Tumours of the spine
Benign
Malignant = CLOSE Call Man
Benign tumours
Osteoblastoma & Osteoid osteoma
Aneurysmal Bone Cysts
Hemangiomas
Giant cell tumours
Eosinophilic granuloma
Radiographs
Malignant Tumours
Solitary plasmocytoma
Osteosarcoma
Secondary Osteosarcoma
Ewing's Sarcoma
Chordoma
Chondrosarcoma
Lymphoma
Metastatic Tumours
Pathophysiology
Diagnosis
Staging (work-up)
"superscan" entity
Computed tomography
Staging
Medical evaluation
Prognosis
Treatment
Radiotherapy
Braces
Epidural Hemangiomas
Epidural Lipomas
Extradural Meningiomas
Neurofibromas
Lymphomas
Spinal tumours in children
Primary bone tumours
Benign
posterior elements = AO3
- anuerysmal bone cyst
- osteoblastoma
- osteoid osteoma
- osteochondroma
anterior elements = HEN & PlGS
- haemangoma
- eosinophillic granuloma
- neuroblastoma
- Paget's disease
- infection
- giant cell tumor
- sarcoid
Malignant = CLOSE Call Man
- chordoma
- lymphoma
- osteosarcoma
- usually secondaries
- malignant transformation in Paget's or after DXT
- secondaries
- Ewing's - usually secondaries
- chondrosarcoma
- myeloma
Primary Tumours of Bone
- uncommon
- if > 18 yr old -> 80% malignant
Benign tumours
Osteochondromas
- either single or multiple
- among the most common bone lesions
- vertebral involvement 7%
- neurologic compromise rare
- when occurs, routine imaging studies may not be adequate
- tomography outlines the bony lesion
- visualised w/ myelography or MRI
- slow progression
- excision, en bloc or piecemeal
- excellent recovery of neurologic function with little likelihood of recurrence
Osteoblastoma & Osteoid osteoma
- osteoblastic lesions differentiated primarily by size
- great propensity for the spine
- usually involving the posterior elements
- present in the 2nd or 3rd decade
- most common complaint is of back pain
- typically persistent and unrelated to activity
- often most noticeable at night
- good response to aspirin
- often deformity
- scoliosis
- usually disappears after tumour removal
- radiographic demonstration difficult
- lesion less than 2.0 cm in diameter ( osteoid osteoma ) easily missed
- sclerotic lesion with lucency centrally - "nidus"
- CT demonstrates the lesion well - if cuts are at the wrong level or are too wide the tumour may be missed
- bone scan - most sensitive method
- "double density" sign
DD
- osteomyelitis
Rx
- excision - curettage & bonegraft
Aneurysmal Bone Cysts
- rare in spine
- under 30 yr.
- usually pain
- most commonly in the lumbar spine
- involving the posterior elements approximately 60%
- tendency to involve adjacent vertebrae
- may involve three or more vertebrae in sequence
- radiographs
- expansile, osteolytic cavity w/ strands of bone forming a bubbly appearance
- cortex is often egg-shell thin & blown out
DD
- benign lytic lesions
Rx
- total excision or curettage
- provides a high rate of cure
Hemangiomas
- common lesions in spine - 10%
- deformity or pain uncommon
- soft tissue extension with nerve root and cord compression have been documented
- radiographs
- characteristic
- prominent vertical striations produced by the abN thickened trabeculae
- CT/MRI imaging - more information about thecal impingement
Rx
- lesions are radiosensitive - frequently respond to radiotherapy alone
- when cord compression develops - surgical treatment
- angiography to establish the vascular source for the tumour, to identify the primary vascular supply to the cord =( artery of Adamkiewicz), and to consider preoperative embolization or operative ligation
Giant cell tumours
- most slow-growing, locally aggressive tumours that do not metastasise
- in the third & fourth decades
- most commonly in the vertebral body radiographs
- lytic appearance & marginal sclerosis
- may expand the surrounding cortical bone extensively as enlarges
CT
- shows extension of tumour
- early identification of recurrence - used in postoperative follow-up on a routine basis
Rx
- complete excision is the treatment of choice
- often anterior approach and vertebrectomy necessary
- if adequate resection -> radiotherapy is not necessary
Eosinophilic granuloma
- produces focal destruction of bone
- most commonly seen in children < 10 years
- vertebral involvement 10 to 15%
- vertebral body typically involved
- neurologic symptoms may develop w/ or w/out associated vertebral collapse
Radiographs
- central lytic lesion w/ poorly defined margins & permeative bone destruction
- may produce a marked periosteal reaction
- bony destruction may produce cavitation, partial vertebral collapse, or a classic vertebra plana after complete collapse
- "coin-on-end" appearance
DD
- osteomyelitis or a high-grade sarcoma
- biopsy necessary to differentiate
Rx
- many heal w/out treatment
- biopsy + irradiation & immobilisation
Malignant Tumours
Multiple Myeloma & Solitary Plasmacytoma
- two manifestations of B-cell lymphoproliferative diseases
- natural history differs significantly
Multiple myeloma
- uncommon
- incidence 2-3/100,000 in the general population
- differ in terms of age & sex distributions and survival
- 50% male : female
- age of onset 60 yr.
- rapidly progressive & lethal w/in 2-3 years despite local or systemic treatment
- spinal lesion represents a metastasis - outcome is even worse
radiology
- diffuse permeative appearance
- bone scan usually not hot ---> radiologic "bone surveys"
- CT scan in spine: "Swiss cheese" pattern
DD
- mets
- Ewing's sarcoma(age diff.)
Solitary plasmocytoma
- rare - 3% of all plasma cell neoplasms
- isolated lesion
- 70% male - if in spine: 75%
- age 50 yr
- treatment may provide long-term disease-free survival or cure
radiology
- lytic lesion - benign or aggressive
Rx
- radiation
- if vertebra collapse & cord compression -> decompression + stabilisation
- chemotherapy if systemic
- follow up w/ Se protein electrophoresis & Bence Jones protein
Osteosarcoma
- 2% in the spine
- poor prognosis - survival ten months from the time of diagnosis
- anterior elements involved in 95%
radiographic findings
- both Iytic & sclerotic lesions
- cortical destruction, ST calcification & collapse in advanced cases
CT
- demonstrates intraspinal & paraspinal ST masses more clearly
Rx
- aggressive surgical approach ( wide resection or vertebral body resection )
- combined w/ radiotherapy
Secondary Osteosarcoma
- arises 2° in pagetoid or previously irradiated bone
- patient older than those w/ 1° osteosarcoma - over age of 60
- 30% of all osteosarcomas of the spine
Paget's disease
- patients who develop an osteosarcoma
- have polyostotic dx
- aggressive lesions - extensive bony destruction & metastasizing early
- typically anaplastic
- poor prognosis
Post-irradiation osteosarcoma
- most patients received in excess of 5000 rads
- for non osseous disease - Hodgkin's lymphoma, breast & cervical carcinoma
- skeletal disease - GCT & Ewing's sarcoma
- survival slightly better than for those w/ Paget's disease
- radiographic evidence of radiation osteitis
Ewing's sarcoma
- under 30 yr.
- 4% of all Ewing's tumours arise in the spinal column
- most originating in the sacrum
- prognosis generally poorer than for those w/ extremity lesions
- neurologic compromise common
metastatic Ewing's sarcoma
- arising in the epidural space, w/out bony involvement
- rare but documented phenomenon
radiographs
- permeative appearance
- "onion-skinned" type periostitis
- collapse of the vertebral body - vertebra plana
DD
- eosinophilic granuloma
- infection
Rx
- multi-agent chemotherapy & high-dose radiotherapy
- surgical treatment - for decompression of neural elements & stabilisation
Chordoma
- rare malignancy
- in all age groups, but predominantly in 5-6th decade of life
- arises from remnants of the primitive notocord
- found in the sacrococcygeal & suboccipital regions of the spine
- occasionally from notocordal rests within the vertebral body in the thoracic or lumbar region
- slow local spread, but capable of distant metastases
symptoms
- constipation, urinary frequency, or nerve root compression
- firm, fixed pre-sacral mass palpated on rectal examination
Rx
- surgical extirpation the only curative procedure
- wide margin must be obtained
- biopsy after appropriate staging studies
- open biopsy through a direct posterior approach
- incision must be excised en bloc w/ the tumour at the time of the definitive resection
- local recurrence bad prognostic sign
Chondrosarcoma
- 10% in the spinal column
- over age 40
- slow growing & relatively resistant to radiotherapy & chemotherapy
- tendency to recur locally - poor prognosis
radiographically
- large area of bone destruction
- associated soft tissue mass & flocculent calcifications of this mass (chondroid mass)
- vertebral lesion primarily lytic, w/ sclerotic margins & with no mottled calcification
CT & MRI
- demonstrating the extent of the lesion & evaluating cord compromise
Rx
- surgical excision
Lymphoma
- may present as a systemic dx. w/ skeletal manifestations or as an isolated bony tumour
- referred to in the past as a reticulum cell sarcoma
- considered a metastatic lesion by some authors
- account for a significant number of spinal neoplasms requiring treatment
Metastatic Tumours
- by far the most common skeletal tumours seen by the orthopaedist
- spine is the most common site of skeletal involvement
Breast cancer
- most common source of bony metastasis - 40%
- 65 - 85% of women w/ breast cancer develop skeletal dx. before death
Bronchogenic & prostatic carcinomas
- most common in men 15% & 7%
Multiple myeloma & lymphoma
- common sources of disseminated skeletal lesions 10%
Renal, gastrointestinal & thyroid carcinomas
- 5% to 2%
Pathophysiology
- distribution of metastatic dx. in the skeleton is influenced by three factors
- tumor emboli from bloodstream arrest in the natural filters - capillary beds of the liver, lungs & bone marrow
- tumors of the lung - directly through the segmental arteries
- carcinomas of the breast - paravertebral venous plexus in the thoracic region
- prostate drains through the pelvic plexus (Batson's plexus)
- nature of the tissue bed in which the embolus comes to rest
- "seed and soil" theory
- certain tissues provide a more favourable environment for the survival of the tumour embolus
- intrinsic factors of tumour cells
- may give one cell line advantage in growing in the medullary space
- prostaglandins
- osteoclast activating factors by breast cancer cells
- tumor emboli from bloodstream arrest in the natural filters - capillary beds of the liver, lungs & bone marrow
Diagnosis
Symptoms
Pain
- begins insidiously, progresses & persists at rest
- localised, at least initially
- often worse at night
Primary cancer
- usually diagnosed months or years before symptoms of spinal involvement
Neurological deficit
- late symptom
Staging (work-up)
History
- primary tu.
- age >40 yr.
Complete examination
- Iymphadenopathy, hepatomegaly & splenomegaly
Laboratory studies
- FBC, ESR, platelet count & reticulocyte count
- U+E
- uric acid
- alkaline phosphatase, acid phosphatase. serum calcium & phosphate
- protein electrophoresis
Radio-isotope scan(Tc 99)
- screening investigation for metastatic dx.
- areas of increased uptake - plain X-ray films & MRI studies
- 95% accurate for skeletal metastases
- older patient - "hot spots" more likely be d/t osteoarthritic changes or osteoporotic #s
"superscan" entity
- metastatic dx. infiltrates to such an extent that the whole skeleton lights up
- should not be interpreted as a normal scintigraph study
multiple myeloma & aggressive metastatic tumours
- producing extensive bone destruction w/out a significant reparative process of new bone formation - bone scintigrams may appear normal
Computed tomography
- bony lesions
- inferior in specificity & extent of marrow infiltration - MR investigation of choice
MRI
- accurate in showing soft-tissue & marrow extent
Needle biopsies
- confirm the diagnosis of a solitary metastasis
- in widespread metastatic dx. if histologic diagnosis of metastasis has not yet been confirmed
Staging
- carcinomas staged according to their
- local extent (T)
- regional Iymphatic metastases (N)
- distant metastases (lung, bone, liver, etc.) (M)
- and occasionally their histologic grade (G)
Medical evaluation
- systemically ill
- anaemic
- receiving chemotherapy - immunosuppressed & thrombocytopenic
- post-irradiation - skin unsuitable for surgical incision
- preoperative renal function should be monitored
- serum Ca & Ph levels development of malignant hypercalcemia
Prognosis
- tumour type most important determinant
- sex, age, location of metastases & interval between dg. of disease & appearance of metastases
Treatment
Operative
- anterior, posterior or combined resections
- stabilisation augmented with methyl methacrylate
- good to excellent pain relief 80%
- improvement of neurologic deficits
- 40% with posterior decompression
- 70% decompressed anteriorly
Radiotherapy
- historically treatment of choice for osseous metastases involving the spine
- reasonable treatment option for many patients
indications
- spinal pain or neurologic compromise w/out vertebral collapse
- mets from breast carcinoma are usually responsive to radiation
Braces
- used w/ appropriate radiotherapy
- symptomatic relief & bony healing of the lesion occurs in many cases
- neurologic injury can be prevented while medical therapy is instituted
- halo-vest - TLSO
Extraosseous tumours
Extradural tumours
- uncommon
- most are benign soft tissue lesions
Epidural Hemangiomas
- 4% of all epidural tumors & 12% of all intraspinal hemangiomas
- almost always in the upper thoracic spine
- characterized by slowly progressive spinal cord compression
- acute progression if hemorrhage occurs and an epidural hematoma forms
Epidural lipomas
- uncommon
- increased risk in patients w/ Cushing's syndrome
- steroid-induced lipomatosis usually involves the thoracic spine
- signs of cord compression can appear acutely
- MRI demonstrates the full extent of cord compression
- laminectomy and surgical decompression is the treatment of choice in progressive lesions
Extradural Meningiomas
- rare tumors & almost always associated with an intradural component
- tend to be vascular and may erode bone - appearance of an extradural metastasis
- have a tendency to recur
- neurologic recovery is usually satisfactory after decompression
Neurofibromas
- relatively common spinal cord tumor - 25% of all spinal tumors
- 10% extradural and another 10% have both intradural and extradural components
- usually solitary but may be multiple
- usually arise from the spinal nerve root
- most common cause of an hour-glass or dumbbell tumor
- 20% may undergo malignant degeneration
- may produce significant cord compression ---> excision indicated
- intradural exploration to rule out an intradural tumor
Lymphomas
- most common malignancy in the epidural space
- extension from paraspinous nodes or from the vertebral body account for most
- some cases of primary lymphoma are reported
- plain radiographs may show some bony erosion or may be completely normal
- myelography most reliable for demonstrating the presence and extent of the lesion
- surgical decompression usually involves laminectomy
- neurologic improvement depends on the preoperative grade of neurologic deficit
Intradural Tumors
- Extramedullary
- 84% of all intradural tumors
- Neurofibromas-29%
- Meningioma-25%
- Exophytic ependymoma-13%
- Sarcoma-l2%
- Exophytic astrocytoma-6"/o
- Others (vascular tumors, epidermoid tumors, lipoma etc.)-15%
lntramedullary Tumors
- Ependymoma-56%
- Astrocytoma-29"/o
- All others-less than 4% each(including Iipoma, epidermoid, teratoma, demrmoid, etc.)
Spinal tumours in children
- uncommon
complexity of treatment
- Rx. of neoplasm
- management of the spinal deformity!!!
- progressive deformity w/ growth
- laminectomy, rib resection, irradiation may lead to deformity
Primary bone tumours
Benign
- 70% benign
- > 40% made up by osteoblastoma - osteoid osteoma, osteochondroma, ABC
Malignant
- Ewing's sarcoma most common primary, but may be metastasis in spine
Metastatic
- different from adults - most common neuroblastoma, embryonal carcinoma & various sarcomas
Leukaemia
- often presents w/ back pain & vertebral collapse
non-specific symptoms
- lethargy
- anaemia, fever
- periph. leukocyte count & ESR elevated in 60%
radiographs
- may show focal lytic lesion, sclerosis or periosteal reaction
- bone scan unreliable !!!
DD
- osteomyelitis or joint sepsis