Chordoma
Introduction
Introduction to chordoma Chordoma is a locally invasive or malignant tumor that is a common epidural tumor involving the slope and the appendix, formed by embryonic residual or ectopic notochord. It is mainly caused by middle-aged and old people aged 50 to 60, and it also occurs at other ages. These tumors can occur anywhere along the central axis of the spine, but are most common on the sides of the slope and the appendix, which grow slowly and often become sick for more than 5 years before symptoms appear. Although the growth of chordoma is slow and there is little distant metastasis (late metastasis), its local destructiveness is very strong. It is harmful to the human body due to the continued growth of the tumor, and it is easy to relapse after surgery, so it is still a malignant tumor. basic knowledge The proportion of illness: 0.002% Susceptible people: good for middle-aged and elderly people aged 50 to 60 Mode of infection: non-infectious Complications: somatosensory disorder, dyskinesia, fecal incontinence, urinary incontinence
Cause
Chord tumor
(1) Causes of the disease
Chordoma is a congenital tumor. Most chordomas originate from the intramedullary spinal cord residue near the vertebrae. They may also be related to post-infection myelitis and myelitis after vaccination.
(two) pathogenesis
Chordoma can be divided into two types, namely classic type and cartilage type. After the tibia is invaded, it can invade the pelvic cavity forward, and can invade the spinal canal backwards, compressing the cauda equina nerve root, causing the damage of the nerve root at the corresponding site.
The chordoma is a smooth nodule. The tumor tissue is white translucent jelly with a large amount of mucus. It is dark red with extensive bleeding. The edge of the tumor is often lobulated or nodular. There is a fibrous tissue on the surface. The membrane is generally not broken into the adjacent organs. Under the microscope, the tumor cells are small, cuboidal, round or polygonal, the membrane is clear, the cell mass is high, red staining, common vacuoles, and the large vacuoles can reach the general level. Dozens of times the volume of the cell, the so-called "large vacuole cells", the nucleus is round or oval, located in the center, the cells are arranged in a cord-like or irregular glandular cavity, during which time it is mucus, occasionally nuclear large staining Cells, multinucleated cells and mitotic cells.
Prevention
Chordoma prevention
The incidence of chordoma metastasis is not high, and there are more chordomas in the appendix, but there are few tumors in the skull base and above the spine. Generally, metastasis occurs only after 10 years of tumor development. Lymph nodes are often involved, and then the blood runs to the lungs, liver and peritoneum. The occipital chordoid chordoma is rich in cartilage, which is better than the common chordoma.
Complication
Chordoma complications Complications somatosensory disorder dyskinesia disease fecal incontinence urinary incontinence
Symptoms, movement disorders, abnormal reflexes, symptoms of meningitis, and incontinence may occur.
Symptom
Symptoms of chordoma common symptoms calcified sciatica polyps constipation intracranial hypertension spinal cord compression sacral perforation lower abdominal pain hardening paraplegia
symptom
Pain is the earliest symptom, which is caused by tumor expansion or invasion of important tissues or organs. Tumors located in the appendix often cause pain in the tail, followed by local swelling, and gradually grow up, from the subcutaneous uplift, can also develop into the pelvic cavity, compressing the bladder and rectum, causing urinary incontinence, constipation, sciatica and other symptoms. Tumors located in the occipital part of the butterfly can oppress the optic nerve and other cranial nerves, the pituitary gland, the brainstem, etc., and can cause intracranial hypertension in the later stage. There are spinal cord compression around the spinal canal, which can cause root pain, paraplegia, incontinence and so on.
Chordoma is more common in the 40 to 60 years old, the elderly, occasionally seen in children and young people, tumors occur at the ends of the spine, that is, the skull base and the atlas, the former is 35%, the latter is 50%, the other vertebrae is 15 %, rare in the longitudinal axis of the bone, such as the transverse process of the vertebrae, sinus bone, etc., more often with the appendix pain as the first symptom, most of the intraspinal chordoma often experience the symptoms for several months to the diagnosis year.
The clinical symptoms are determined by the location of the tumor: the pillow, the butterfly tumor can produce headache, the symptoms of cranial nerve compression (the most visible optic nerve), the destruction of the pituitary can have pituitary dysfunction, protruding laterally or downward can form in the nasopharynx The mass has a blocked nasal cavity, and there is a pus and bloody secretion. It occurs at the lower end of the slope and at the junction of the skull and neck. It is often a common symptom of headache, occipital or occipital neck junction, and the symptoms can be aggravated when the head position changes. In the thoracic vertebrae, the tumor can invade the vertebral body structure of the corresponding part, and the intervertebral foramen can be inserted into the thoracic cavity. The intercostal nerve can cause segmental burning nerve pain, and even cause pulmonary pleural irritation, which occurs in the appendix. Tumor compression symptoms appear later, often with appendix pain as the main symptom. The typical symptoms are chronic lumbar and leg pain, persistent nighttime aggravation, and the medical history can be as long as 0.5 to 1 year. When the tumor is large, the mass is pushed forward to the pelvic cavity. Pressing the sacral nerve root, causing loss of control of the urine and urination and numbness or pain in the lower extremities and buttocks. The mass can cause mechanical obstruction, causing urinary dysfunction and large Constipation, occurs in other parts of the spinal canal, with local pain as a common symptom.
Physical examination
When the chordoma of the fistula is clinically examined, it can be seen that the ankle is full. The anus can touch the tumor in a round, smooth and flexible state. Most of the slowly growing tumor masses swell forward and are not easy to find, only in the advanced stage. When the tumor breaks into the gluteal muscle, the sacral spine muscle or subcutaneous is found, and the lower abdomen can also touch the mass. The anus is a routine examination of the early detection of the sacral tumor, especially the chronic lower abdominal pain patient who has been cured for a long time. Anal examination is especially important.
A soft, compressible mass in the midline of the infancy, head, cranium or back, which may be translucent or increased by crying. A black hair bundle may appear above the mass or a bald area surrounded by a black hair ring, many skins Damage can be inferred from other spinal cord and associated structural deformities. Skin manifestations of spinal regurgitation include sag lesions, dermal lesions, abnormal pigmentation, hair damage, polypoid lesions, tumors, subcutaneous tissue, and vascular damage.
The sites of chordoma metastasis include the lungs, eyelids and penis.
Examine
Chordoma examination
X-ray inspection
X-ray plain film showed that the tumor was mainly caused by osteolytic destruction, no calcification and ossification, and local destruction of the humerus and its calcified plaque were found. The tumor of the caudal vertebrae produced localized bone from the central or lateral side of the atlas. Destruction can make the bone expand, thin, disappear, located in the chest, lumbar vertebral body vertebral body destruction and depression, but the intervertebral space remains intact, the skull chordoma is more common in the skull base, near the sella, the butterfly pillow cartilage commissure And rock bones, bone destruction boundaries are clear, there may be fragments of broken bones and patchy calcification deposits, and soft tissue masses protruding into the nasopharyngeal cavity, generally larger, smooth edges, chordoma mass protruding cranial cavity When the calcified pineal gland can be displaced, it can cause intracranial hypertension. The chordoma of the cervical spine is often located in the upper cervical vertebra, especially at the junction of the cervical vertebra and the occipital bone. The vertebral body is involved, and the pedicle is occasionally invaded. The soft tissue mass is involved. , often prominent early manifestations, thoracic chordoma is less common.
2. Cyst angiography and barium enema
Helps to determine the extent of the tumor.
3. CT examination
CT has localization and qualitative value for determining tumors, and it has found that tumors have calcification or plaque formation. It has important value and can guide surgery. It can be obviously strengthened after intravenous injection, which helps to clarify the contents of tumor and its surrounding capsule characteristics. The bone scan of the sacral chordoma is often a density reduction or cold nodule. Exclude overlapping bladder shadows during the examination. The bladder should be empty or laterally scanned before the examination. CT can clearly show the chordoma bone destruction and The relationship between soft tissue shadow and cauda equina, large blood vessels and surrounding tissues, contrast agent injection can enhance the clarity of CT images. According to reports in the literature, cystic changes of chordoma can be expressed in spots and low-density areas in CT; angiography It is helpful for the diagnosis of cervical chordoma; myelography can show that the tumor expands in the epidural space, and the growth in the spinal canal can exceed the extent of bone destruction, which is helpful for the formulation of the surgical plan.
4.MRI scan
Magnetic resonance imaging has a localized and qualitative value for tumors. It is a very useful method for evaluating chordoma. When CT scan reveals bone destruction, magnetic resonance examination should be performed routinely. The chordoma T1 image shows low signal or equal signal, T2 image. High signal on the lobulated high-signal lesions and low-signal separation are obvious. It is worth noting that magnetic resonance can distinguish tumor types. Generally, classical chordoma has longer T1 and T2 signals than cartilage-type chordoma.
5. Microscopic examination:
It shows that the tumor tissue changes more, each case is different, even in different regions of the same tumor, the poorly differentiated tissue, the cells are closely arranged, the volume is small, the edge is clear, the mucus component inside and outside the cell is less, and the mature tissue is differentiated. The cells are sparsely arranged, large in size, fusiform or polygonal, with obvious vacuoles in the cytoplasm, fibrous interstitial spaces, and a large amount of mucus accumulation. The mitotic phase is visible when the height is deteriorated.
Diagnosis
Diagnosis and diagnosis of chordoma
diagnosis
According to clinical manifestations, the characteristics of lesions and imaging examinations can generally be diagnosed. Most of the patients are middle-aged, showing localized pain and dysfunction. The tumors in the appendix produce various kinds of oppression. Symptoms, the anterior tibial tumor is more obvious than the growth to the dorsal side. X-ray films show localized bone destruction, bulging to one side, no ossification and calcification in the tumor, and anal finger examination often touches the mass in front of the tibia.
Differential diagnosis
Giant cell tumor of bone, neurofibromatosis and chordoma are common tumors that occur in the tibia. They have the same clinical symptoms. X-ray films are also osteolytic destruction. They are easily confused with each other and need to be identified, but the former two are mostly 20- to 40-year-old young and middle-aged, the giant cell tumor has obvious eccentricity; the destruction of neurofibromatosis surrounds the nerve hole, making it bigger and disappear, there are sclerotic bones around the lesion, other rare benign tumors, due to symptoms Mild, X-ray films have their own imaging features, easy to identify, less common sacral high malignant tumors have a short history, severe pain, affecting sleep, lying position can not afford to be forced position, patients quickly appear mentally weak, weight loss, Weight loss, anemia and fever, X-ray film tumors develop faster, osteolytic or osteogenic puncture points in the posterior median bone destruction, the positive rate can be as high as 90%, so the pathological histology obtained before surgery Diagnosis is not difficult.
It should also be differentiated from acute epidural abscesses, spinal tuberculosis or metastatic tumors.
