Overview
Primary and metastatic tumors occurring in the spinal cord, spinal nerve roots, spinal membranes, and vertebral canal wall tissues mainly manifested as radicular pain, sensory deficits, motor deficits and reflex abnormalities, autonomic dysfunction, and so on The etiology of the disease is unknown, and it may be related to the gene mutation, and the metastasis of other primary tumors The treatment mainly focuses on surgery
Definition
Intradural tumors include primary and metastatic tumors occurring in the spinal cord, nerve roots, spinal membrane and spinal canal wall tissues.
Back pain is a common early symptom of the disease. Pain may also spread from the back to the hips, legs, feet, or arms and may worsen over time.
95% of intraspinal tumors are benign and tend to have a good prognosis and low recurrence rate after simple resection.
Intravertebral tumors often cause severe disability, which in turn severely affects the patient’s quality of life.
Types
Classification according to tumor origin
Primary intradural tumor
The tumor originates from the spinal cord, nerve roots, spinal membrane and the wall of the spinal canal.
Metastatic intraspinal tumor
This refers to tumors from other parts of the body that have metastasized to the spinal canal, and the primary foci are mostly cancers of the lungs, prostate, breast and kidneys.
Classification according to tumor site
Intramedullary tumors
Among the intramedullary tumors, astrocytomas and ventricular meningiomas each account for 1/3, and the others are cavernous vascular malformations, dermatoid or epidermoid cysts, lipomas, teratomas and so on.
Extramedullary subdural tumors
Most of the extramedullary subdural tumors are benign tumors, the most common are chordoma, nerve sheath tumor, neurofibroma, and the rare ones are dermatoid cysts, epidermoid cysts, teratomas, and lipomas that invade from the extramedullary to the intramedullary.
Epidural tumor
Epidural tumors are mostly malignant tumors, originating from the vertebral body or extradural tissues, including sarcomas, metastases and lipomas, etc. In addition, there are chondrosarcomas, chondrocytomas and lipomas. In addition, there are chondrosarcoma, neurofibroma, spinal meningioma, vertebral hemangioma and so on.
Morbidity
Intravertebral tumors are rare and account for about 15% of primary central nervous system tumors [2].
Intramedullary tumors account for about 24% of intraspinal tumors, epidural tumors account for 25%, and extramedullary subdural tumors account for 51% [2].
Intradural metastatic tumors can occur in 10% of cancer patients, with a prevalence in the thoracic segment, followed by the lumbar segment [2].
Etiology
Pathogenesis
The etiology of different types of intraspinal tumors varies.
Primary intradural tumors
The cause of pathogenesis is not yet clear, and may be related to gene mutation, hormonal stimulation, trauma, heredity (such as type 2 neurofibromatosis, Hippel-Lindau syndrome, etc.), ionizing radiation and other factors.
Metastatic intraspinal tumors
Most of them have a clear etiology and are caused by metastasis of primary tumors from other parts of the body to the spinal canal.
Most of them are hematogenous metastasis, and a few are direct invasion by malignant tumors of neighboring organs.
The primary foci are mostly carcinomas of lung, prostate, breast and kidney.
Tip: For more on etiology, please refer to read the section on etiology of the corresponding primary disease.
Symptoms
Some intraspinal tumors do not cause any symptoms, especially if the tumor is small and does not compress or irritate nearby tissues. However, as the tumor grows, it can cause certain symptoms, especially pain.
Main Symptoms
Radicular pain
Radicular pain is all distinct from somatic referred pain. Physiologically, radicular pain is pain triggered by an ectopic discharge from a nerve root or dorsal root ganglion.
Radicular pain is the most common early symptom of intradural tumors. It is often the first symptom of extramedullary occupying lesions, and is also occasionally seen in intramedullary tumors, with the most severe pain in extradural metastases.
The site of pain is consistent with the nerve distribution in the plane where the tumor is located. Back pain is a common early symptom. Pain may also spread from the back to the hips, legs, feet, or arms and may worsen over time.
Early on, the pain is intermittent and unilateral, with significant nighttime episodes and worsening with coughing, sneezing, and exertion.
In the later stages, the pain is persistent, symmetrical and band-like, with a band-like sensation in the trunk and radiating pain in the limbs.
Sensory impairment
Hyperalgesia
Hyperalgesia is a perceptual disorder in which the sensibility to external stimuli is reduced. For example, a strong pain stimulus only causes slight sensation or even no sensation.
Sensory confusion
Patients may experience hallucinations and illusions.
Loss of sensation
This is characterized by a lack of sensory response to stimuli when the patient is conscious.
Spinal cord hemisection syndrome
Ipsilateral paralysis and loss of deep sensation below the plane of the tumor, and absence of contralateral pain and temperature sensation.
Movement disorder and reflex abnormality
The tumor compresses the anterior root of the nerve or the anterior horn of the spinal cord, which may present with muscle relaxation, reduced resistance during passive activities; muscle weakness and reduced muscle size.
Tumor compression of the spinal cord, manifested by muscle tension and increased resistance during passive activities, may appear in different parts of the body, especially in the arms or legs.
Autonomic dysfunction
Bladder and rectal dysfunction is the most common, mainly manifested as abnormal urinary and bowel functions.
Little or no sweating on the trunk below the tumor plane.
Horner’s syndrome: pupil constriction, normal reaction to light, sunken eyes on the sick side, ptosis and little or no sweating on the affected side of the face.
Urinary incontinence after overfilling of the bladder.
Constipation.
Uncontrolled outflow of loose feces.
Spinal deformities
Intradural tumors can directly invade the bony structures of the spine resulting in deformity of the spine, most commonly seen in extramedullary tumors.
Intramedullary spinal cord tumors can cause spinal cord nerve function damage, cause paraspinal muscle strength imbalance, and cause spinal deformity.
Other symptoms
Tumors in the high cervical segment or below the lumbosacral segment can cause increased intracranial pressure, which manifests as headache, vomiting, and edema of the optic nerve papilla.
Consultation
Department of Medicine
Neurology
Patients are advised to seek medical treatment promptly when they develop symptoms such as back pain, loss of sensation or even loss of sensation, muscle relaxation and weakness, and abnormal urination and defecation.
Neurosurgery
If you are diagnosed with a tumor in the spinal canal and need surgical treatment, you can choose neurosurgery.
Medical Oncology
If a patient is diagnosed with this disease, he or she may choose medical oncology for internal medicine treatment.
Preparation
Preparing for the consultation: registration, preparation of documents, and frequently asked questions.
Tips for Medical Treatment: Registration, Preparation of Documents, Frequently Asked Questions
X-rays, CT or MRI examinations may be required. Avoid wearing metallic clothing such as shirts with buttons, blouses with sequins, and dresses with zippers.
Record the symptoms, duration and other relevant information to give your doctor more information.
Preparation Checklist for Doctor’s Visit
Symptom checklist
Pay particular attention to the time of onset of symptoms, special manifestations, etc.
Is there pain in a certain part of the body? How long has it lasted?
Are there any symptoms such as decreased sensation, sensory confusion or loss of sensation?
Is there muscle atrophy, such as thinning of one calf?
Is there any part of the body that does not sweat?
Are there any urinary or bowel abnormalities such as incontinence, fecal incontinence, or constipation?
How long have these symptoms been present?
Medical History Checklist
Any history of other tumors, such as lung, prostate, breast, kidney cancer, etc.?
Have hormones been applied?
Has there been ionizing radiation?
Any family history of malignant tumors?
Any history of drug or food allergy?
Checklist
Examination results in the last six months, which can be brought to the doctor’s office
Imaging tests: X-ray, CT, MRI, spinal angiography.
Laboratory tests: blood routine, stool routine, urine routine, biochemical tests.
Other tests: Lumbar puncture examination.
Diagnosis
Diagnosis is based on
Medical history
The patient may have the following medical history:
History of primary tumor, such as lung cancer, prostate cancer, breast cancer, etc.
History of hormone application.
History of ionizing radiation.
History of trauma.
Clinical manifestations
Symptoms
Patients may have symptoms related to radicular pain, sensory disturbances, motor disturbances and reflex abnormalities, and autonomic dysfunction.
Physical signs
Patients may have neurologic abnormalities such as decreased or elevated muscle strength, muscle weakness, and sensory deficits.
Lumbar puncture examination
Cerebrospinal fluid cell count and protein quantification: the more complete the obstruction and the lower the location of the tumor in the spinal canal, the higher the protein content. At the same time, it may be accompanied by the change of cell count, but the specificity is poor.
Cerebrospinal fluid kinetic examination: e.g., Quackenstedt test.
Imaging
MRI
It is the best examination for diagnosing intraspinal tumor, which can clearly show the tumor, cerebrospinal fluid and neural tissues, but the visualization of spinal bone is not as good as that of CT and X-ray film.
CT
It can see enlargement of the vertebral canal at the lesion site, destruction of the posterior margin of the vertebral body by compression, and filling of soft tissues in the vertebral canal.
X-ray film
It has high diagnostic value for tumors occurring in the vertebral body, such as hemangiomas, giant cell tumors, metastatic tumors, and chordomas. About half of the intravertebral canal tumors can be seen as bony changes, such as thinning of the pedicle, widening of the pedicle distance, and enlargement of the intervertebral foramen in oblique view.
Spinal angiography
Spinal angiography can exclude spinal arteriovenous malformations and help in differential diagnosis.
Differential diagnosis
Intravertebral tumors need to be differentiated from cervical spondylosis, lumbar disc herniation, spinal cavernous disease, and spinal tuberculosis.
Cervical spondylosis
Similarities: both can present with radicular pain, sensory disturbances and other symptoms.
Differences:
The average age of onset of cervical spondylosis is over 50 years old and the course of the disease is longer.
Patients have neck and shoulder pain and sensory abnormalities, but the plane of sensory impairment is irregular
X-ray film shows the formation of bony cribriform bone at the posterior margin of cervical vertebral body, narrowing of the intervertebral space, and shortening of the anterior and posterior diameters of the spinal canal.MRI shows the narrowing of the cervical spinal canal, compression of the spinal cord in several intervertebral discs in the form of a string of beads, and narrowing of the spinal canal and the subarachnoid space.
Lumbar disc herniation
Similarities: both can present with symptoms such as radicular pain.
Differences:
Mostly seen in young adults, often with a history of lumbar trauma.
It manifests as unilateral sciatica, numbness in the lateral calf, plantar and perineal area, positive straight leg raising test, pain aggravated by activity and alleviated by bed rest.
X-ray radiographs showed narrowing of the intervertebral space; MRI showed that the intervertebral disc had a bird’s beak-like posterior protrusion compressing the dural sac and spinal cord.
Spinal Cord Cavernous Disease
Similarities: Both can present with symptoms such as sensory impairment, motor impairment, autonomic nerve damage.
Differences:
Spinal cord cavernous disease is more common in young people, preferably in the upper thoracic and lower cervical segments, and has a longer course.
MRI can help confirm the diagnosis.
Spinal tuberculosis
Similarities: both can present with symptoms such as radicular pain.
Differences:
Spinal tuberculosis can also present with symptoms such as low-grade fever, fatigue, lethargy, night sweats, loss of appetite & anemia.
Spinal tuberculosis is mostly seen in the thoracic spine, X-ray film can see the destruction of vertebral body, narrowing of the intervertebral space and paraspinal pike shadow, lumbar spine tuberculosis can show the lumbar large muscle shadow increases.MRI can see the vertebral body is low signal, intervertebral discs and intervertebral space is involved or paraspinal abscess formation.
The blood sedimentation of spinal tuberculosis is often obviously elevated, and anti-tuberculosis treatment is effective.
Treatment
Treatment purpose: relieve pain, improve quality of life, prolong survival time.
Treatment principle: benign tumors are mainly treated by surgery, malignant tumors need to be supplemented with radiotherapy and other treatments when necessary in addition to surgery.
Surgery
Principle of surgery
Except for patients with poor general condition or extensive metastasis, early surgical treatment should be performed.
Intradural tumors must be resected by microscopic neurosurgical techniques in order to maximally protect the spinal cord and nerve function.
Satisfactory functional recovery is often achieved after total resection of benign extramedullary tumors.
Surgical approach
Clearly demarcated intramedullary tumors such as ventricular meningiomas and astrocytomas may also have the potential to preserve spinal cord function with total resection of the tumor.
For infiltrative intramedullary tumors, which are difficult to be removed completely, dorsal fasciotomy and decompression of the spinal canal are preferred to improve the symptoms of spinal cord compression.
The resection of intramedullary tumors should be performed by hemi-laminoplasty or laminoplasty to minimize the impact of surgery on spinal stability.
For patients whose spinal stability has been destroyed by the tumor or spinal deformity has occurred, internal spinal fixation fusion should be carried out and spinal deformity should be corrected at the same time as the resection of intraspinal tumor.
Radiation therapy
For intraspinal tumors, radiation therapy is effective for certain malignant tumors and can be used as postoperative adjuvant therapy.
Drug therapy
Bisphosphonates
Bisphosphonates have the ability to modulate bone turnover, prevent osteolysis, reduce the incidence of bone-related events and relieve bone pain in patients with bone metastases.
Chemotherapeutic agents
Depending on the nature of the primary tumor, effective chemotherapy is also available. Specific drugs are associated with specific tumors, as detailed in the treatment section of each term.
Prognosis
Cure
Cure varies widely among different types of intradural tumors. No authoritative statistics are available, and the specific data need to be evaluated in general terms with reference to the patient’s specific type of pathology.
Benign
95% of intradural tumors are benign tumors with good prognosis and low recurrence rate after simple resection.
For example, as shown in a study, in 27 patients included with residual spinal nerve sheath tumors after surgery, 30% were found to have evidence of tumor regrowth after an average of 5 years of follow-up [9].
Malignancy.
Some intraspinal tumors are malignant, difficult to operate, with many postoperative complications, risk of metastatic recurrence, and poor prognosis.
For example, in malignant nerve sheath tumors (MNSTs), some studies have shown that even after maximal resection plus adjuvant therapy, many tumors still recur; the 5-year survival rate is 40% to 50% [10].
Daily
Daily management
Dietary management
Diet should be light, easily digestible and nutritious.
It is recommended to consume more protein-rich foods such as beef and lamb, fish, eggs, and milk to replenish sufficient energy for the body.
Consume more foods rich in vitamins and dietary fiber, such as fresh vegetables and fruits.
Avoid cold, greasy, spicy and irritating foods such as fried chicken, cream and animal offal.
Life management
Quit smoking and drinking, avoid strenuous work, have a regular routine, avoid staying up late and get enough sleep.
Maintain a healthy weight, take appropriate activities such as slow walking, tai chi, qigong, breathing exercises, etc., and avoid crowded places.
During radiotherapy, avoid rubbing and scratching the skin, keep it clean and dry, and ban the use of soap and towel for bathing.
Psychological support
Maintain a good mood and mindset, and face the disease positively.
Learn to confide in friends and family members to avoid excessive pressure, which may cause mental illness, and seek help from a psychiatrist if necessary.
Face the disease squarely, do not be blindly optimistic or pessimistic, and face the disease with a calm mind.
Family members should give adequate company to the patient, create a cozy family atmosphere, comfort the patient and help him/her get through the difficulties.
Disease monitoring
Patients should pay attention to their own condition in daily life, and seek medical treatment promptly if symptoms such as pain, masses, neurological dysfunction, etc. worsen or reappear.
Follow-up
Importance of follow-up
Regular follow-up helps to detect recurrence and metastasis of intraspinal tumor in time.
Follow-up time
It is recommended that patients undergo follow-up examinations every 3 to 6 months after active treatment. Please follow your doctor’s instructions.
Review items
Tumor markers, X-rays, CT, MRI, lumbar puncture and other tests may be needed for follow-up.
Prevention
Different types of intravertebral tumors have different etiologies, and the etiology of many diseases is still unclear, so there are no accurate and effective preventive measures at present. It is recommended to have regular medical checkups, find out the changes in oneself in time, and seek medical treatment in time, so as to strive for early detection and early treatment.
Prevention of Primary Intravertebral Tumor
In rare cases, primary intraspinal tumors may be caused by certain genetic disorders, such as neurofibromatosis type 2 (NF2), and Schipper-Lindau syndrome (VHL syndrome).
If a first-degree family member (sibling or parent) has one of these disorders, it is important to have genetic counseling or testing to see if you also have the disorder. Detecting spinal tumors at an early stage usually has a better outcome.
Preventing metastatic intraspinal tumors
Not all metastatic intraspinal tumors can be prevented. Many patients already have metastatic intraspinal tumors when they are first diagnosed.
If some type of cancer at another site has already been diagnosed, prevention is accomplished with adjuvant or neoadjuvant therapy. The goal of adjuvant therapy is to shrink the primary tumor and minimize the possibility of metastasis.