Hemangioblastoma
ANATOMY OF THE SPINE
The human spine is formed by individual vertebrae and connective tissue discs in between. The vertebrae form the spinal canal. There are seven cervical, twelve thoracic, and five lumbar vertebrae. The intervertebral discs are the link between the individual vertebral bodies.
Normal anatomy of the spine. Longitudinal section and cross sections through the cervical, thoracic and lumbar spine.
The little joints that link the vertebrae together are known as facet joints. They help to stabilize the spine and, together with the intervertebral discs, allow a certain degree of mobility of the spinal cord. The spinal canal should be wide enough to allow nerve roots to float freely in cerebrospinal fluid.
The front border of the spinal canal is built by the vertebral bodies and intervertebral discs, the side by the intervertebral joints (facets) and back by the ligamentum flavum (yellow band) and vertebral arches. Discs consist of an outer fibrous ring (annulus fibrosus), which surrounds an inner gel-like center (nucleus pulposus).
The spinal cord and nerve roots lie within the spinal canal. The spinal cord extends downwards approx. to the 1st lumbar vertebra. Below, only nerve roots are present in the spinal canal. At the level of the intervertebral disc the nerve roots pass through the neural root foramina to exit the spinal canal. The spinal cord and nerve roots conduct electric-like signals from the skin and joints to the brain, and process of movement is initiated from the brain to the muscles.
DESCRIPTION
Hemangioblastoma is a rare tumor of the central nervous system that originates from the vascular system. Hemangioblastomas usually occur in mid-aged adults. If associated with Von Hippel-Lindau syndrome - at a younger age. They usually located in the cerebellum, brain stem or spinal cord. They are classed as grade one tumors under the World Health Organization's classification system. Hemangioblastomas can cause an abnormally high number of red blood cells in the blood. If surgical extraction of the tumor can be achieved, the outcome for hemangioblastoma is very good, Persons with Von Hippel-Lindau syndrome have a less favourable prognosis than those who have sporadic tumors since those with Von Hippel-Lindau syndrome usually have more than one lesion.
CAUSE OF SYMPTOMS
Acute compression of the spinal cord and/or nerve roots
SYMPTOMS AND SIGNS
Hemangioblastoma of the conus medullaris. A sagittal and transverse T1 MRI image + gadolinium enhancement.
Severe back pain
Pain, numbness and weakness in the limbs
Unsteady gait (especially in dark rooms)
Stiffness, numbness and weakness of the hands and legs
Retention, or unintended urinary incontinence
Numbness of the genitals and loss of sexual function
THE DIAGNOSIS IS BASED ON
Medical history Clinical exam Computer tomography Magnetic resonance imaging (MRI) TREATMENT
Usually treatment is surgical
Non-surgical treatment may include
Opioids Nonsteroidal anti-inflammatory drugs Corticosteroids WHEN SHOULD AN OPERATION BE PERFORMED?
Neurological symptoms caused by compression of the spinal cord or nerve roots
WHAT IS THE GOAL OF SURGERY?
Complete tumor removal
Obtaining material for histological examination
To release the compressed nerve roots and/or spinal cord
To preserve the protective function of the spine
HOW IS SURGERY PERFORMED?
The choice of surgery depends on the localization and size of the tumor
WHICH OTHER DISEASES SHOULD BE EXCLUDED (DIFFERENTIAL DIAGNOSIS)?
Another spine tumor The condition is treated by medical spetialists in the field of:
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