Ewing's sarcoma
Introduction
Introduction to Ewing's sarcoma Ewing's sarcoma (Ewing'ssarcoma), also known as undifferentiated reticuloma, is a primitive malignant tumor of the bone that originates in the bone marrow. Ewing (1921) first reported that it was named "diffuse hemangioendothelioma of bone". Later, Oberling (1928) believed that it originated from bone marrow reticular cells and called it "reticular sarcoma". However, people have long had disagreement on their tissues. The literature has always been named after surnames. Currently, Ewing's sarcoma has been recognized. Is an independent bone tumor, but there are still different opinions on its source and nature, such as mesenchymal cells, osteoblasts, accounting for 10% to 14.2% of malignant bone tumors, but this disease is rare in China. The age of onset is more common in adolescents, slightly more common in men, early and can occur lung metastasis, poor prognosis, but sensitive to radiation therapy. basic knowledge The proportion of sickness: 0.003%-0.005% Susceptible population: The age of onset is more common in adolescents, and is more common in men. Mode of infection: non-infectious Complications: anemia, pleural effusion
Cause
Ewing's sarcoma etiology
Cause:
The cause is not clear and may be related to radioactive factors and genetic factors.
pathology
(1) Seen by the naked eye: Tumors occur mostly in the trunk, spreading from the center of the backbone to the metaphysis, and are destroyed from the flesh. The tumor is nodular, soft in texture, without capsule, gray in white, and bleeding or necrosis in some areas. Dark red or brown, after tumor necrosis, can form a pseudocyst, filled with liquefied necrotic material, after the tumor destroys the cortical bone, it can invade the soft tissue, forming an "onion skin"-like layer of periosteal hyperplasia in the periosteum and its surroundings. This is the basis for the typical performance of the X-ray.
(2) Microscopic changes: the tumor cells are round or polygonal, the morphology is quite consistent, the cytoplasm is few, the staining is shallow, the membrane is unclear, the nucleus is round or elliptical, the size is relatively uniform, the particles are fine, and the distribution is uniform. The mitotic phase is more common. The cells in the tumor tissue are abundant, and the cells are arranged in a nest. Occasionally, about 20 tumor cells are arranged in a ring shape to form a "fake daisy-shaped group" structure. The tumor tissue often has large necrosis, and there may be a tumor around the tumor. New bone formation is a reactive new bone, not a component of the tumor itself.
Prevention
Ewing's sarcoma prevention
Avoid contact with radioactive materials and early detection and early diagnosis. Currently considered effective drugs for Ewing sarcoma are cyclophosphamide, doxorubicin, dactinomycin, vincristine, carmustine and the like. There are also many joint schemes, and the better ones are the CVD scheme (CTX+VCR+DACT+VCDA) and the CVDA scheme (adding ADM to the CVD scheme).
Complication
Ewing's sarcoma complications Complications anemia pleural effusion
Easy to have fever, infection and other diseases.
Symptom
Ewing's sarcoma symptoms common symptoms leukocytosis persistent pain, body discomfort, pleural effusion
1. Pain
It is the most common clinical symptom. About 2/3 of the patients may have intermittent pain. The degree of pain is different. It is not serious at the initial onset, but it quickly becomes persistent pain. Depending on the location, local pain will follow the tumor. Spread spread, such as occurs in the pelvic area, pain can be radiated along the lower limbs, affecting hip joint activity; if it occurs in the long bone adjacent to the joint, there will be lameness, joint stiffness, and joint effusion, this tumor rarely combined with pathological fractures, Located in the spine, it can produce radiation pain, weakness and numbness in the lower extremities.
2, lump
With the increase of pain, local lumps appear, the mass grows rapidly, the surface can be red, swollen, hot, painful inflammation, tenderness, venous engorgement on the surface, sometimes the mass grows in soft tissue, 2~3 Within a month, the head can be enlarged, which occurs in the tumor of the tibia. The mass can protrude into the pelvic cavity and can reach the mass in the lower abdomen or anus.
3, systemic symptoms
Patients are often accompanied by systemic symptoms such as elevated body temperature of 38 to 40 ° C, general discomfort, fatigue, loss of appetite and anemia.
In addition, the location of the tumor is different, can also cause other symptoms, such as lesions located at the lower end of the femur, can affect the function of the knee joint, and cause repeated effusion of the joint; lesions located in the rib can cause pleural effusion.
Examine
Examination of Ewing's sarcoma
1, X-ray performance:
It is an invasive destructive lesion in the long bone marrow cavity. Although the spot-like or wide-range flaky osteolytic changes are most prominent, the lesion range and edge are blurred, and the skin is very irregularly defective and damaged. The periosteal reaction is much earlier. Reactive new bone formation produces Codman triangle, or the periosteal reaction is concentric, and the layered arrangement changes like onion skin. The former is more common than the latter. Although the long bones are mainly found in the backbone, it is not uncommon to originate from the dry end. Soft tissue lumps with clear boundaries are sometimes displayed outside the bone. Mainly manifested as bone destruction and soft tissue mass, and in some cases, bone marrow sclerosis can also be seen.
2, laboratory inspection:
There may be anemia, leukocytosis and accelerated erythrocyte sedimentation rate. Serum lactate dehydrogenase activity can also increase white blood cells often increase by up to 10,000 to 30,000. Due to the formation of a large number of new bone periosteum, serum phosphatase can be slightly increased, which has great diagnostic significance for adults. Tumor glycogen staining was positive, which was different from the negative staining of stromal cell sarcoma. There is also information that the positive test of this week has a certain diagnostic value for this tumor.
3. Transfer:
The tumor develops rapidly, and can be widely metastasized through the bloodstream at an early stage, often metastasized to organs such as the lungs and liver, but rarely to local lymph nodes. The tumor has a tendency to easily metastasize to other bones and significantly invade other bones, suggesting its multicentric origin.
4, angiography:
Angiography is very valuable for diagnosis. 90% of the lesions can show an increase in blood vessels and expansion.
5. CT and MRI:
Can better determine the extent of the tumor and invasion of soft tissue. MRI showed extensive bone destruction at the tumor, showing a soft tissue mass; a uniform long T1 signal on the T1-weighted image; and a long T2 high signal on the T2-weighted image. On the CT, it is shown as a soft tissue mass derived from bone tissue, and the bone is extensively destroyed.
6, nuclear bone scan:
Not only can the extent of the primary lesion be displayed, but other lesions throughout the body can also be found.
Diagnosis
Diagnosis and diagnosis of Ewing's sarcoma
diagnosis
Histologically, Ewing's sarcoma is difficult to distinguish from metastatic neuroblastoma, primary reticulum sarcoma, multiple myeloma, and metastatic osteoma. As noted above, a diagnosis requires a combination of clinical, radiological, and histological examinations. Children under 5 years of age need to be differentiated from neuroblastoma, which has catecholamine in the urine, with a positive rate of 90%. The long bone metastasis of neuroblastoma is mostly symmetrical. Although there is extensive bone destruction, the soft tissue mass is still small, and the primary tumor can often be found by intravenous pyelography or paravertebral mass.
Differential diagnosis
Mainly associated with acute suppurative osteomyelitis, primary reticulocyte sarcoma, neuroblastoma bone metastasis, and osteosarcoma.
(1) acute suppurative osteomyelitis: the disease is acute, often accompanied by high fever, pain is more severe than Ewing's sarcoma, often accompanied by jumping pain, suppuration is not aggravated, some cases with other parts of the chest infection, early X-ray film The affected bone changes are not obvious, and then there will be spotted sparse destruction in the cancellous bone of the medullary cavity. At the same time of bone destruction, bone hyperplasia will occur soon, and more dead bones will appear; puncture examination, in the early stage of osteomyelitis Bloody liquid or purulent liquid is aspirated, positive for bacterial culture, and Ewing's sarcoma is no, exfoliative cytology is helpful for diagnosis, and osteomyelitis has obvious effect on anti-inflammatory treatment. Ewing's sarcoma is extremely sensitive to radiotherapy.
(2) Primary reticulocyte sarcoma: more common in 30~40, long course, general condition, clinical symptoms are not heavy, X-ray shows irregular osteolytic destruction, sometimes ice-like, no periosteum Reaction, pathological examination, multi-nuclear nucleus, pleomorphic, reticulated fiber is rich, surrounded by tumor cells, histochemical examination, no glycogen in the patina.
(3) neuroblastoma bone metastasis: more common in children under 5 years old, 60% from the retroperitoneal, 25% from the mediastinum, often no obvious symptoms of primary disease, metastatic pain, swelling, and more pathological fractures, Urine examination of catecholamines is elevated, often difficult to identify on X-ray films; pathologically neuroblastoma cells are pear-shaped, forming a true chrysanthemum; under the electron microscope, there are secretory granules in the tumor cells.
(4) Osteosarcoma: The clinical manifestations of fever are mild, mainly pain, heavy at night, and the tumor penetrates the cortical bone into the soft tissue. The formed mass is mostly on the side of the bone. There is ossification in the skin. The size of the bone reaction is often inconsistent. Common Codman triangles and radial bone needles change, and the pathological tumor cells are not arranged in a pseudo chrysanthemum.
