Back and Neck
Posted on: Aug 16
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Tags: spinal, chordoma, surgery, neurology, article

Treating Spinal Chordoma with a Multidisciplinary Approach for Superior Outcomes

Many patients with vertebral chordomas present with progressive neck or back pain. Because of neurological symptoms from spinal cord impingement, surgical intervention is commonly the first step in managing a chordoma, with total tumor resection and high dose proton therapy associated with superior outcomes.

Understanding spinal chordomas

Chordomas, while exceedingly rare, are locally aggressive primary bone tumors that require complex, coordinated care. At Indiana University Health, physicians take a multidisciplinary approach to treatment of chordomas to achieve the best possible outcomes for patients.

Believed to develop from undifferentiated remnants of the embryonic notochord, chordomas may arise anywhere along the vertebral column, from the clivus to the coccyx. Chordomas of the mobile spine have distinct treatment considerations from tumors of the clivus and sacrum. A soft tissue component is often present and may invade the paravertebral musculature. Although commonly described as benign, chordomas have malignant potential, and nodal metastases and hematogenous dissemination may develop in more than one-third of patients.1

Complete surgical resection for superior tumor control

En bloc surgical resection is preferred when technically possible. Referral to a spine surgeon with expertise in oncologic surgery and complex spinal reconstruction maximizes the chance of complete tumor removal. Care must be taken to reduce the risk of iatrogenic tumor seeding in the route of surgical access.2 A staged surgical procedure is often required to achieve complete tumor resection and spine stabilization. The use of non-metallic spinal stabilization hardware, such as PEEK, reduces or eliminates CT and MRI artifacts to improve the quality of follow-up imaging and to facilitate delivery of postoperative radiation. In patients without impending neurological compromise, preoperative radiotherapy should be considered, which allows a smaller area to be irradiated and may reduce the risk of tumor seeding during subsequent resection.

Extent of resection is a key prognostic factor for disease control. Patients for whom a gross total resection can be achieved have superior outcomes.3 When complete tumor removal is not possible, maximal debulking remains essential as patients with minimal residual disease may be controlled with high dose proton therapy, but those with substantial residual gross disease have inferior outcomes.4

Advantages of proton therapy as a complementary treatment

Even in patients with an apparent gross total resection, the risk of local recurrence is high.5 Adjuvant radiotherapy with x-rays has not markedly improved the disease control as chordomas are extremely radioresistant. The close proximity of the spinal cord, esophagus, kidneys and bowel limit the ability of x-ray based radiation modalities to safely deliver higher treatment doses for vertebral chordomas.

Proton therapy is a modality of radiation that provides two advantages for treatment of chordomas. The first is the opportunity to safely provide dose escalation to doses of 75-80 Gy to these radioresistant tumors, improving the chance of local control compared to x-ray based radiation modalities. The second advantage is avoidance of unnecessary radiation dose to viscera anterior to the spine, including the bowel, eliminating the nausea, emesis and diarrhea that are otherwise potential side effects of spinal irradiation, and reducing irradiation of the esophagus in treatment of cervical chordomas to limitthe severity of odynophagia. Both of these advantages are possible due to a unique physical property of proton therapy, known as the Bragg peak. This allows the radiation dose to penetrate in to the depth of the target and abruptly terminate, sparing all tissues beyond that point from unnecessary exit dose radiation.6 The advantages of proton therapy for chordomas are borne out in numerous published series of patients with both cranial and extracranial chordomas, showing tumor control superior to results seen in patients treated with x-rays.7,8

1Walcott BP, Nahed BV, Mohyeldin A, et al. Chordoma: current concepts, management, and future directions. Lancet Oncol 2012;13:e69-76.
2Arnautovic KI, Al-Mefty O. Surgical seeding of chordomas. J Neurosurg 2001;95:798-803.
3Stacchiotti S, Casali PG, Lo Vullo S, et al. Chordoma of the mobile spine and sacrum: a retrospective analysis of a series of patients surgically treated at two referral centers. Ann Surg Oncol 2010;17:211-219.
4Staab A, Rutz HP, Ares C, et al. Spot-scanning-based proton therapy for extracranial chordoma. Int J Radiat Oncol Biol Phys 2011;81:e489-496.
5Tzortzidis F, Elahi F, Wright D, et al. Patient outcome at long-term follow-up after aggressive microsurgical microsurgical resection of cranial base chordomas. Neurosurgery 2006;59:230-237.
6McDonald MW, Fitzek MM. Proton Therapy. Curr Probl Cancer 2010;34:257-296.
7Staab A, Rutz HP, Ares C, et al. Spot-scanning-based proton therapy for extracranial chordoma. Int J Radiat Oncol Biol Phys 2011;81:e489-496.
8Ares C, Hug EB, Lomax AJ, et al. Effectiveness and safety of spot scanning proton radiation therapy for chordomas and chondrosarcomas of the skull base: first long-term report. Int J Radiat Oncol Biol Phys 2009;75:1111-1118.

Case Study

Case study of a patient presenting with progressive back pain and found to have a tumor of the thoracic spine at T11. A biopsy diagnosed chordoma and the patient came to IU Health for treatment. 

On T2 weighted MRI, the tumor (*) is hyperintense and compressing the spinal cord. A complete T11 vertebrectomy was performed in a stage procedure and a non-metallic PEEK expandable cage was placed in consideration of planned proton therapy. 

Postoperative MRI confirmed a gross total resection of the tumor. 

A CT myelogram was performed in the radiation treatment positioning devices to precisely delineate the position of the spinal cord. The PEEK cage (arrow on axial image) causes no imaging artifact or dose attenuation in the critical area of the resection, unlike the streak artifact seen by metal screws (arrow on sagittal image).

The proton dose distribution is shown, covering the tumor bed to 79.2 Gy (RBE) and approximating a minimum of 74 Gy (RBE) against the thecal sac while keeping the surface of the spinal cord below 60 Gy (RBE). The patient is more than a year out from therapy and has no evidence of recurrence and no toxicities from therapy. Images courtesy of Mark McDonald, MD.

Mark McDonald, MD

Mark McDonald, MD, is an assistant professor of radiation oncology at Indiana University School of Medicine and has been a radiation oncologist at the Indiana University Health Proton Therapy Center since 2009, where he treats adult patients with tumors of the brain and spine, and head and neck cancers. He is a member of the American College of Radiology Appropriateness Criteria Expert Panel on Head and Neck Cancer, and past member of the Appropriateness Criteria Expert Panel for Brain Metastases. Dr. McDonald is a graduate of The Ohio State University College of Medicine and Public Health, and he completed his internship and residency training at Emory University School of Medicine in Atlanta.

Richard Rodgers, MD

After earning his medical degree from Indiana University School of Medicine, Richard Rodgers, MD, completed a general surgery internship and neurological surgery residency at Indiana University School of Medicine, and a neuro-trauma and neuro-critical care fellowship from the University of Miami Miller School of Medicine.

Dr. Rodgers’ clinical interests include disorders of the spine (including complex degenerative spine disorders, spine trauma and spinal cord injury, tumors and spinal instrumentation), with a special focus on minimally invasive techniques. He is also the director of neuro-trauma and neuro-critical care for the Indiana University Department of Neurological Surgery. He is currently involved in the American Medical Association, Indiana State Medical Association, Indianapolis Medical Society, American Association of Neurological Surgeons, Congress of Neurological Surgeons, AANS/CNS Joint Section on Neuro-trauma and Critical Care, AANS/CNS Joint Section on Disorders of the Spine and Peripheral Nerves, Neuro-critical Care Society, and the Neurosurgical Society of Indiana.

Tags: spinal, chordoma, surgery, neurology, article
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Treating Spinal Chordoma with a Multidisciplinary Approach for Superior Outcomes

Many patients with vertebral chordomas present with progressive neck or back pain. Because of neurological symptoms from spinal cord impingement, surgical intervention is commonly the first step in managing a chordoma, with total tumor resection and high dose proton therapy associated with superior outcomes.

Understanding spinal chordomas

Chordomas, while exceedingly rare, are locally aggressive primary bone tumors that require complex, coordinated care. At Indiana University Health, physicians take a multidisciplinary approach to treatment of chordomas to achieve the best possible outcomes for patients.

Believed to develop from undifferentiated remnants of the embryonic notochord, chordomas may arise anywhere along the vertebral column, from the clivus to the coccyx. Chordomas of the mobile spine have distinct treatment considerations from tumors of the clivus and sacrum. A soft tissue component is often present and may invade the paravertebral musculature. Although commonly described as benign, chordomas have malignant potential, and nodal metastases and hematogenous dissemination may develop in more than one-third of patients.1

Complete surgical resection for superior tumor control

En bloc surgical resection is preferred when technically possible. Referral to a spine surgeon with expertise in oncologic surgery and complex spinal reconstruction maximizes the chance of complete tumor removal. Care must be taken to reduce the risk of iatrogenic tumor seeding in the route of surgical access.2 A staged surgical procedure is often required to achieve complete tumor resection and spine stabilization. The use of non-metallic spinal stabilization hardware, such as PEEK, reduces or eliminates CT and MRI artifacts to improve the quality of follow-up imaging and to facilitate delivery of postoperative radiation. In patients without impending neurological compromise, preoperative radiotherapy should be considered, which allows a smaller area to be irradiated and may reduce the risk of tumor seeding during subsequent resection.

Extent of resection is a key prognostic factor for disease control. Patients for whom a gross total resection can be achieved have superior outcomes.3 When complete tumor removal is not possible, maximal debulking remains essential as patients with minimal residual disease may be controlled with high dose proton therapy, but those with substantial residual gross disease have inferior outcomes.4

Advantages of proton therapy as a complementary treatment

Even in patients with an apparent gross total resection, the risk of local recurrence is high.5 Adjuvant radiotherapy with x-rays has not markedly improved the disease control as chordomas are extremely radioresistant. The close proximity of the spinal cord, esophagus, kidneys and bowel limit the ability of x-ray based radiation modalities to safely deliver higher treatment doses for vertebral chordomas.

Proton therapy is a modality of radiation that provides two advantages for treatment of chordomas. The first is the opportunity to safely provide dose escalation to doses of 75-80 Gy to these radioresistant tumors, improving the chance of local control compared to x-ray based radiation modalities. The second advantage is avoidance of unnecessary radiation dose to viscera anterior to the spine, including the bowel, eliminating the nausea, emesis and diarrhea that are otherwise potential side effects of spinal irradiation, and reducing irradiation of the esophagus in treatment of cervical chordomas to limitthe severity of odynophagia. Both of these advantages are possible due to a unique physical property of proton therapy, known as the Bragg peak. This allows the radiation dose to penetrate in to the depth of the target and abruptly terminate, sparing all tissues beyond that point from unnecessary exit dose radiation.6 The advantages of proton therapy for chordomas are borne out in numerous published series of patients with both cranial and extracranial chordomas, showing tumor control superior to results seen in patients treated with x-rays.7,8

1Walcott BP, Nahed BV, Mohyeldin A, et al. Chordoma: current concepts, management, and future directions. Lancet Oncol 2012;13:e69-76.
2Arnautovic KI, Al-Mefty O. Surgical seeding of chordomas. J Neurosurg 2001;95:798-803.
3Stacchiotti S, Casali PG, Lo Vullo S, et al. Chordoma of the mobile spine and sacrum: a retrospective analysis of a series of patients surgically treated at two referral centers. Ann Surg Oncol 2010;17:211-219.
4Staab A, Rutz HP, Ares C, et al. Spot-scanning-based proton therapy for extracranial chordoma. Int J Radiat Oncol Biol Phys 2011;81:e489-496.
5Tzortzidis F, Elahi F, Wright D, et al. Patient outcome at long-term follow-up after aggressive microsurgical microsurgical resection of cranial base chordomas. Neurosurgery 2006;59:230-237.
6McDonald MW, Fitzek MM. Proton Therapy. Curr Probl Cancer 2010;34:257-296.
7Staab A, Rutz HP, Ares C, et al. Spot-scanning-based proton therapy for extracranial chordoma. Int J Radiat Oncol Biol Phys 2011;81:e489-496.
8Ares C, Hug EB, Lomax AJ, et al. Effectiveness and safety of spot scanning proton radiation therapy for chordomas and chondrosarcomas of the skull base: first long-term report. Int J Radiat Oncol Biol Phys 2009;75:1111-1118.

Case Study

Case study of a patient presenting with progressive back pain and found to have a tumor of the thoracic spine at T11. A biopsy diagnosed chordoma and the patient came to IU Health for treatment. 

On T2 weighted MRI, the tumor (*) is hyperintense and compressing the spinal cord. A complete T11 vertebrectomy was performed in a stage procedure and a non-metallic PEEK expandable cage was placed in consideration of planned proton therapy. 

Postoperative MRI confirmed a gross total resection of the tumor. 

A CT myelogram was performed in the radiation treatment positioning devices to precisely delineate the position of the spinal cord. The PEEK cage (arrow on axial image) causes no imaging artifact or dose attenuation in the critical area of the resection, unlike the streak artifact seen by metal screws (arrow on sagittal image).

The proton dose distribution is shown, covering the tumor bed to 79.2 Gy (RBE) and approximating a minimum of 74 Gy (RBE) against the thecal sac while keeping the surface of the spinal cord below 60 Gy (RBE). The patient is more than a year out from therapy and has no evidence of recurrence and no toxicities from therapy. Images courtesy of Mark McDonald, MD.

Mark McDonald, MD

Mark McDonald, MD, is an assistant professor of radiation oncology at Indiana University School of Medicine and has been a radiation oncologist at the Indiana University Health Proton Therapy Center since 2009, where he treats adult patients with tumors of the brain and spine, and head and neck cancers. He is a member of the American College of Radiology Appropriateness Criteria Expert Panel on Head and Neck Cancer, and past member of the Appropriateness Criteria Expert Panel for Brain Metastases. Dr. McDonald is a graduate of The Ohio State University College of Medicine and Public Health, and he completed his internship and residency training at Emory University School of Medicine in Atlanta.

Richard Rodgers, MD

After earning his medical degree from Indiana University School of Medicine, Richard Rodgers, MD, completed a general surgery internship and neurological surgery residency at Indiana University School of Medicine, and a neuro-trauma and neuro-critical care fellowship from the University of Miami Miller School of Medicine.

Dr. Rodgers’ clinical interests include disorders of the spine (including complex degenerative spine disorders, spine trauma and spinal cord injury, tumors and spinal instrumentation), with a special focus on minimally invasive techniques. He is also the director of neuro-trauma and neuro-critical care for the Indiana University Department of Neurological Surgery. He is currently involved in the American Medical Association, Indiana State Medical Association, Indianapolis Medical Society, American Association of Neurological Surgeons, Congress of Neurological Surgeons, AANS/CNS Joint Section on Neuro-trauma and Critical Care, AANS/CNS Joint Section on Disorders of the Spine and Peripheral Nerves, Neuro-critical Care Society, and the Neurosurgical Society of Indiana.

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