISSN] 0749-8047
  • [Journal-full-title] The Clinical journal of pain
  • [ISO-abbreviation] Clin J Pain
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Analgesics; 0 / Anesthetics, Local; 76I7G6D29C / Morphine; MN3L5RMN02 / Clonidine; Y8335394RO / Bupivacaine
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9. Brindza P, Chaloupka R, Grosman R: [Our experience with chordoma treatment]. Acta Chir Orthop Traumatol Cech; 2009 Oct;76(5):382-7
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  • [Title] [Our experience with chordoma treatment].
  • PURPOSE OF THE STUDY: The aim of the study was to evaluate the group of patients operated on for chordoma at our department.
  • We present diagnostic and surgical management options relevant to the localisation of chordoma in the spine, and evaluate the results in terms of post-operative complications, tumour recurrence and patient survival.
  • MATERIAL AND METHODS: Between 1989 and 2002 a total of 20 patients diagnosed with chordoma were treated.
  • The cervical spine was affected in 20 %, thoracic in 15%, lumbar in 25 % and sacral in 40 % of the patients.
  • The average time from the onset of complaints till disease diagnosis was 7.2 months.
  • RESULTS: The average survival time was 63 months, with eight patients (40 %) surviving for more than five years.
  • Of the patients with chordoma of the mobile spine, 66 % were treated by a combined antero-posterior procedure involving somatectomy, vertebral body replacement and posterior stabilisation; for sacral spine chordoma, a dorsal approach was always used.
  • DISCUSSION: Chordomas are rare, slow-growing tumours usually diagnosed with a delay, particularly when localised in the sacral spi- ne.
  • At present magnetic resonance imaging is the essential diagnostic method allowing us to plan the appropriate surgical management.When the mobile spine is affected, a combined antero-posterior procedure including somatectomy, vertebral body replacement with a graft or implant and posterior stabilisation is used.When the sacral spine is involved, some authors prefer en bloc resection from the posterior approach, others use a combined antero-posterior procedure.
  • Chordomas are known to have a high risk of local recurrence.
  • Post-operative radiotherapy, which makes the disease-free interval longer, is recommendedúúú chemotherapy has no effect.
  • CONCLUSIONS: Chordomas are associated with serious diagnostic and therapeutic problems, with frequent local recurrence.
  • [MeSH-major] Chordoma / surgery. Spinal Neoplasms / surgery

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  • (PMID = 19912701.001).
  • [ISSN] 0001-5415
  • [Journal-full-title] Acta chirurgiae orthopaedicae et traumatologiae Cechoslovaca
  • [ISO-abbreviation] Acta Chir Orthop Traumatol Cech
  • [Language] cze
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Czech Republic
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10. McPherson CM, Suki D, McCutcheon IE, Gokaslan ZL, Rhines LD, Mendel E: Metastatic disease from spinal chordoma: a 10-year experience. J Neurosurg Spine; 2006 Oct;5(4):277-80
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metastatic disease from spinal chordoma: a 10-year experience.
  • OBJECT: Metastastic lesions have been reported in 5 to 40% of patients with spinal and sacrococcygeal chordoma, but few contemporary series of chordoma metastastic disease exist in the literature.
  • Additionally, the outcome in patients with chordoma-induced metastastic neoplasms remains unclear.
  • Anderson Cancer Center in Houston to determine the incidence of metastatic disease in a contemporary series of spinal and sacrococcygeal chordoma as well as to determine the outcomes.
  • METHODS: Thirty-seven patients underwent surgery for spinal and sacrococcygeal chordoma between June 1, 1993, and March 31, 2004.
  • All records were reviewed, and appropriate statistical analyses were used to compare patient data for preoperative characteristics, treatments, and outcomes.
  • The authors identified seven patients (19%) in whom metastatic disease developed; in three the disease had metastasized to the lungs only, in two to the lungs and liver, and in two to distant locations in the spine.
  • There were no significant differences in age, sex, tumor location, or history of radiation treatments between patients with and those without metastases.
  • In two (12%) of 17 patients who underwent en bloc resection, metastatic disease developed, whereas it developed in five (25%) of 20 patients treated by curettage (p = 0.42).
  • The median time from first surgery to the appearance of metastatic disease, as calculated using the Kaplan-Meier method, was 143.4 months (95% confidence interval [CI] 66.8-219.9).
  • The median survival duration of patients with metastatic disease after the first surgery was 106 months (95% CI 55.7-155.7), and this did not differ significantly from that in patients in whom no metastases developed (p = 0.93).
  • CONCLUSIONS: Spinal chordoma metastasized to other locations in 19% of the patients in this series.
  • Surgery and chemotherapy can play a role in controlling metastatic disease.
  • [MeSH-major] Chordoma / secondary. Sacrum. Spinal Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Female. Follow-Up Studies. Humans. Male. Middle Aged. Retrospective Studies. Survival Rate. Time Factors. Treatment Outcome

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  • (PMID = 17048762.001).
  • [ISSN] 1547-5654
  • [Journal-full-title] Journal of neurosurgery. Spine
  • [ISO-abbreviation] J Neurosurg Spine
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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11. Rhomberg W, Böhler FK, Novak H, Dertinger S, Breitfellner G: A small prospective study of chordomas treated with radiotherapy and razoxane. Strahlenther Onkol; 2003 Apr;179(4):249-53
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  • [Title] A small prospective study of chordomas treated with radiotherapy and razoxane.
  • PURPOSE: To evaluate the local effect of conventional photon irradiation in chordomas if the radiosensitizing agent razoxane is added.
  • The rationale for this procedure were improved results previously seen in soft tissue and chondrosarcomas with this combination.
  • PATIENTS AND METHODS: Between 1988 and 1996, five patients with histologically confirmed chordomas of the skull base or the spine (three females, two males) were irradiated with 6- and 25-MeV photons under razoxane medication, one patient was treated with a telecobalt unit.
  • Single doses of 180-200 cGy were given five times a week.
  • The median total tumor dose was 63 Gy (range 54-67 Gy).
  • The drug was started 3-5 days before the first irradiation, and continued until the end of radiotherapy.
  • RESULTS: After a potential median follow-up time of 10 years, three of the five patients are alive and show neither symptoms nor signs of recurrence in CT or MR images.
  • One patient with persistent sacral chordoma died after 8 years from cardiac insufficiency, and another patient died after 6.5 years from a bleeding complication following surgery for recurrence.
  • Acute side effects included mucosal reactions, two of five patients developed a leukopenia WHO grade 3 due to razoxane.
  • The cases are unselected, and the follow-up time is of considerable duration.
  • The treatment can easily be performed at any institution and is tolerated fairly well.
  • [MeSH-major] Antineoplastic Agents / therapeutic use. Chordoma / drug therapy. Chordoma / radiotherapy. Immunosuppressive Agents / therapeutic use. Lumbar Vertebrae. Radiation-Sensitizing Agents / therapeutic use. Razoxane / therapeutic use. Sacrum. Skull Base Neoplasms / drug therapy. Skull Base Neoplasms / radiotherapy. Spinal Neoplasms / drug therapy. Spinal Neoplasms / radiotherapy. Thoracic Vertebrae
  • [MeSH-minor] Adolescent. Adult. Aged. Cobalt Radioisotopes / therapeutic use. Combined Modality Therapy. Dose Fractionation. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Photons / therapeutic use. Prospective Studies. Radioisotope Teletherapy. Radiotherapy Dosage. Time Factors. Tomography, X-Ray Computed

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  • (PMID = 12707714.001).
  • [ISSN] 0179-7158
  • [Journal-full-title] Strahlentherapie und Onkologie : Organ der Deutschen Röntgengesellschaft ... [et al]
  • [ISO-abbreviation] Strahlenther Onkol
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Cobalt Radioisotopes; 0 / Immunosuppressive Agents; 0 / Radiation-Sensitizing Agents; 5AR83PR647 / Razoxane
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12. Schick U, Marquardt G: Pediatric spinal tumors. Pediatr Neurosurg; 2001 Sep;35(3):120-7

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pediatric spinal tumors.
  • We present an overview of the clinical outcome and surgical treatment of 34 pediatric spinal tumors, carried out in our center from 1981 to 1999.
  • Extradural tumors included tumors such as aneurysmal bone cysts, Ewing's sarcoma, histiocytomas, chordomas and an angiofibroma.
  • Following decompression of the spinal cord, the neurological status improved with good functional recovery in 23 children and had deteriorated at follow-up in 5 children.
  • Relapse occurred in 12 cases, including histiocytomas, chordomas, medulloblastoma and tumors associated with genetic disorders.
  • Eighteen children underwent a combined treatment with chemotherapy and/or radiotherapy.
  • The spectrum of pathology is wide and requires multidisciplinary treatment.
  • [MeSH-major] Neoplasm Recurrence, Local / surgery. Spinal Neoplasms / surgery
  • [MeSH-minor] Adolescent. Antineoplastic Agents / therapeutic use. Child. Child, Preschool. Combined Modality Therapy. Decompression, Surgical. Female. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Recovery of Function. Retrospective Studies. Treatment Outcome

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  • [Copyright] Copyright 2001 S. Karger AG, Basel
  • (PMID = 11641619.001).
  • [ISSN] 1016-2291
  • [Journal-full-title] Pediatric neurosurgery
  • [ISO-abbreviation] Pediatr Neurosurg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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13. Jena R, Luhana F, Brooke SL, Geater AR, Jefferies SJ, Burton KE, Laing RJ, Burnet NG: Conformal rotation therapy with central axis beam block is a feasible alternative to intensity-modulated radiotherapy for chordomas of the cervical spine. Clin Oncol (R Coll Radiol); 2004 Oct;16(7):449-56
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Conformal rotation therapy with central axis beam block is a feasible alternative to intensity-modulated radiotherapy for chordomas of the cervical spine.
  • AIMS: Paraspinal tumours, such as chordoma, represent a treatment challenge for oncologists, requiring high dose to the target volume without exceeding the tolerance dose of the spinal cord.
  • We present a simpler technique--conformal rotation therapy with a central axis beam block (CRT + BB), which can provide similar dose distributions.
  • MATERIALS AND METHODS: A patient with a cervical chordoma developed postoperative recurrence and was treated with high-dose palliative radiotherapy.
  • Treatment was delivered using CRT + BB, with three fixed beams and three coplanar arcs.
  • A dose of 62 Gy in 31 fractions was delivered to the 100% isodose, giving a maximum spinal cord dose of 49.6 Gy.
  • The patient relapsed 2 years later, and was re-treated using the same technique to a dose of 57 Gy in 30 fractions.
  • Estimates of spinal cord repair rates in primates were used to determine the tolerance dose of the spinal cord for re-treatment.
  • The patient remained well for a further 25 months before developing local recurrence, which was treated with palliative chemotherapy.
  • RESULTS: Re-treatment plans using CRT + BB and IMRT were compared.
  • Dose-volume histograms show equivalence of dose to the spinal cord, although the IMRT plan delivered a slightly higher dose to tumour and lower dose to surrounding soft tissues.
  • CONCLUSION: Treatment using CRT + BB requires careful planning and discussion with neurosurgeons before surgery.
  • The normal curvature of the cervical spine must be eliminated if possible, and the patient must be immobilised with the neck horizontal.
  • If these geometric constraints can be satisfied, then CRT + BB can be used as a safe and effective alternative treatment to IMRT for tumours at this site.
  • [MeSH-major] Cervical Vertebrae / pathology. Chordoma / radiotherapy. Neoplasm Recurrence, Local / prevention & control. Radiotherapy, Conformal / methods. Spinal Cord Neoplasms / radiotherapy

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  • (PMID = 15490805.001).
  • [ISSN] 0936-6555
  • [Journal-full-title] Clinical oncology (Royal College of Radiologists (Great Britain))
  • [ISO-abbreviation] Clin Oncol (R Coll Radiol)
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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14. Lam CH, Nagib MG: Nonteratomatous tumors in the pediatric sacral region. Spine (Phila Pa 1976); 2002 Jun 1;27(11):E284-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • One patient underwent a posterior resection and will undergo a second stage anterior approach later to allow for chemotherapy and radiation to shrink the intrapelvic portion of the tumor.
  • CONCLUSIONS: In contradistinction to adults, in whom chordomas and metastases are the most common primary and secondary tumors, the pediatric group does not have a predominant pathology.
  • Multiple therapeutic methods may be required, including adjuvant chemotherapy and, possibly, embolization.
  • [MeSH-major] Bone Cysts, Aneurysmal / diagnosis. Ependymoma / diagnosis. Ganglioneuroma / diagnosis. Neuroectodermal Tumors, Primitive / diagnosis. Sarcoma, Ewing / diagnosis. Spinal Neoplasms / diagnosis
  • [MeSH-minor] Back Pain / etiology. Child. Coccyx / pathology. Constipation / etiology. Female. Gait Disorders, Neurologic / etiology. Humans. Lung Neoplasms / diagnosis. Lung Neoplasms / secondary. Male. Retrospective Studies. Sacrococcygeal Region. Sacrum / pathology. Treatment Outcome

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  • (PMID = 12045531.001).
  • [ISSN] 1528-1159
  • [Journal-full-title] Spine
  • [ISO-abbreviation] Spine
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 12
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15. Hof H, Welzel T, Debus J: Effectiveness of cetuximab/gefitinib in the therapy of a sacral chordoma. Onkologie; 2006 Dec;29(12):572-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Effectiveness of cetuximab/gefitinib in the therapy of a sacral chordoma.
  • BACKGROUND: Chordomas are rare tumors of the skull base and the spine.
  • Treatment is difficult since conventional radiotherapy or chemotherapy have only limited effects.
  • PATIENT AND METHODS: A patient with a sacral chordoma and pulmonary metastases received initial surgery and radiotherapy for a local recurrence.
  • As tumor biopsies revealed the expression of the EGF receptor an individual treatment approach with a combination of cetuximab and gefitinib was performed.
  • RESULTS: Under the treatment with cetuximab/gefitinib the local recurrence and the pulmonary metastases showed a partial response over the follow-up period of 9 months.
  • To date no treatment failure was observed.
  • CONCLUSIONS: The inhibition of the EGF pathway seems to be an effective measure in the treatment of a chordoma.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / administration & dosage. Chordoma / drug therapy. Neoplasm Recurrence, Local / drug therapy. Sacrum. Spinal Neoplasms / drug therapy
  • [MeSH-minor] Antibodies, Monoclonal / administration & dosage. Antibodies, Monoclonal, Humanized. Cetuximab. Humans. Male. Middle Aged. Quinazolines / administration & dosage. Treatment Outcome

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  • (PMID = 17202828.001).
  • [ISSN] 0378-584X
  • [Journal-full-title] Onkologie
  • [ISO-abbreviation] Onkologie
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Switzerland
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Quinazolines; PQX0D8J21J / Cetuximab; S65743JHBS / gefitinib
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16. Sciubba DM, Cheng JJ, Petteys RJ, Weber KL, Frassica DA, Gokaslan ZL: Chordoma of the sacrum and vertebral bodies. J Am Acad Orthop Surg; 2009 Nov;17(11):708-17
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Chordoma of the sacrum and vertebral bodies.
  • Chordomas are relatively rare tumors of bone.
  • These primary malignant lesions occur throughout the spinal column and often show advanced growth at the time of diagnosis.
  • Because such tumors are minimally responsive to radiation and chemotherapy, surgical resection is the mainstay of treatment.
  • However, surgical morbidity may be substantial given the propensity for chordomas to abut or surround neural, vascular, and visceral structures.
  • Thus, early recognition is essential, and treatment by a multidisciplinary team is ideal.
  • [MeSH-major] Cervical Vertebrae. Chondroma / diagnosis. Chondroma / therapy. Sacrum. Spinal Neoplasms / diagnosis. Spinal Neoplasms / therapy. Thoracic Vertebrae
  • [MeSH-minor] Combined Modality Therapy / methods. Diagnosis, Differential. Humans. Magnetic Resonance Imaging

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  • (PMID = 19880681.001).
  • [ISSN] 1067-151X
  • [Journal-full-title] The Journal of the American Academy of Orthopaedic Surgeons
  • [ISO-abbreviation] J Am Acad Orthop Surg
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 46
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17. Munshi HG, Merajver SD, Valdez R, Baker LH, Cooney KA: Metastatic dedifferentiated chordoma with elevated beta-hCG: a case report. Am J Clin Oncol; 2002 Jun;25(3):274-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metastatic dedifferentiated chordoma with elevated beta-hCG: a case report.
  • Chordomas are relatively uncommon bone tumors, and fewer than 10% of cases are classified as dedifferentiated chordomas.
  • These tumors tend to be more clinically aggressive than chordomas and have a higher incidence of early distant metastases.
  • In this case report, we describe a 24-year-old man with dedifferentiated chordoma and multiple pulmonary metastases.
  • Unfortunately, the patient's clinical condition deteriorated rapidly and he died before receiving any treatment for his cancer.
  • However, chemotherapy may play a useful role in the management of systemic disease in patients with dedifferentiated chordoma.
  • [MeSH-major] Chordoma / blood. Chordoma / secondary. Chorionic Gonadotropin, beta Subunit, Human / blood. Spinal Neoplasms / blood. Spinal Neoplasms / pathology

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  • (PMID = 12040287.001).
  • [ISSN] 0277-3732
  • [Journal-full-title] American journal of clinical oncology
  • [ISO-abbreviation] Am. J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chorionic Gonadotropin, beta Subunit, Human
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18. Yang C, Hornicek FJ, Wood KB, Schwab JH, Choy E, Mankin H, Duan Z: Blockage of Stat3 with CDDO-Me inhibits tumor cell growth in chordoma. Spine (Phila Pa 1976); 2010 Aug 15;35(18):1668-75
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Blockage of Stat3 with CDDO-Me inhibits tumor cell growth in chordoma.
  • STUDY DESIGN: An experimental study to investigate the activation of Src/Stat3 pathways in chordomas and blockage of this pathway as a potential strategy for chordoma treatment.
  • OBJECTIVE: To investigate the activation of Src/Stat3 pathway in chordomas cells and to determine the efficiency of inhibiting this pathway by 2-cyano-3,12-dioxooleana-1,9 (11)-dien-28-oic acid-methyl ester (CDDO-Me) as a potential chemotherapeutic agent for chordoma treatment.
  • SUMMARY OF BACKGROUND DATA: The advent of molecularly targeted therapies has raised interest for their use in the treatment of chordomas.
  • Unfortunately, the current understanding of molecular markers in chordomas is limited.
  • The function of Stat3 pathway in chordomas has not been elucidated.
  • METHODS: The expression of key components of the Src/Stat3 signaling cascade was evaluated by Western blot in chordoma tissues and chordoma cell lines.
  • The effects of CDDO-Me on chordoma cell growth were evaluated in these chordoma cell lines by MTT assay.
  • Finally, chordoma cells were grown in a 3-dimensional (3D) culture and treated with CDDO-Me.
  • RESULTS: The key components of the Src/Stat3 signaling cascade, including Stat3, pStat3, Src, pSrc, Bcl-xL, and Myeloid Cell Leukemia-1, were all highly expressed in chordomas.
  • Expression of the key components of the Src/Stat3 signaling cascade was inhibited in chordoma cells after treatment with CDDO-Me.
  • The growth of chordoma cells was inhibited and apoptosis associated poly (ADP-ribose) polymerase cleavage was detected after treatment with CDDO-Me.
  • Additionally, CDDO-Me has a synergistic effect on cisplatin or doxorubicin-induced chordoma cell death (P < 0.001).
  • Finally, expression of pSrc and pStat3 and chordoma cell growth was inhibited by treatment of CDDO-Me using 3D culture.
  • CONCLUSION: The Src/Stat3 signaling pathway was activated in chordomas.
  • Blockage of Src/Stat3 pathway by CDDO-Me is a potential strategy for chordoma treatment and may be focus for future research.
  • [MeSH-major] Chordoma / drug therapy. Chordoma / pathology. Growth Inhibitors / therapeutic use. Oleanolic Acid / analogs & derivatives. STAT3 Transcription Factor / antagonists & inhibitors. Spinal Neoplasms / drug therapy. Spinal Neoplasms / pathology
  • [MeSH-minor] Antineoplastic Agents, Phytogenic / therapeutic use. Humans. Organ Culture Techniques

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  • (PMID = 20386502.001).
  • [ISSN] 1528-1159
  • [Journal-full-title] Spine
  • [ISO-abbreviation] Spine
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / R01-CA119617
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 0 / Growth Inhibitors; 0 / STAT3 Transcription Factor; 0 / STAT3 protein, human; 6SMK8R7TGJ / Oleanolic Acid; CEG1Q6OGU1 / methyl 2-cyano-3,12-dioxoolean-1,9-dien-28-oate
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19. Rutz HP, Weber DC, Goitein G, Ares C, Bolsi A, Lomax AJ, Pedroni E, Coray A, Hug EB, Timmermann B: Postoperative spot-scanning proton radiation therapy for chordoma and chondrosarcoma in children and adolescents: initial experience at paul scherrer institute. Int J Radiat Oncol Biol Phys; 2008 May 1;71(1):220-5
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Postoperative spot-scanning proton radiation therapy for chordoma and chondrosarcoma in children and adolescents: initial experience at paul scherrer institute.
  • PURPOSE: To evaluate postoperative spot-scanning proton radiation therapy (PT) and intensity-modulated PT (IMPT) for chordoma and chondrosarcoma in pediatric patients.
  • METHODS AND MATERIALS: Between 2000 and 2005, 10 patients (six male patients, four female patients; six chordomas, four chondrosarcomas), aged 10-20 years (median, 16 years), were treated at our institute.
  • Tumor sites were in the brain (one case), skull base (five cases), cervical (three cases), and lumbar spine (one case).
  • Total dose was 74.0 cobalt Gray equivalents (CGE) for chordoma, and 63.2-68.0 CGE for chondrosarcoma (median, 66.0), depending on histopathological grading and whether the patient had concurrent chemotherapy.
  • RESULTS: Median follow-up time was 36 months (range, 8-77 months).
  • Radiation treatment was well tolerated.
  • CONCLUSIONS: Postoperative spot-scanning PT, delivered in combination with and without IMPT, for chordoma and chondrosarcoma in children and adolescents was tolerated without unacceptable adverse event and initial outcome is perfectly satisfactory in this small cohort.
  • Longer follow-up time and larger cohort are needed to more fully assess tumor control, adverse events, as well as functional and cosmetic outcome.
  • [MeSH-major] Chondrosarcoma / radiotherapy. Chordoma / radiotherapy. Protons / therapeutic use
  • [MeSH-minor] Adolescent. Adult. Brain Neoplasms / radiotherapy. Brain Neoplasms / surgery. Child. Female. Head and Neck Neoplasms / radiotherapy. Head and Neck Neoplasms / surgery. Humans. Male. Neoplasm Recurrence, Local / radiotherapy. Postoperative Period. Radiation Injuries / complications. Radiotherapy, Intensity-Modulated. Skull Base Neoplasms / radiotherapy. Skull Base Neoplasms / surgery. Spinal Neoplasms / radiotherapy. Spinal Neoplasms / surgery

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  • (PMID = 18068310.001).
  • [ISSN] 0360-3016
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Protons
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20. Stacchiotti S, Marrari A, Tamborini E, Palassini E, Virdis E, Messina A, Crippa F, Morosi C, Gronchi A, Pilotti S, Casali PG: Response to imatinib plus sirolimus in advanced chordoma. Ann Oncol; 2009 Nov;20(11):1886-94
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Response to imatinib plus sirolimus in advanced chordoma.
  • BACKGROUND: Imatinib (IM) is active in advanced chordoma.
  • The evidence of upstream and/or downstream mammalian target of rapamycin (mTOR) pathway activation prompted us to combine an mTOR inhibitor, sirolimus, to IM in IM-resistant advanced chordoma.
  • PATIENTS AND METHODS: Since July 2007, 10 progressive advanced chordoma patients with secondary resistance to IM, and biochemical and/or immunohistochemical evidence of upstream and/or downstream mTOR effector activation, started IM (400 mg/day) plus sirolimus (2 mg/day) on a named basis.
  • RESULTS: The mean treatment duration was 9 months.
  • Seven patients had a positron emission tomography response.
  • Post-treatment biopsy in one responsive patient confirmed S6 switch off.
  • CONCLUSION: In addition to PDGFRB, mTOR pathway can be activated in chordomas and the combination of IM plus rapalogs may be effective in IM-resistant chordomas.

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  • (PMID = 19570961.001).
  • [ISSN] 1569-8041
  • [Journal-full-title] Annals of oncology : official journal of the European Society for Medical Oncology
  • [ISO-abbreviation] Ann. Oncol.
  • [Language] ENG
  • [Publication-type] Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Benzamides; 0 / Piperazines; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.11.1 / Oncogene Protein v-akt; W36ZG6FT64 / Sirolimus
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