chondrosarcoma
Introduction
Introduction to chondrosarcoma Chondrosacomaofbone is a malignant tumor originating from cartilage tissue. Most of it is secondary to benign cartilage tumors, such as endogenous chondromas and osteochondromas. The types are complex and sometimes cause difficulty in diagnosis. According to the development process of the tumor, it can be divided into primary and secondary. The former has an early onset age, high malignancy, rapid development and poor prognosis. The latter is a malignant change of benign tumors such as osteochondroma and chondroma. Later, the development is slow and the prognosis is slightly better, accounting for 4% of chondrosarcoma. basic knowledge The proportion of illness: 0.032% Susceptible people: no special people Mode of infection: non-infectious Complications: fracture
Cause
Chondrosarcoma etiology
(1) Causes of the disease
The cause of the disease is unknown. The tumor is derived from chondrocytes or mesenchymal tissue and originates from the bone of any cartilage internalized bone. Giovanni Tallini and other scholars have found that patients with chondroma often have a chromosome 7, GIII cartilage. Tumor patients often have abnormalities of 17p1, suggesting that the CerbB-2 oncogene is abnormally expressed, and the number of chromosome 7 is increased. The change of 17p1 may be related to the occurrence of chondrosarcoma.
(two) pathogenesis
1. The cause of chondrosarcoma is unknown. Experimental pathology suggests that chondrosarcoma is associated with viral infection, while marginal chondrosarcoma is associated with genetic factors.
2. The gross pathological chondrosarcoma is large in most tumors. The maximum diameter of the tumor is more than 4cm, and the larger one can reach more than 20cm. It is reported that 50% of the chondrosarcoma has a maximum diameter of more than 10cm, which occurs in the tibia, scapula, ribs, etc. Chondrosarcoma on flat or irregular bone, often with clinical symptoms when the tumor grows large, central chondrosarcoma often occurs in long tubular bone and often causes destruction of the cortical bone and invasion into soft tissue, which is different from An important feature of chondroma, peripheral chondrosarcoma is a large extranodal nodular tumor. Even if it invades soft tissue, it often has a good dividing line. The thickness and shape of the cartilage cap are helpful for diagnosis. The thickness of the cartilage cap in adult benign osteochondroma is often less than 1cm. If the thickness of the cartilage cap is greater than 1cm, it is suspicious. If it is greater than 2cm, it may be malignant. In children and adolescents, their benign osteochondroma cap In addition, the thickness of the typical benign osteochondroma cap is uniform and smooth, but in chondrosarcoma, it is irregular, rough or Granular.
In the cut surface of the gross specimen, the tumor is lobulated, grayish white or gray-blue with hyaline hyaline cartilage. The content of the tumor is solid, but it is easy to cut in areas without calcification. In some cases, there may be bleeding, mucus-like Change and cystic changes, the intramedullary boundary of central chondrosarcoma, difficult to distinguish by the naked eye, because its edges are often blurred, this is different from osteosarcoma, the latter's tumor range is often easy to determine, in differentiation In good chondrosarcoma, there are often spotted yellow to yellow-white calcifications.
3. Microscopy In chondrosarcoma, the diagnosis of highly malignant and poorly differentiated chondrosarcoma is easy, and it is difficult to distinguish between low-grade and well-differentiated chondrosarcoma and endogenous chondroma. It is suggested that chondrosarcoma-based tumors The number of cells, the degree of cell differentiation, the degree of mitosis and other histological features, can be divided into grade 4 (I ~ IV), chondrosarcoma, the smaller the number, indicating that the degree of tumor differentiation is high, invasive is small, according to statistics, most cases belong to I, II level.
Usually, chondrosarcoma is rich in round to oval cells with fat nucleus, and the nucleus is deeply stained; there are a large number of binuclear cells, mononuclear or multinuclear giant tumor cells. In highly malignant tumors, this cell has Polymorphism, sometimes mitosis can be seen, chondrosarcoma is mostly composed of cartilage lobes, which are different from those usually seen in benign endogenous chondromas, without the surrounding boundary composed of woven bone or lamellar bone, benign osteochondroma The histological identification before and after malignant transformation is mainly based on the arrangement of cells. In the chondrosarcoma, the cells are arranged in disorder, and in the osteochondroma, the chondrocytes are arranged in a granular shape.
Prevention
Chondrosarcoma prevention
Because it is a chromosomal abnormal hereditary disease, there is no effective preventive measure.
Complication
Chondrosarcoma complications Complications
Later, it can cause pathological fractures.
Symptom
Chondrosarcoma Symptoms Common Symptoms Radioactive Pain Joint Pain Bone Cyst Joint Swelling Bone Pain Fibrous Structure
1. Symptoms and signs
Chondrosarcoma is generally slow, the most common symptom is pain, which begins with dull pain, intermittent, gradually worsening, followed by a slowly growing mass. The symptoms exist for an average of 1 to 2 years. The tender mass, the joint can be restricted in activity, and the mass can be touched with heat.
2. Good hair
In large case statistics, long tubular bone is a good site for chondrosarcoma, accounting for about 45% of all cases, of which femur is the most common part, accounting for about 25% of all patients, and the lower extremity bones account for about chondrosarcoma. 35%, and the upper limbs were 14%. The remaining common sites were the tibia (25%) and the ribs (8%). The less common sites were the spine (7%), the scapula (5%), and the sternum (2%). ), rare parts are the skull, mandible, maxilla, humerus, humerus, ulna, clavicle, humerus and small bones in the hands and feet.
Most of the chondrosarcoma in the long tubular bone is located at the metaphysis, but when the epiphysis is closed, the tumor can invade the epiphysis. The primary is rare in the diaphysis, and the proximal end of the femur, tibia, tibia and tibia is more common. At the distal end, the distal cartilage sarcoma of the humerus is rare.
In addition to hematological tumors, chondrosarcoma is the most common malignant tumor of the scapula, ribs, sternum, small bones of the hands and feet, and chondrosarcoma of the ribs and sternum often occurs at the junction of the costal cartilage. In the hand, chondrosarcoma occurs in the near The phalanx and metacarpal bones, but the distal phalanges and carpal bones are rare. In addition to the talus and calcaneus, chondrosarcoma rarely occurs in the bone below the calf joint. Chondrosarcoma can occur in various segments of the spine, but The thoracic vertebrae are the most common and often located in the vertebral arch and spinous processes.
Primary chondrosarcoma, the age of onset is generally under 30 years old, more men than women, occurs in the long bones of the extremities, especially the lower end of the femur, the upper end of the humerus and the upper end of the humerus are the most common, other such as ribs, tibia The scapula or sternum also has an onset, which is rare in short bones. The main symptom is blunt-like blunt pain, which can be changed from intermittent to persistent, and affects the adjacent joints to restrict their activities, sometimes local spasms and masses. However, there is no obvious tenderness, and the surrounding skin may have congestion and redness.
Secondary chondrosarcoma is generally more than 30 years old adults, more common in men, occurs in the pelvis, followed by the scapula, femur and tibia, the main manifestation of the mass, slow course, pain is not obvious, no red heat around the skin, When the joint is close to the joint, it can cause joint swelling and limited activity. For example, stimulating the nerve can cause radiation pain, numbness, etc. The tumor located in the chest and pelvis is generally difficult to find until the tumor compresses the internal organs, and the corresponding symptoms are discovered.
3. Classification
The biological behavior of chondrosarcoma is variable, which has certain difficulties in judging the prognosis. It is commonly used in histological grading, and also in combination with biochemical indicators. Chondrosarcoma is histologically divided into transparent, mucinous, fibrocartilage, mixed and Transparent cell type, it is generally believed that the degree of malignancy of the transparent type is low, while the fiber type, fibrocartilage type, and mixed type are highly malignant. From the onset of the disease, the chondrosarcoma is divided into two major categories: primary and secondary. Primary features sarcoma characteristics from the beginning, secondary refers to secondary irradiance after irradiation, malformation osteitis, poor fibrous structure, solitary bone cyst, Maffucci syndrome, Ollier disease, multiple hereditary callus, cartilage Maternal tumor, cartilage myxofibrosis, etc., derived from benign cartilage tumors, from the site, chondrosarcoma is divided into central and peripheral type; as well as paracortical or periosteal chondrosarcoma, and extramedullary mucin-like cartilage Sarcoma, etc., in addition, there are dedifferentiated chondrosarcoma, mesenchymal chondrosarcoma and clear cell chondrosarcoma.
According to typical symptoms, signs, X-ray examination, radionuclide scanning and CT, general diagnosis is not difficult.
Examine
Examination of chondrosarcoma
No special examination items, Marcove et al. performed glucose metabolism tests on 75 patients with chondrosarcoma and found that there was a decrease in intravenous glucose tolerance.
1.X-ray performance
(1) Central type
More common than the surrounding type, the primary is more, the primary, the tumor occurs in the metaphyseal medullary cavity, showing a large single- or multi-room translucent area, irregular edges, irregularities between them Calcification points and broken bone fragments, sometimes a large number of flocculent calcifications and ossified plaques cover the bone area, forming a uniform shadow, tumor swelling and growth, can make the cortical bone thin, once the tumor penetrates the cortical bone or concurrent pathology Fracture, the tumor quickly invades the surrounding soft tissue, forming a soft tissue mass, which can be seen in the calcification point. Due to the stimulation of the tumor, there is often a multi-layered new bone formation under the periosteum, which causes the cortical bone to thicken or the tumor to wear through the new bone. "Cuff" sign, the X-ray of secondary tumors is: the osteolytic translucent shadow of the tumor increases, the edge is blurred, the calcification point increases, the calcification shadow disappears in the aggregated pile, and finally the tumor penetrates the cortex, in the soft tissue. Development and formation of calcification shadows, the central manifestation of chondrosarcoma is a large-volume thick-wall translucent area, trabecular formation and central multi-lobed intramedullary bone destruction, there are many scattered in the area The punctate, ring-shaped or flaky calcifications are often described as "cotton-like", "bread-like" or "popcorn", and in the later stages, the cortical bone is destroyed, and the tumor penetrates the cortical bone. It is blurred, there is tumor infiltration in soft tissue, but there is not necessarily a calcification shadow with increased density, and the periosteal reaction is less. The cortical bone on the endosteal side often has a shell-like depression, which is caused by the lobular contour of the tumor and pathological fracture. The tumor can be quickly penetrated into the soft tissue, and a dense shadow of calcification appears in the extra-bone mass.
(2) Peripheral type
Soft central type is rare, its recurrence is more than the primary, malignant changes of osteochondroma, often on the basis of primary osteochondroma, the cartilage cap is enlarged and thickened, forming a cartilage tissue with blurred boundaries, tumor In the soft tissue inside and around, there are a lot of scattered spot-like or flocculent irregular calcification, sometimes there may be thick and long new bone formation, and the bone part can also show signs of trabecular disturbance and destruction. Rapid growth and the typical chondroma disappeared due to tumor destruction. In a few cases, cuff-like periosteal reaction can occur. Peripheral chondrosarcoma shows that the soft tissue next to the lesion is very light, with little calcification. Shadows, and radial spurs perpendicular to the surface, their outer sides become flat, which is the identification point of the radial spurs of osteosarcoma, the medullary cavity is generally not affected, the cortical bone is rarely invaded, but can be seen in early cases The epithelium is picked up and has a lip-like appearance. Codman triangles can also appear.
2. Radionuclide scanning can be used to examine central and peripheral chondrosarcoma. In central osteosarcoma, there is always nuclear enrichment in the lesion, and the range of nuclide aggregation does not exceed the true limit of the tumor. Radionuclide scanning is very reliable for determining the boundary of central chondrosarcoma and for finding concealed disseminated lesions. In peripheral chondrosarcoma, radionuclide scanning can clarify the metabolic activity of the tumor. For an osteochondroma, in the examination If there is no nuclear enrichment of radionuclides, the possibility of malignant transformation of osteochondroma can be virtually eliminated.
3. CT examination is used in both central and peripheral chondrosarcoma. It can understand the extent of tumor in bone and soft tissue. If the growth of soft tissue mass of tumor is biased to one side, it is lobulated, indicating that the tumor is Growth along the direction of least resistance can suggest a low-grade malignancy. If the soft tissue mass of the tumor grows in all directions without being limited by the anatomical boundary, the tumor is highly malignant, and the cap of the peripheral chondrosarcoma The thickness of the lid can help to analyze the presence of osteochondromas with or without malignant transformation.
4. Microscopic examination mainly shows that the tumor tissue is disorderly arranged, the chondrocytes of different sizes, and a few mucous-like cells are widely scattered in the matrix of the cartilage tissue, and some cartilage has irregular calcification or ossification, the same tumor Different parts, the degree of differentiation is not the same, and needs to be carefully examined.
Diagnosis
Diagnosis and diagnosis of chondrosarcoma
Differential diagnosis
1. A benign tumor of chondroblastoma, which is considered by most scholars to be derived from osteophyte cartilage. The course of disease is longer, with an average of more than 2 years. The common site is the central or eccentric growth of the long bone end, which is characterized by round or oval-shaped bone-like destruction. The cortical bulge is thinner and can have irregular calcification and rough texture, but its boundary is clear. The general specimen sees the surrounding hardening, the irregular wall of the tumor wall is bulged, and the outer cortex is thinner or only the fibrous capsule.
2. Endogenous chondroma is a common benign cartilage tumor, including isolated endogenous chondroma and multiple endogenous chondroma. Some scholars believe that the disease is characterized by abnormal cartilage dysplasia, common parts are hands and feet. Small bone, isolated or multiple osteolytic changes, scattered calcification, pathological fractures, but the tumor does not invade soft tissue.
3. Osteochondroma is the most common benign bone tumor. It is a "precancerous lesion" of marginal chondrosarcoma. It occurs in the long tubular trunk and is a slow-growing painless mass. X-rays are like antler-like. Some are like a small beak-like protrusion, some like a hill-like bulge, the tenderness is not obvious, when the tumor grows rapidly in a short period of time, the pain is intensified, and the thickness of the adult cartilage cap is greater than 1.0cm, and the size of the child and adolescent is 3.0cm. Consider malignant changes.
4. The chondroma tumor also has scattered sand-like calcification points, but it is smaller and less than the chondroma, the cortical bone is intact, and there is no periosteal reaction.
5. Osteosarcoma This is composed of tumorous bone-like tissue and bone tissue evolved from sarcomatous connective tissue. The tumor bone contained in osteosarcoma is characteristic.
6. Cartilage fibrosarcoma is benign from the perspective of tissue arrangement, and the recurrence rate is low.
