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Rare disease 'spinal cord tumor': early detection and prompt treatment decide the prognosis

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Rare disease 'spinal cord tumor': early detection and prompt treatment decide the prognosis

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Gangnam St. Peter's Hospital  26-03-11 15:04 

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 Cervical and lumbar disc herniation are common conditions, with millions of patients a year. But there is a rare disease that can be mistaken for a disc problem and that, if patients endure the pain and keep putting off a hospital visit, can even lead to paralysis of all four limbs: spinal cord tumor, in which a tumor develops in the spinal cord or spinal canal and compresses and invades nerve tissue.

Spinal cord tumors are a relatively uncommon, rare condition, reported to occur in about 2 to 4 people per 100,000 population each year. At present it is difficult to identify a clear cause, and they are known to appear mainly in people in their 40s and 50s. The problem is that the early symptoms resemble those of disc herniation, making them hard to distinguish on clinical symptoms alone, so diagnosis requires detailed imaging such as MRI.

Yoon, Kangjun, President of St. Peter's Hospital, a general hospital focused on spine, joint and cerebro-cardiovascular care, said, “We recently had a case of a patient who came in with disc symptoms but was diagnosed with a spinal cord tumor and underwent surgery.” He stressed, “If symptoms keep getting worse, it is dangerous to assume it is simply a disc; you need a detailed examination at a hospital with specialists and specialized imaging capability.”

The spinal canal that runs down the center of the body is generally known to be around 15mm in diameter. Within the space inside the dura of the spinal canal lie the spinal cord, part of the central nervous system, together with other major structures and the body’s principal nerve bundles. Because of this, when a tumor develops inside the spinal canal, even a small one compresses the spinal cord and nerves and causes a variety of symptoms, including pain. In addition, if the tumor grows and nerve compression becomes severe, there is a high risk that it will lead to serious loss of bodily function, such as loss of sensation, motor paralysis, gait disturbance and bowel dysfunction.

Spinal cord tumors are divided into various types according to where they occur and the type of cell involved. By location they are divided into extradural tumors, intradural extramedullary tumors and intramedullary tumors, and the pattern of symptoms differs accordingly. Extradural tumors, which account for a large share of all types, are, as the name suggests, tumors that develop outside the dura; they cause weakness and paralysis of the upper and lower limbs over a period of hours or days.

Intradural extramedullary tumors develop between the inside of the dura and the outside of the spinal cord parenchyma, causing asymmetric nerve root pain or sensory abnormalities, and in some cases Brown-Séquard syndrome. Intramedullary tumors, which arise within the spinal cord, can cause abnormal sensation and pain along with motor impairment, sensory impairment and urinary dysfunction.

Subtypes also differ according to the cell in which the spinal cord tumor originates. Extradural tumors are in most cases metastatic tumors that have spread from another cancer. Among intradural extramedullary tumors, schwannomas, which arise from the nerve sheath surrounding a peripheral nerve, and meningiomas, which arise from the meninges surrounding the spinal cord, account for about 70% of all types. Intramedullary tumors, meanwhile, may be classified as ependymomas, astrocytomas, hemangioblastomas and others.

In particular, when a tumor develops in the cauda equina, the terminal structure of the spinal cord, the main symptoms are sciatica and motor and sensory abnormalities in the inner thighs on both sides, symptoms that are easily mistaken for a lumbar disc herniation.

Beyond this stage, as the tumor continues to develop, overall nerve function becomes blunted, and the longer this is left unaddressed the more likely the nerve damage is to worsen. If the tumor invades one lateral half of the spinal cord, loss of motor function together with loss of sensory functions such as pain and temperature sensation becomes pronounced. When rapid progression — as with a metastatic malignant tumor — produces the symptoms of spinal cord transection, loss of sphincter function and paralysis of motor and sensory function follow.

That is why precise diagnosis at an early stage is so important in spinal cord tumors. The problem, as mentioned earlier, is that it is not easy to distinguish its presentation from that of a lumbar disc herniation.

President Yoon, Kangjun said, "Early spinal cord tumors cause neurological symptoms similar to those of lumbar disc herniation, such as back pain, radiating leg pain, sensory abnormalities, and muscle weakness, so they are easily mistaken for a disc problem and care is needed," adding, "Rather than automatically assuming that back pain or other physical abnormalities are a disc problem or muscle pain and letting them pass, it is better to see a specialist with extensive experience in the spine field for a thorough examination and diagnosis."

With spinal cord tumors, nerve damage accumulates over time and symptoms risk deteriorating seriously, so diagnosis and treatment at the earliest possible stage is the first priority. Choosing a hospital where the wait for an appointment is six months or more therefore carries a risk that symptoms will worsen. If symptoms are progressing quickly or neurological abnormalities are becoming more severe, rather than insisting on a large hospital it is worth seeking out a specialty hospital with sufficient expertise and actively bringing forward the schedule for consultation and examination.

Above all, the most important thing when choosing a hospital is expertise in spine surgery. Because spinal cord tumors arise where major nerves of the body run, the precision of the surgery has a great effect on the prognosis. It is therefore best to look for a hospital with the right systems in place: medical staff with experience and capability in spine surgery, up-to-date equipment, multidisciplinary collaboration, and patient management.

President Yoon explained, “In surgery for a spinal cord tumor, microsurgical instruments such as the cavitron ultrasonic surgical aspirator (CUSA) are used as part of a surgical strategy that further improves safety during the operation and minimizes damage to the surrounding nerves.” He added, “Above all, what matters is whether a specialized surgical strategy can be established to suit the location and characteristics of the tumor.”

Spinal cord tumors are diagnosed first through neurological examination of sensory changes, muscle strength, balance, coordination, vision, and hearing, followed by imaging. X-rays, CT, and MRI are taken to examine the affected area and make the diagnosis. If a metastatic tumor is suspected, a whole-body PET-CT scan is also performed.

The direction of treatment is then decided according to the tumor's location and type and the degree of nerve compression. For benign tumors and primary spinal cord tumors, complete resection by surgery is the first option considered. Most extradural spinal cord tumors can be removed by laminectomy. For intradural extramedullary tumors such as neurofibromas, however, complete removal is known to require cutting the nerve root.

In some cases, only part of the tumor is removed or radiation therapy is given. Metastatic tumors in particular are often spread across several sites, so complete resection of every tumor may be difficult. In such cases, part of the tumor lesion compressing the nerve may be removed and chemotherapy and radiation therapy then considered as well.

The prognosis after surgery for benign spinal cord tumors is generally known to be good. When initial treatment is received at the right time, nerve function is often maintained normally or improves after surgery. However, if sensation has been lost because of reduced nerve function or paralysis before or after surgery, careful attention is needed to prevent thrombophlebitis, pulmonary embolism, and pressure sores.

President Yoon said, "Spinal cord tumor is above all a condition in which early diagnosis and treatment largely determine the prognosis, so rather than simply waiting a long time it is important to choose a hospital by looking at the medical team’s experience and infrastructure," adding, "It is a rare disease, but once it develops it can lead to loss of motor function and even nerve paralysis, so the key is to detect it quickly through specialized, detailed examination and to treat it promptly."




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