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Primary non-lymphoproliferative tumours of the spine are uncommon and make up less than 5% of bone neoplasms. Metastatic disease of the spine is much more common.Approximately 40-80% of patients who die from cancer have bony metastases at the time of death, with the spine being the most common metastatic skeletal location. Problems can be caused by direct compression on the spinal cord, vascular obstruction and invasive infiltration.
In children, the common benign tumours include osteoid osteomas and osteoblastoma.
Primary spinal tumours
Bone-producing tumours of the spine
- Osteoid osteoma - benign and locally self-limiting:
- Typically presenting in children aged 10-20 years - mostly males.
- They involve the axial skeleton about 10% of the time.
- Over half of osteoid osteomas are found in the lumbar region; the next most common site is the cervical region, then the thoracic region and then the least common site - the sacral region.
- Osteoid osteomas are usually symptomatic.
- They can result in painful scoliosis, muscular atrophy, radicular pain and gait disturbances secondary to pain and splinting.
- Osteoblastoma - benign but expand locally and are aggressive:
- They occur in young patients in the second or third decade of life.
- They are twice as common in males as they are in females.
- Patients typically complain of dull localised pain and paraesthesiae.
- There may be paraparesis and, if the tumour is large enough, paralysis.
- Giant cell tumours - most are benign:
- Malignancy occurs in only a small minority of cases and is usually related to previous irradiation in the area of the tumour.
- These are more common in women and occur in the third to fifth decades of life.
- They can increase dramatically in size during pregnancy, secondary to hormonal influences.
- The symptoms include pain with radicular pattern and neurological impingement with weakness and sensory deficits.
- Osteosarcoma - a malignant spindle cell lesion which produces osteoid, the organic portion of the bone matrix, secreted by osteoblasts:
- Osteosarcomas of the spine are rare.
- They typically present in patients in the fourth decade of life and have a male predominance.
- Osteosarcomas are most common in the lumbosacral segments.
- Patients often present with pain and a palpable mass.
- Neurological symptoms are found in the majority of patients.
- Serum alkaline phosphatase may be elevated.
Cartilage-producing tumours of the spine
- Osteochondroma - they also are commonly referred to as exostosis:
- It is a benign lesion with cartilaginous cap.
- Osteochondromas make up 4% of all solitary spine tumours.
- They occur in patients aged 20-30 years.
- They are more common in males.
- Osteochondromas are more common in the cervical spine.
- Chondrosarcoma - malignant cartilage-producing tumours:
- Chondrosarcoma is the second most common non-lymphoproliferative tumour of the spine.
- Chondrosarcomas comprise about 10% of all spinal tumours.
- Men are affected more often than women.
- The mean age at presentation is about 45 years.
- The thoracic spine is the most common site.
- The most common symptoms are pain, a palpable mass and neurological complaints.
- Multiple myeloma and plasmacytoma - multiple myeloma is the most common primary malignancy of bone and the spine:
- Multiple myeloma is a systemic disease that affects middle-aged people and is characterised by areas of local bone destruction.
- The underlying cell line is the malignant plasma cell, which produces abnormal quantities of immunoglobulins.
- Patients complain of pain that may be worse at night.
- Lymphoma - this is associated with a large infiltrate of lymphoid cells.
Tumour of notochordal origin
- Chordoma - chordomas are uncommon tumours:
- However, they are the most common primary malignant tumour of the spine in the adult, excluding lymphoproliferative tumours and metastases.
- Presentation is often subtle with a gradual onset of pain, numbness, motor weakness and constipation or incontinence.
- Constipation is a uniform finding in most patients with sacrococcygeal lesions.
- Chordomas are slow-growing lesions and are often very large at the time of presentation.
Round cell tumour
- Ewing's sarcoma - Ewing's sarcoma is the most common non-lymphoproliferative primary malignant tumour of the spine in children:
- Patients with Ewing's sarcoma usually present aged 10-20 years.
- The most common site of occurrence in the spine is the sacrococcygeal region.
When classifying benign lesions they may be latent (stage 1), active (stage 2) or aggressive (stage 3).
Approximately 30% of patients with cancer develop symptomatic spinal metastases during the course of their illness. Up to 90% of cancer patients have metastatic lesions within the spine at the time of death. The most common tumours that metastasise to the spine are as follows:
- Systemic or constitutional symptoms tend to be more common with malignant or metastatic disease than in benign lesions.
- Symptoms usually develop slowly.
- Back pain is the most frequent symptom for patients with either benign or malignant neoplasms of the spine:
- With extradural lesions pain is typically aggravated by coughing or straining.
- It may be radicular, localised to the back or felt generally in an extremity.
- Neurological deficits secondary to compression of the spinal cord or nerve roots also can be part of the presentation:
- The degree of neurological compromise can vary from slight weakness or an abnormal reflex to complete paraplegia, depending on the degree of encroachment.
- The loss of bowel or bladder continence can occur from neurological compression or can be secondary to a local mass effect from a tumour in the sacrococcygeal region of the spine, as occurs in chordomas.
- There may also be motor deficits, paraesthesia, numbness in legs and loss of sphincter control.
See also separate Examination of the Spine article.
- Local spinal tenderness
- Motor deficits
- Sensory changes
- FBC and differential white cell count should be taken.
- Renal function and electrolytes should also be requested.
- ESR, or CRP to help to distinguish between neoplastic and infectious processes.
- Elevations in serum calcium or alkaline phosphatase also can provide evidence for neoplastic bone processes. Specific studies such as serum electrophoresis or urine electrophoresis also can be performed to evaluate the likelihood of multiple myeloma or plasmacytoma.
- Plain X-ray, CT scan or MRI scan should be requested.
- Myelography may also be useful.
Confirmation of diagnosis and ascertaining the specific tumour type is by biopsy of the spine lesion after all radiographic studies have been completed.Biopsy can be performed with open or by percutaneous image-guided technique. Percutaneous needle biopsies may not supply adequate tissue for the diagnosis of a primary tumour of the spine.
Decompression and excision where possible. Radiotherapy and chemotherapy where indicated.
Symptoms respond to non-steroidal anti-inflammatory drugs (NSAIDs) or salicylates. Treatment is resection of the nidus by open surgical approach or by percutaneous CT scan-guided resection.
Wide local resection is the treatment of choice whenever possible. This sometimes is limited by the proximity of vital vessels or neural tissue in the spine. Tumour recurrence may occur, especially for aggressive osteoblastomas.
Giant cell tumour
Although most are benign, the lesions are locally aggressive and their size and location may not allow complete resection. Those that cannot be excised should be curetted. Radiation is reserved for surgically inaccessible tumours. The recurrence rates can be up to 50%.
Complete surgical resection is usually curative. Clinical symptoms usually improve following removal of the exostosis. Incomplete resection can lead to recurrence of the lesion.
Complete resection of the vertebra and strut bone grafting may be necessary for complete excision. Cure is possible when complete resection can be achieved but this is only possible in a minority of cases. If wide marginal resection cannot be achieved, the tumour recurrence and mortality rates are high. Chemotherapy is sometimes used to help to decrease the size of the mass. Metastases depend on the grade of the primary chondrosarcoma. The lungs are the most frequent sites of metastasis.
Before chemotherapy, the survival rate for patients was low. This was due to the difficulty of resecting these lesions. Radiation and chemotherapy are the current mainstays of treatment of Ewing's sarcoma in the spine. There is almost 100% local control and excellent long-term survival rate in patients with non-sacral sarcomas. Sacral tumours have a lower local control rate and much poorer long-term survival rate. This is secondary to their tendency for delayed presentation and therefore larger size. The most important prognostic indicator for survival is response to chemotherapy.
Surgical resection is the treatment of choice. However, resection of spinal lesions is often incomplete due to the size and location of the tumour at the time of presentation. Adjuvant chemotherapy and radiation therapy often are employed with varying degrees of success. Spinal osteosarcomas have a very poor prognosis, with deaths usually occurring within the first year after diagnosis. Only a few patients have been reported to survive longer than two years.
Surgical resection is the treatment of choice. The prognosis depends on whether the tumour can be resected completely. The location and size at presentation often mean that incomplete resection only is possible. Patients with sacrococcygeal tumours often have improved survival because the surrounding structures are more expendable and allow better resection. These patients usually have 8-10 years of survival as opposed to 4-5 years of survival for other spinal sites. Death usually is related to local recurrence and invasion rather than metastatic disease. However, chordomas can metastasise. The most common sites of metastases are the liver, lungs, regional lymph nodes, peritoneum, skin and heart.
Generally sensitive to radiation and chemotherapy. Surgery for stabilisation is indicated in myelomas of the spine when destruction of the vertebral body exists to such an extent that collapse and possible kyphosis with canal compromise could result. Adjuvant radiation therapy may be used postoperatively once healing of the surgical site has been obtained.
Complications associated with spinal tumours can be divided into the following:
- Complications associated with the tumour, its recurrences or its metastases:
- Neurological complications include radicular pain or focal weakness from impingement on a nerve root and complete or incomplete paraplegia from direct pressure on the spinal cord. See also separate Spinal Cord Injury and Compression article.
- Complications associated with the surgical, radiation or chemotherapeutic treatment of the tumours:
- Complications that result from the treatment modality employed may be related to structures sacrificed during the surgical resection to obtain clear margins, structures in the path of radiation therapy or the systemic effects of chemotherapy.
Further reading and references
Metastatic spinal cord compression in adults: diagnosis and management; NICE Clinical Guideline (November 2008)
Kaloostian PE, Zadnik PL, Etame AB, et al; Surgical management of primary and metastatic spinal tumors. Cancer Control. 2014 Apr21(2):133-9.
Sciubba DM, Gokaslan ZL; Diagnosis and management of metastatic spine disease. Surg Oncol. 2006 Nov15(3):141-51. Epub 2006 Dec 20.
Clarke MJ, Mendel E, Vrionis FD; Primary spine tumors: diagnosis and treatment. Cancer Control. 2014 Apr21(2):114-23.