Pediatric soft tissue sarcoma

Introduction to pediatric soft tissue sarcoma Soft tissue sarcoma (softtissuesarcoma) is a malignant tumor that occurs in connective tissue, including subcutaneous tissue, muscle, tendon, blood vessels, connective tissue space, and cavity organ matrix. However, it occurs in the bone, reticuloendothelial system, glial and other parts. The cells of soft tissue sarcoma originate from primitive mesenchymal stem cells, which are located in non-segmental mesoderm and can grow in different parts of the body. The clinical manifestation of soft tissue sarcoma is a mass, but the mass itself has no function, so only when the mass enlarges and compresses the surrounding tissue, it produces symptoms. Including fibrosarcoma, malignant fibrous histiocytoma, liposarcoma, muscle tissue tumor, angioendothelioma, angiosarcoma, synovial sarcoma, malignant peripheral nerve sheath tumor, alveolar soft tissue sarcoma, dermatoid sarcoma, etc., which is diagnosed by pathology Sliced, but without characteristic histochemistry or immunology, biological markers. The treatment of soft tissue sarcoma is mainly surgical resection, the degree of malignancy is quite different, and the effects of radiotherapy and chemotherapy are not the same for each specific tumor. Treatment with a whole tumor resection or amputation, chemotherapy alone can make the tumor shrink, there are reports of radiotherapy can be long-term treatment, the more the tumor is in the proximal end of the limb, the worse the prognosis, and if there is tumor necrosis, bleeding, invasion Blood vessels and lymph nodes have a poor prognosis. basic knowledge Sickness ratio: 0.01%-0.02% Susceptible people: children Mode of infection: non-infectious Complications: tonsillar carcinoma retinoblastoma

Pediatric soft tissue sarcoma etiology

(1) Causes of the disease

Fibrosarcoma

Modern fibrosarcoma refers to a malignant tumor composed of fibroblasts and the fibers they produce. The main component is the degenerative spindle cells that maintain the ability to produce reticulin. Virchow (1863) first proposed the diagnosis of fibrosarcoma, but Significantly different from modern meaning, other types of tumors derived from facultative fibroblasts have been excluded.

2. leiomyosarcoma

Leiomyosarcoma has been reported in the mid-19th century. It is generally believed that mesenchymal cells derived from smooth muscle cells or directed muscle smooth muscles have unclear etiology of leiomyosarcoma. Trauma, ionizing radiation or other damage have an effect on tumor development. Recently, studies have indicated that Epstein-Barr virus is a risk factor for leiomyosarcoma in immunodeficient patients.

3. Malignant peripheral nerve sheath tumor

Intrinsic cellular components originating from the peripheral nerve sheath, which should exclude tumors of the neuroepithelium (neural sheath) or peripheral neurovascular system, although many examples suggest that malignant peripheral schwannomas are derived from Schwann cells, and some may also show fibroblasts. Or evidence of peripheral nerve cell differentiation, however, some people disapprove of the use of the name of peripheral schwannomas, which suggests that malignant Schwannoma appears to be better.

4. Liposar sarcoma

It is a malignant tumor characterized by differentiation of fat cells, and irregular fat cells appear at different stages of differentiation.

5. Acinar soft tissue sarcoma

Primary in the mesenchymal tissue, but it has a saclike (organ-like) structure, which is different from the usual arrangement of sarcoma cells. Christopherson et al (1952) first reported the disease with acinar sarcoma. The cause of this tumor is unclear. There are several hypotheses about the origin of the organization, but they are inconclusive. The WHO (1992) classification is classified into other categories.

6. Vascular epithelioma

Very rare, characterized by the proliferation of tumor cells outside the basement membrane, which can be benign or malignant, and malignant can be called vascular sarcoma, also known as angioendothelioma.

7. Angiosarcoma (angiosarcoma)

It refers to vascular sarcoma, which should include angiosarcoma (hemangiosarcoma) and lymphangiosarcoma (lymphangiosarcoma), because lymphangiosarcoma is very rare, so only the former is described.

8. Synovial sarcoma

Sabrazes (1934) first used the term synovial sarcoma, which was mistaken for the synovial membrane of the joint. Later, numerous studies found that there were epithelial glands in the tissue structure, and occasional interstitial, fusiform sarcoma components showed angles. Protein and epithelial membrane antigen, it can be seen that its tissue source has nothing to do with the synovial membrane of the joint. It is now classified as an unidentified tumor. The name still uses synovial sarcoma. The important feature of histology is biphasic differentiation.

9. Epithelioid sarcoma

The tissue origin of epithelioid sarcoma is unknown. Because of the similar epithelial-like cells under the microscope, Enziger et al. (1970) officially named epithelioid sarcoma.

10. Lymphatic sarcoma

Tumors originate from the lymphatic endothelium, and the cause is related to chronic lymphedema caused by radiotherapy and other causes. Congenital lymphedema can also occur.

11. Malignant mesothelioma

Malignant mesothelioma (malignant mesothelioma), rare in children, has reports of family morbidity, and some people believe that it is related to exposure to asbestos and cerium oxide.

(two) pathogenesis

1. Staging of soft tissue sarcoma

The staging method proposed by Ennrking (MTS) in the early 1980s is simple and clear. It highlights the characteristics of sarcoma. It is summarized as GTM system and has many clinical applications. In 1987, MTS and AJC unified their staging. The details are as follows: :

Phase I: IA: G1T1N0 M0 G1: Low grade.

IB: G1T2N0 M0 G2: Moderately malignant.

Phase II: IIA: G2T1N0 M0 G3: Highly malignant.

IIB: G2T2N0 M0 T1: indoor.

Phase III: IIIA: G3T1N0 M0 T2: Outdoor.

IIIB: G3T2N0 M0 N0: no lymph node metastasis.

Stage IV: IVA: G1 ~ 3T1 ~ 2N M0 N1: lymph node metastasis.

IVB: G1 to 3T1 to 2N0 to 1 MLM0: no distant transfer.

ML: Remote transfer.

(The meaning of G includes histology, clinical and imaging findings).

2. Pathological changes

(1) fibrosarcoma: the mass is round or elliptical, the face is gray or yellowish white, the size is different, the small tumor seems to have a capsule, the large boundary is unclear, often infiltrating the tissue, and the tumor is interlaced by a capsule under light microscope. The arranged shuttles form fibroblasts with less cytoplasm, and the bundles of cells are separated by collagen fibers. The histological morphology is relatively uniform. Congenital and infant fibrosarcoma cells are more mitotic, and lymphocytes may be scattered.

(2) malignant fibrous histiocytoma: the tumor is mostly nodular, different in size, 2 ~ 20cm, more without capsule, the cut surface is grayish white, fish-like, may have hemorrhagic necrosis, sometimes contains cystic cavity, the main tumor under the microscope It is composed of tissue-like cells and fibroblast-like cells. It is pleomorphic. Typical and atypical mitotic figures are more common in tissue-like cells. Tumors are often found in limbs, followed by trunk, intra-abdominal, retroperitoneal, skeletal, and breast. It is possible that the mass is a single nodular shape, which can be rarely seen at the beginning, but it is often easy to make local deep fascia, sometimes ulcerated, and the tumor can be transferred to regional lymph nodes and distant areas at an early stage, and deep tumors can be distantly metastasized. The symptoms of the stove precede the condition of the primary.

(3) liposarcoma: liposarcoma is divided into 5 types of tissue: well-differentiated type, mucin-like type, round cell type, polymorphic type and mixed type. This type is related to prognosis, and the well-differentiated type has the best prognosis. This type and mucus type have good prognosis.

(4) leiomyosarcoma: generally divided into three categories:

1 retroperitoneal and intraperitoneal (large omentum, mesentery, gastrointestinal tract and other organs).

2 skin and subcutaneous tissue.

3 vasogenic leiomyosarcoma.

The tumors are tough and vary in size. The center is often necrotic and covered with no membrane. Under light microscopy, the cells are long-spindle type, rich in cytoplasm and more common in mitotic figures.

(5) Infant hemangiopericytoma: The tumor is multi-atrial, the size is different, the boundary is clear, the capillaries in the tumor are tightly clustered under the microscope, the tumor is composed of flat cells of blood vessels, a large number of epithelial cells proliferate between the blood vessels, and the tumor cells are directed to the blood vessels. In edema, mitosis and necrosis can be seen. In children, this does not suggest malignancy. In a few cases, the tumor can infiltrate into surrounding tissues, and even distant metastasis is definitely malignant.

(6) malignant hemangioendothelioma: the occurrence of this tumor is considered to be related to chronic lymphedema. It is also believed that radioactive ray radiation can cause this tumor. The diameter of the tumor is in the range of a few centimeters, the cut surface is gray or grayish red, the shape is irregular, and the shape is soft. The cavity can be seen in different sizes of the cavity. Under the microscope, the extensively proliferating endothelial cells can be seen. The structure is irregularly shaped, and the cells are anastomosed into a net. The nuclear division is common.

(7) Lymphatic sarcoma: Irregular vasculature is seen in the tumor, which is lined with malignant endothelial cells. The cells are oval or prismatic cytoplasm, and the factor VIII-related antigen is also a marker of this tumor.

(8) Synovial sarcoma: The tumor mainly occurs in the joint, which is a round or multi-atrial mass, attached to the tendon around the joint, the outer part of the tendon sheath or joint capsule, the cut surface is yellow or grayish white, and there are two kinds of tumor under light microscope. Morphological cells, such as cancerous epithelial cells and prismatic cells like microsarcoma.

(9) Malignant mesothelioma: The tumor mainly occurs in the serosa of the body cavity, such as the pleura, peritoneum, pericardium, testicular sheath, etc. The pleura of children is the most common, accounting for about 85%. The histology is divided into: Epithelial type, fiber type and epithelial and fiber mixed type, under the light microscope, there are many papillary cells and fibrosarcoma cells in the tumor, nucleoli are prominent, and nuclear mitosis is more common.

(10) Maglicn schwannoma: The typical lesion is a large nerve path with a diamond-shaped mass. The distal and proximal ends of the spinal nerve are evenly thickened, indicating that the tumor spreads to the epicardium. The tumor is generally larger than 5 cm. The surface is succulent and may have hemorrhagic or necrotic areas. It is similar to fibrosarcoma under light microscopy, but the cells of this tumor have irregular contours, and the nucleus is corrugated, curved or comma-shaped.

(11) malignant mesenchymal tumor (malignant mesenchymoma) is a soft tissue sarcoma from the mesenchymal component, which is rare in children, more men than women.

Tumors have a variety of malignant soft tissues. Rhabdomyosarcoma, hemangioendothelioma, synovial sarcoma, malignant schwannosarcoma and leiomyosarcoma are the most common. Because of histological diversity, the performance is also diverse. In general, it resembles rhabdomyosarcoma.

(12) Acinar soft tissue sarcoma: Alveolar soft tissue sarcoma is a clinical-pathological entity, rare in children, more common in adolescents around 15 years old, more women than men, no obvious boundaries of tumor, soft And fragile, the cut surface is yellowish white or grayish red, the center contains necrotic and hemorrhagic areas, there are thick blood vessels around the tumor, and the dense trabeculae under the light microscope divides the tumor into different sizes of cavities, and the lumens have distinct boundaries. Tumor cell island, necrosis and shedding in tumor cells.

(13) Epithelioid sarcoma: Epithelioid sarcoma is a rare sarcoma that occurs in adolescents. More men than women, this tumor accounts for about 1% of all soft tissue tumors.

The tumor is one or several lumps located in the deep part, 0.5 to 5 cm in diameter, closely related to the tendon or fascia. The tumor is smooth or lobulated, solid, the edges are unclear, the cut surface is sticky, and the gray-white interval is yellow-brown. In the hemorrhage area, under the microscope, the cells are clearly arranged in a small knot, the center has a tendency of necrosis and degeneration, and the cytoplasm is eosinophilic, showing epithelial morphology, and mitosis is more common.

Pediatric soft tissue sarcoma prevention

1. Avoid harmful substances (promoting factors)

It is able to help us avoid or minimize exposure to harmful substances.

Some related factors of tumorigenesis are prevented before the onset, and many cancers can be prevented before they are formed. A report in the United States in 1988 compared the international malignant tumors in detail and proposed many external factors of known malignant tumors. In principle, it can be prevented, that is, about 80% of malignant tumors can be prevented by simple lifestyle changes, and traced back. In 1969, Dr. Higginson's research concluded that 90% of malignant tumors are caused by environmental factors," "Environmental factors", "lifestyle" refers to the air we breathe, the water we drink, the food we choose to make, the habits of activities, and social relationships.

2. Improve the body's immunity against tumors

Can help to strengthen and strengthen the body's immune system and cancer.

The focus of our current cancer prevention efforts should first focus on and improve those factors that are closely related to our lives, such as quitting smoking, eating properly, exercising regularly, and losing weight. Anyone who follows these simple and reasonable lifestyles can Reduce the chance of cancer.

Promote the body's immunity against cancer: The most important thing to improve the function of the immune system is: diet, exercise and control troubles, healthy lifestyle choices can help us stay away from cancer, maintain good emotional state and appropriate physical exercise can make the body The immune system is at its best, and it is also good for preventing tumors and preventing other diseases. In addition, studies have shown that appropriate activities not only enhance the body's immune system, but also reduce the incidence of colon cancer by increasing the peristalsis of the human intestinal system. Here we mainly understand some of the problems of diet in preventing tumors.

Human epidemiology and animal studies have shown that vitamin A plays an important role in reducing the risk of cancer. Vitamin A supports normal mucosa and vision. It directly or indirectly participates in most of the body's tissue functions. Vitamin A is present in animal tissues. In the liver, whole eggs and whole milk, the plant is in the form of -carotene and carotenoids, which can be converted into vitamin A in the human body. Excessive intake of vitamin A can cause adverse reactions in the body and -carrot This is not the case with carotenoids, and the low vitamin A content in the blood increases the risk of malignant tumors. Studies have shown that those with low levels of vitamin A intake in the blood increase the likelihood of lung cancer, while those with low blood levels in smokers Levels of vitamin A ingestors have the potential to double lung cancer. Vitamin A and its mixture can help remove free radicals in the body (free radicals can cause damage to genetic material), and secondly stimulate the immune system and help differentiate cells in the body. Ordered tissue (while the tumor is characterized by disorder), some theories suggest that vitamin A can help early carcinogens Invasion mutated cells become reversed the occurrence of the normal growth of cells.

In addition, some studies suggest that supplementation with -carotene alone does not reduce the risk of cancer, but rather increases the incidence of lung cancer. However, when -carotene binds to vitamin C, E and other antitoxin substances, its protective effect. It is shown, because it can increase free radicals in the body when it is consumed by itself. In addition, there are interactions between different vitamins. Both human and mouse studies have shown that the use of -carotene can reduce 40% of vitamins in the body. At E-level, a safer strategy is to eat different foods to maintain a balanced vitamin to protect against cancer, as some protective factors have not been discovered so far.

Vitamin C, E is another anti-tumor substance that prevents the harm of carcinogens such as nitrosamines in food. Vitamin C protects sperm from genetic damage and reduces the risk of leukemia, kidney cancer and brain tumors in their offspring. Vitamin E can reduce the risk of skin cancer. Vitamin E has the same anti-tumor effect as vitamin C. It is a scavenger that protects against toxins and scavenges free radicals. The combination of vitamins A, C and E protects the body against toxins. Better than applying it alone.

At present, research on phytochemistry has attracted widespread attention. Phytochemistry is a chemical found in plants, including vitamins and other substances found in plants. Thousands of plant chemicals have been found, many of which have anticancer properties. The protective mechanism of these chemicals not only reduces the activity of carcinogens but also enhances the body's immunity against carcinogens. Most plants provide antioxidant activity that exceeds the protective effects of vitamins A, C, and E, such as a cup of cabbage. Contains 50mg of vitamin C and 13U of vitamin E, but its antioxidant activity is equivalent to the antioxidant activity of 800mg of vitamin C and 1100u of vitamin E. It can be inferred that the antioxidant effect in fruits and vegetables is far better than what we know. The effect of vitamins is strong, and no doubt natural plant products will help prevent cancer in the future.

Pediatric soft tissue sarcoma complications Complications tonsillar carcinoma retinoblastoma

Fibrosarcoma can occur in any part of the body, with up to 50% of the lower extremities. Mainly the feet, ankles and calves, so the upper limbs are more hands, wrists and forearms, the trunk, the retroperitoneum and the parotid gland, the oral mucosa, the tonsils, the mastoids, etc. can also grow this tumor. Burn scars, retinoblastoma, Hodgkin's lymphoma are easy to grow this tumor after radiotherapy. The tumor is a painless mass that grows rapidly and can be doubled in 2 to 3 weeks. The boundary is often unclear. When the nerve is compressed, such as the peroneal nerve or its branches, compression symptoms can occur. It can produce various types of compression or local aggression, and metastasis can occur.

Symptoms of soft tissue sarcoma in children Common symptoms Tumors are pulled by tumors

Fibrosarcoma

Fibrosarcoma originates from fibroblasts and is a malignant tumor of fibrous tissue. It is more common in children under 5 years old. Some children are found at birth, also known as "congenital and infant fibrosarcoma". Boys are slightly more than girls.

Fibrosarcoma can occur in any part, the lower extremity is about 50%, mainly the foot, ankle and calf, so the upper limbs are more hands, wrists and forearms, trunk, retroperitoneum and parotid gland, oral mucosa, tonsil, mastoid, etc. Can also grow this tumor, burn scar, retinoblastoma, Hodgkin's lymphoma is easy to grow this tumor after radiotherapy, the tumor is a rapid growth of painless mass, can be doubled in 2 to 3 weeks, its boundary is often Unclear, when the nerve is compressed, such as the peroneal nerve or its branches, it can produce symptoms of compression. There are several reports of the simultaneous separation of several distant tumors of the same limb. X-ray examination shows that the soft tissue block or the long bone cortex of the limb is thickened. Very few bone destruction can occur, and lung metastases can occur in infants.

Treatment should be extensive and complete resection of the tumor, including at least 3cm of tissue around the tumor, resection can not cause recurrence, surgery is required, if necessary, amputation is considered, fibrosarcoma is low in radiosensitivity, so there is advocated high-dose radiotherapy (6Gy) Especially in the case of multiple recurrences or avoidance of amputation, the opinions of chemotherapy are inconsistent. Some people advocate the use of rhabdomyosarcoma program in recurrence and metastasis cases, and some advocate preoperative radiotherapy and chemotherapy.

2. Malignant fibrous histiocytoma

Most of the first diagnosis of painless mass, the tumor is often larger, the boundary is clear, harder, more deep, regional lymph nodes can be swollen, when the adjacent bone is invaded, the tumor can be fixed, accompanied by tenderness, malignant fibrous histiocytoma (malignant fibrous histiocytoma, MFH), also known as fibrous histiocytoma, fibrinous sarcoma, malignant fibrous xanthoma, etc., first described by OBrien Stout in 1964, more common in middle-aged and elderly, children are less common.

3. liposarcoma (liposarcoma)

The larger and deeper lumps found by chance occur mostly in the deep muscles of the deep fascia or in the vicinity of the intermuscular fascia vessels and nerves, the retroperitoneal space, the flexion of the main joints of the extremities and the inner thighs, and the tumor boundaries are unclear. Hard, substantial, tenderness is not obvious, liposarcoma originated from primitive mesenchymal cells, more common in adults, rare in children, mostly in 10 to 15 years old, no difference between men and women, liposarcoma from adipose tissue, so it grows in all parts of the body, Most occur in the retroperitoneum, the other is the thigh and knee, tumor growth is hidden, slow, no obvious boundaries, no special clinical manifestations, treatment: complete surgical resection is the best treatment, radiotherapy is not exact, chemotherapy is only in surgery It is used when it is ineffective or impossible to implement. The commonly used drugs are cyclophosphamide, vincristine, actinomycin D (dactinomycin), but the effect is not clear.

4. Angiosarcoma

The tumor is nodular, painless, purple-red, superficially easy to hemorrhage and ulceration, clinical manifestations can be divided into 3 types: superficial diffusion type (can be multifocal), nodular and ulcer type.

5. leiomyosarcoma

Leiomyosarcoma (liomyosarcoma) can occur in any organ or tissue containing smooth muscle, most commonly found in the gastrointestinal tract, adults, children, but more common in adults, children with leiomyosarcoma, the main site of the gastrointestinal tract including the retroperitoneum, trachea Smooth muscles in the bronchi and lung, general gastrointestinal and subcutaneous tissue tumors have a good prognosis, retroperitoneal, mesenteric prognosis is poor, gastrointestinal leiomyosarcoma can be distributed in various parts from the stomach to the rectum, can be more Tumors can cause intestinal obstruction, intussusception, physical examination can touch the mass, children can have anemia, abdominal pain, gastrointestinal bleeding, etc., gastrointestinal diagnosis can be done X-ray barium meal angiography, typical signs are marginal filling defects There may also be ulceration, fibrous gastroscope, and enteroscopy have diagnostic effects. The treatment is complete resection of the tumor, but the scope is wide. For example, the gastrointestinal tract should be resected at least 5 cm proximal and distal, and the tumor can be metastasized by blood. To the liver, or planted in the greater omentum, peritoneum, lymph node metastasis, radiotherapy and chemotherapy have a certain effect.

6. Infant hemangiopericytoma (infantile hemangiopericytoma)

In 1942, Stont and Murray first described hemangiopericytoma, which is characteristic in children. It is often called "vascular hemangioblastoma". It is more common in infants around 1 year old, only about 50% develop malignant, and tumors grow in subcutaneous tissues or In the striated muscle, it is more common in the lower limbs, especially in the thigh and groin, the head and face, the mediastinum, the retroperitoneum and the pelvis. The tumor is a slow-growing painless mass with abundant blood vessels, so the local temperature may be slightly increased, sometimes Weak pulsation, but the above situation rarely attracts attention, the tumor may be accompanied by hypoglycemia, masculine performance, the cause is unknown.

Infant vascular epithelial cell tumors are mostly benign, local resection can be cured, no radiotherapy, chemotherapy, malignant surgery, chemotherapy, but there is no consensus.

7. Malignant hemangioendothelioma

Malignant angioendothelioma (malignant angioendothelioma), also known as angiosarcoma, is rare in children, mostly in the skin, soft tissue, liver and spleen, tumors have the highest incidence of skin, followed by soft tissue, breast, liver, spleen, heart, etc. Where, skin tumors are more common in the head and neck, followed by upper and lower limbs, trunk, showing nodules higher than the skin, diameter 1 ~ 2cm, the surface is often necrotic ulceration.

Pathological diagnosis of this tumor is easy to be confused with cancer and vascular rich metastatic carcinoma, epithelial cell synovial sarcoma, highly differentiated fibrosarcoma and intravascular papillary endotheliosis, immunohistochemical staining is helpful for differential diagnosis For example, the factor VIII-related antigen is synthesized by endothelial cells and a specific marker for tumors derived therefrom, and the lentil agglutinin can confirm the vascular endothelium properties of the tumor.

The tumor is widely spread or distantly metastasized, and the mortality is high. The tumor is often transferred to the lungs and liver, and to the regional lymph nodes. The sensitivity to radiotherapy is low. Only the early limbs are limited, and the trunk tumor may be radically operated and resected.

8. Lymphatic sarcoma (lymphangiosarcoma)

Lymphatic sarcoma (lymphangiosarcoma) is extremely rare in children, highly malignant, and the tumor presents a tough mass, such as color green ecchymosis, higher than the skin surface, similar skin, subcutaneous tissue, muscle fibrosis, tumors occur in the limbs , to the near distal development, there is no effective treatment for lymphangisarcoma, tumors are not sensitive to radiotherapy, adult cases are mostly amputated, patients with chronic and congenital lymphedema should be regularly checked, if there are tumor symptoms, that is to do Radical surgery.

The exact source of synovial sarcoma is unknown, and may be derived from mesenchymal tissue that differentiates into synovial cells. It is more common in adolescents, and there are reports of infants. More males than females, tumors occur in lower limbs, knees are most common, and others are in turn. , , hip; upper limbs mainly occur in the wrist, shoulder, elbow and hand joints, can also occur in the head and neck, chest and abdomen wall.

Clinically, more than a mass or a tumor area is touched near the joint. The child may have spontaneous pain or tenderness, and there are few serious dysfunctions. The poorly differentiated tumor may have weight loss and exercise obstruction. Head and neck cancer may cause Difficulty swallowing or breathing, the tumor on the X-ray film is a round or oval soft tissue mass, which may have lobulated shape, about 20% of cases have periosteal reaction or bone erosion, calcification in pediatric cases is more common than adults, CT A central necrotic area of the mass was found by scanning.

The treatment is simple tumor resection, no adjuvant treatment, the tumor is easy to relapse or spread, 80% of the tumor has lung metastasis, 20% have regional lymph node metastasis, 23% have bone metastasis, and it is generally recommended to remove the tumor and also remove the muscle group near the tumor. Or amputation, regional lymph node dissection, radiotherapy and chemotherapy are not sure, chemotherapy commonly used vincristine, cyclophosphamide, actinomycin D (dactinomycin), daunorubicin and so on.

9. Malignant mesothelioma

Thoracic lesions often have chest pain and dyspnea, and may have pneumothorax and pleural effusion. If the tumor occurs in the abdominal cavity or the testicular sheath, it may be more likely to have a mass and a tumor. The tumor has a tendency to be partially infiltrated and spread widely along the serosal surface. There is a tendency to metastasize to the lungs, brain and other parts.

10. Malignant schwannomas

30% to 43% of children with malignant schwannomas come from neurofibromatosis, 50% of adults are from the main nerve trunk, this tumor is a gradually increasing body surface mass, with varying degrees of pain, sudden enlargement or pain of the mass Because it may turn into malignant, it is necessary to do biopsy as soon as possible. Malignant schwannomas are a very aggressive tumor. It is easy to relapse after resection. The distant metastasis is mainly to the lungs, followed by the liver and bone. In patients with neurofibromatosis, the degree of malignancy is higher. Because the tumor is related to the main nerve trunk, the tumor is generally completely removed from the tumor, and the amputation is performed. It is not necessary to perform lymph node dissection. The radiotherapy of the tumor is ineffective, and the chemotherapy can be performed according to the rhabdomyosarcoma program. not sure.

11. Malignant stromal tumor

Can occur in any part of the body, the most common in the thigh and retroperitoneum, the signs depend on tumor collapse and invasion of surrounding tissues and organs, femoral tumor can block venous return, lower extremity edema, retroperitoneal tumor often misdiagnosed as abdominal tumor, whole body The situation can be rapidly deteriorated, and the tumor should be extensively removed, but it is generally impossible. Preoperative radiotherapy can make the tumor volume smaller, so that surgical resection can be performed. Postoperative radiotherapy can prolong the recurrence. The chemotherapy regimen is the same as the rhabdomyosarcoma program.

12. Acinar soft tissue sarcoma

Tumors occur in children in the head and neck, especially in the eyelids and tongue. Adults are more common in the lower extremities. Because the blood vessels in the tumor are rich, sometimes they can touch the blood vessels. Therefore, they are generally asymptomatic and easily overlooked. Brain, bone metastasis, lymph node metastasis is rare, treatment with radical surgery to remove primary tumors and metastases, postoperative chemotherapy, radiotherapy combined application.

13. Epithelioid sarcoma

Most tumors occur in the limbs, hands, forearms, and early symptoms are mild. It is a slow-growing, painless hard nodule that may ulcerate. Long-term unhealed, the tumor can slowly spread to the proximal fascia and tendon of the limb. Transfer to lymphoid tissue, heart, lungs, brain, bones and skin.

Examination of soft tissue sarcoma in children

1. Thick needle aspiration biopsy

Select the appropriate timing and location (in the short-term preoperative period, the needle point is designed on the skin to be removed). Under local anesthesia, use a trocar with a suitable caliber to penetrate the body. After entering the pseudo-envelope, pull out the needle core. Repeat the puncture and suction under negative pressure until the satisfactory tumor tissue is obtained. During the puncture, care should be taken not to break through the pseudo-envelope in other directions of the tumor, to avoid damage to the adjacent important structure and to avoid tumor spread. The tumor is too small and too deep. It is not suitable for use.

2. Fine needle aspiration biopsy

With the continuous improvement of the diagnostic level of soft tissue tumors by cytological examination methods, the method of fine needle aspiration cytology has gradually increased in the clinical application of sarcoma. Some authors report that it can be classified according to this, because tissue damage is small due to fine needle aspiration. Relatively safe, it can be carried out under the supervision of B-ultrasound or CT. Its indications are obviously widened by thick needle aspiration. The operation method is similar to that of thick needle aspiration. When B-ultrasound or CT is performed, it needs to be implemented by relevant professionals. The surgeon cooperates.

3. Cut biopsy

Select the appropriate site, make a small incision on the skin to be excised, cut the tumor tissue layer about 1cm × 1cm × 1cm, and send it to the pathology examination. The incision is tightly sutured. If it is frozen, the dressing can be changed. The surgical instruments are restarted according to the non-contaminating operation, and the skin of the biopsy area is removed by fusiforms, such as paraffin section examination, and the operation is selected after the diagnosis is confirmed.

4. Excision biopsy

For small, benign, potentially large superficial masses, a biopsy may be performed and re-extracted if necessary.

The failure rate of biopsy was 8.2% to 10%, and the complication was 18.2%.

5. Imaging examination (X-ray film, CT, MRI, etc.)

The imaging of most soft tissue tumors can provide the depth, boundary, relationship with surrounding tissues, the density of the tumor itself, cystic or substantial and blood supply. According to the above information, the nature of the tumor can be roughly estimated. The design and release of the surgical plan and the evaluation of the effect of chemotherapy provide a basis.

6. Ultrasound examination

Ultrasound can determine the presence of the tumor and the relationship between the tumor itself and the surroundings.

Diagnosis and diagnosis of soft tissue sarcoma in children

Pediatric soft tissue sarcoma is a malignant tumor that occurs in connective tissue, including subcutaneous tissue, muscle, tendon, blood vessels, connective tissue space, and cavity organ matrix. However, it occurs in areas such as bones, reticuloendothelial system, and glial. The cells of soft tissue sarcoma originate from primitive mesenchymal stem cells, which are located in non-segmental mesoderm and can grow in different parts of the body. Histopathological diagnosis is difficult, and the lack of cytokeratin and fine-stranded protein intermediates in immunohistochemical diagnosis has diagnostic value.

According to the clinical manifestations and biopsy tissue examination, the auxiliary examination was confirmed. Identification with other different tumors, mainly by biopsy.

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