Find out what symptoms accompany osteosarcoma

General information

Primary tumors of the skeletal system are rare. They are observed between the ages of 15 and 40 years, that is, children, adolescents and young people are more often affected. In 10% of cases, the disease is detected in people over 60 years of age - in them the tumor may be associated with radiation or develop against the background of benign diseases of the skeletal system. The term “bone cancer” is applied to various types of oncopathology of the skeletal system. Among malignant tumors of this system, the most common are osteogenic sarcoma (synonymous with osteosarcoma in 50-60% of cases), Ewing's sarcoma (the second most common type of tumor), chondrosarcoma , giant cell tumor , fibrosarcoma .
Secondary bone tumors (bone metastases) are much more common. The source of metastatic tumors are primary tumors of the lung, prostate, breast and thyroid gland. Bone sarcomas are a group of tumors of nonepithelial origin. In 80-90% of sarcomas, they are highly malignant tumors that are localized in different bones of the skeleton. However, the tubular bones most often affected are the femur and tibia. The tumor develops in the metaphyses (growth part) of long bones, especially in the area of ​​the knee joint. Men get sick more often than women. An increased risk of the disease is observed with genetic abnormalities: hereditary retinoblastoma and Li–Fraumeni syndrome .

Clinical manifestations of osteosarcoma of the jaw

An early sign of the development of osteogenic sarcoma of the jaw:

• 
  pain in a healthy tooth;

• itching in the gum area;
• loosening of some teeth.

Jaw pain occurs in the later stages. Sometimes the onset of pain is preceded by a sensory disturbance in the area where the infraorbital and mental nerves diverge. In the place where osteosarcoma develops, a swelling is formed, the density and size of which may vary. Palpation of the tumor is accompanied by slight pain.

If a large tumor forms, the soft tissues swell, the surface above the tumor becomes thinner, and dilated veins become visible under the skin. If a tumor develops in the area of ​​the masticatory muscles of the temporomandibular joint, then at an early stage a contraction of the jaws can be observed. Sometimes osteosarcoma of the jaw in the later stages is accompanied by a pathological fracture.

The onset of the disease is asymptomatic. When the tumor grows to a large size and the process of decay begins, the patient’s body temperature rises to 40˚C. Osteogenic sarcoma of the jaw first penetrates the lungs, then the skeleton is affected.

Pathogenesis

Carcinogenesis is triggered under the influence of many factors, which include:

• radiation treatment for other cancers;
• suppression of tumor suppressor genes;
• prosthetics performed;
• hereditary diseases;
• complex chromosomal aberrations.

Frequent deletion, as well as loss of heterozygosity of 3q13, are specific aberrations for osteosarcomas. One of the tumor suppressor genes is localized in this region, which regulates cell proliferation and plays a major role in tumor pathogenesis. 3q12 deletion is associated with progression and poor prognosis.

An important morphological feature of bone tumors is the infiltrative nature of growth, or circular (encompasses the entire area of ​​the bone around the circumference). The tumor spreads through the bone marrow spaces and replaces them, destroys and surrounds the bone beams. Neoplastic cells are characterized by severe cellular atypia. The architecture of the newly formed bone determines the tumor potential.

Classification

The most common malignant bone tumors are:

• osteogenic sarcoma;
• Ewing's sarcoma;
• chondrosarcoma;
• fibrosarcoma;
• parosteal sarcoma;
• fibrous histiocytoma;
• lymphosarcoma.

Bone sarcoma

The most common tumor develops from osteoblasts and in the process of neoplasm develops altered, atypical bone. This tumor is called differently - synonyms are osteogenic sarcoma, osteosarcoma, osteoidsarcoma, osteoblastosarcoma. Chondrosarcoma and fibrosarcoma are distinguished as independent forms.

Classic osteosarcoma is a highly malignant intraosseous tumor. If a tumor appears in an unchanged bone, it is a primary sarcoma; if it develops after irradiation or against the background of Paget’s disease , it is a secondary sarcoma. The tumor affects the metaphysis (growth zone) of the tubular bone of the arm or leg. Active growth processes occur in the metaphyses, so they are associated with a high risk of tumor transformation. The appearance of a tumor is associated with the rapid growth of bone tissue.

The peak incidence is observed in children aged 10-14 years. They are more likely to affect the tubular bones of the leg - osteosarcoma of the femur in the hip joint, as well as the bones of the knee joint (femur and tibia). When the femur is affected, a large lesion zone of 5-10 cm is revealed. Hemorrhages , necrosis and cystic cavities are found in the newly formed bone. The tumor gradually destroys the periosteum. Sarcoma of the hip occurs at any age, but is more common in young men.

It is characterized by aggressive growth and metastasizes early. Malignant formation of the knee joint in most cases is a secondary metastatic process and less often - as a primary process. In adults, osteosarcoma affects the spine and maxillofacial bones. This type of tumor rarely develops in the area of ​​the coccyx, shoulder girdle, ulna and humerus. Primary osteosarcoma of the ribs is less common, but secondary (metastatic) tumors predominantly affect the ribs, vertebrae and pelvic bones. Malignant transformation of Paget's disease to sarcoma occurs in 1% of cases and the peak incidence occurs at 60 years of age and older. Of all the bones, the jaw is the most commonly affected. Jaw cancer is a tumor with rapid aggressive growth and early metastases to the lungs (most often). There are isolated cases of metastases to the stomach and soft tissues. Damage to the lower jaw is somewhat more common than to the upper jaw (corresponding to 58% and 42%).

Hand bone cancer in most cases is metastatic. In the bones of the skull there are various types of primary sarcomas, as well as secondary metastatic tumors from the primary focus (uterus and prostate). Skull bone tumors cause headaches due to compression of the brain. Spinal cancer is characterized by active growth and rapid progression. Patients develop movement disorders: when the lumbar region is affected, the lower limbs and the functions of the pelvic and genital organs are affected. Cervical spine cancer is very dangerous and requires immediate treatment. When the cervical vertebrae are damaged, the functions of the arms and legs are affected.

Ewing's sarcoma

This type of tumor occurs in children and adolescents. Develops in bone tissue and soft tissues. It is aggressive and metastasizes quickly. There is a high mortality rate in the first year of diagnosis. If we consider damage to the inert system, then in 70% this type of tumor occurs in tubular bones, flat bones (scapula, ribs) and pelvic bones. Compared to osteosarcoma, the diaphyses of bones are more often affected. Many patients have genetic mutations that cause disruption of cell differentiation processes.

Chondrosarcoma

The neoplasm develops from cartilage tissue. Occurs in people over 40 years of age. The course of this type of cancer is difficult to predict. In some, chondrosarcoma develops slowly, while in others it develops very quickly and metastasizes . This type of tumor affects any bone, but the femur and pelvic bones are predominant. The tumor can also develop in flat bones (scapula, skull, ribs). Most often, the course of chondrosarcomas is slow and metastases appear late. The appearance of pain is characteristic, and when the pelvis is damaged, the nerve roots are involved, therefore, in addition to pain, the function of the pelvic organs will be impaired.

Fibrosarcoma

A rare tumor that develops from connective tissue. Tumor cells are located between the collagen of the connective tissue and look like a spindle. In most cases, fibrosarcoma appears after radiation treatment.

Classification of osteosarcomas according to the TNM system

• T0 - no data on the tumor process.
• T1 - formation no more than 8 cm.
• T2 - neoplasm larger than 8 cm.
• T3—tumor continuity is disrupted.

Involvement of lymph nodes (“N”) is rare, since bones do not have a lymphatic system. But if there are metastases to the nodes, this is a poor prognostic sign. Regional lymph nodes are nodes that correspond to the location of the primary tumor.

• N0 - no metastases in the lymph nodes.
• N1 - metastases are present.

Osteosarcoma is characterized by the development of hematogenous metastases, which in the diagnosis of o. By the time of diagnosis, 20% of patients already have small metastases in the lungs. Metastases can also be in the pleura, pericardium, bones, kidneys, and central nervous system.

• M0 - no distant metastases.
• M1 - distant metastases are present (“a” - to the lungs, “b” - to organs and tissues).

An important point is histological examination, which confirms the diagnosis, determines the type of tumor and its malignancy. There are five forms of osteosarcomas, with different degrees of differentiation.

Metastatic bone lesions

The course of metastatic bone lesions is not asymptomatic - pain is the first manifestation of bone metastases. Most patients describe it as unbearable. Patients with metastases also experience:

• pathological fractures of tubular bones;
• vertebral compression fractures;
• spinal cord compression;
• hypercalcemia.

A quarter of breast cancer have fractures of long bones associated with destruction of the cortical layer. radiation therapy or surgery is required .

Benign bone formations

Bones are affected by benign tumors ( chondroblastoma , fibrous dysplasia , osteoclastoma , aneurysmal cyst and others), with which a malignant formation must be differentiated.

Aneurysmal bone cyst

A benign tumor-like bone lesion, which is characterized by the proliferation of connective tissue. It contains cavities filled with blood. A distinctive feature of this cyst is its spread through the epiphyseal plate, as well as to adjacent bones. The cyst appears at any age, but more often in adolescents and children. Its most common location is the bones of the spine, the distal part of the femur, the proximal part of the humerus and tibia, and the pelvic bones. A secondary cyst is formed after damage to benign chondroblastoma , fibrous dysplasia , giant cell tumor and malignant neoplasms - osteogenic sarcoma .

Chondroblastoma

A benign tumor that forms at the articular ends of a bone. It develops from chondroblasts and resembles mature cartilage tissue. It is characterized by slow growth and a favorable course, but there is information about cases of a malignant course or malignancy by chondroblastoma. Like all bone tumors, it affects children and adolescents, and in rare cases, older people. Clinical manifestations are pain and swelling of the joint. Treatment is surgical. The prognosis is favorable.

Diagnosis of the disease

Dental instructions for oncological diagnostics require the following activities:

• collecting a medical history and identifying the patient’s main complaints;
• visual and instrumental examination of the maxillofacial area;
• radiography of the bone tissue of the jaws;
• palpation of regional lymph nodes;
• general and detailed blood test;
• biopsy or histological analysis.

The key way to evaluate patients with osteosarcoma is radiography. On X-ray photographs, the doctor determines individual areas of bone dissolution or replacement with connective tissue.

Recently, dental oncologists have begun to actively use computed tomography, which makes it possible to detect areas of atypical ossification outside the jaw. Using this technique, the specialist also assesses the size, shape and exact location of the malignant neoplasm.

X-ray of osteosarcoma of the mandible

The final diagnosis is established only by the results of histological and cytological analysis. To do this, the surgeon removes a small area of ​​abnormal tissue and sends it to the laboratory for microscopic analysis. Based on the results of the biopsy, a treatment and rehabilitation plan for the cancer patient is formed.

Causes

The causes of bone tumors are not well understood. To a certain extent, bone cancer is caused by:

• Chronic diseases of the skeletal system ( osteomyelitis , Paget's disease , benign tumors ).
• Smoking.
• Injuries.
• Foreign body in the bone (orthopedic implants). Tumors of the skeletal system are more often detected in patients who have had implants used for osteosynthesis or have undergone joint replacement. Dentures use hexavalent chromium, which is a carcinogen.
• Previous radiation therapy , which induces the development of osteosarcoma. Radiation-induced sarcomas develop 3 years after irradiation.
• State of immunodeficiency .
• Skeletal abnormalities ( Olier disease ).
• Mutations of the p53 gene and the retinoblastoma Rb1 gene.
• Hereditary diseases ( retinoblastoma , Li-Fraumeni syndrome , Rothmund-Thompson syndrome , Werner syndrome , absent thumb).
• Benign bone tumors that can become malignant.
• Taking the drug Natpara (a drug for the treatment of hypoparathyroidism ). This drug increases the risk of osteosarcoma.

There is evidence of an increased risk of osteosarcoma in children born weighing more than 4500 g and above average height. However, in most patients no specific factors are identified.

Symptoms and manifestations of bone cancer

A characteristic manifestation of malignant bone formation of any location is constant, aching pain, disturbing for several weeks or months, not eliminated by analgesics and increasing over time.

For a long time, patients associate the appearance of pain with a former injury, so they do not consult a doctor and this becomes the reason for a late diagnosis. Pain from bone tumors does not depend on physical activity and bothers patients even at rest. Sarcoma is characterized by pain that worsens at night and relief does not occur in any position of the limb. Symptoms of osteosarcoma also include swelling of the tissue in the area of ​​the tumor. But local swelling appears later - at first it is a deeply located tumor in the soft tissues, and then the swelling area increases, the affected area swells, and a venous network appears on the skin, sometimes the skin turns red.

When joints are affected, swelling appears in their area. Cancer of the knee joint or any other joint disrupts the function of the joints - they bend poorly and movement is limited. Movements are impaired not only when there is a tumor in the joint, but also in the nearest joint when the bone is damaged. A tumor of the joints changes their shape. In some cases, effusion appears in the joint cavity.

Photo of osteosarcoma of the knee joint

Bone cancer is accompanied by fragility, so patients often experience spontaneous fractures due to minor trauma. When the tumor process affects the spine, difficulties appear when walking, weakness in the limbs, and paretic phenomena. A tumor of the leg bones is accompanied by numbness of the limb and lameness. Symptoms of advanced bone sarcoma include abdominal pain, nausea and vomiting, which is associated with increased levels of calcium in the blood ( hypercalcemia ) due to bone breakdown. In the advanced stage, general symptoms are also noted - fatigue and weight loss. The appearance of general symptoms indicates a process with metastasis.

Symptoms of jaw cancer

Osteosarcoma that occurs in the thickness of the jaw is called central, and that developing in the peripheral parts is called peripheral. For a long time, signs of bone cancer do not appear. Then pain appears that is not associated with tooth pathology. Long-lasting pain should alert the patient. The tumor process can be recognized using radiography and MRI. With a tumor formation in the lower or upper jaw, pain in the teeth occurs for no reason and radiates to the ear and eye. It gets worse at night and becomes unbearable, forcing patients to wake up and take painkillers.

Palpation of a tumor in any part of the jaw is painful. In addition, osteogenic sarcoma of the jaw changes the sensitivity of the trigeminal nerve and causes changes in tooth position. The teeth within the tumor become mobile. paresthesia and anesthesia occurs (Vincent's symptom).

Photo of sarcoma of the lower jaw

As the jaw grows, it becomes deformed. The tumor quickly grows, spreads to the surrounding tissues and compresses them, causing exophthalmos and congestion in the soft tissues. Lymph nodes are often not enlarged. The mucous membrane of the alveolar process at the site of the tumor is red and swollen, and when the mucous membrane is injured, ulcers appear on the gums. In some cases, osteosarcoma festeres (the temperature rises, weakness and malaise appear).

Symptoms and course

Patients with malignant tumors of the jaws, of course, belong to the contingent of the most severe patients due to the difficulty and danger of surgical intervention, often great difficulties in choosing a prosthetic method, and finally, due to little hope for a favorable outcome, not only long-term, but also immediate.

The presence of a malignant neoplasm in the oral cavity often leads to disruption of eating and speech functions quite early on. A number of special conditions under which tumors of the oral cavity and jaws develop undoubtedly influence the early formation of ulcers and tumor disintegration. The temperature of the oral cavity, a humid environment, constant exposure to mechanical insults (eating) and the inability to thoroughly cleanse are the factors that make tumors prone to early ulceration if the oral mucosa is also affected by the tumor. The emerging ulcer quickly increases both in surface and in depth, due to necrosis of the bottom and walls. From this moment on, the clinical picture changes sharply for the worse, inflammatory phenomena appear to varying degrees, intoxication intensifies, and the body's resistance sharply decreases.

Intense bad breath, difficulty eating, and difficulty swallowing create especially difficult living conditions for the patient. Difficulties in eating with sarcoma of the jaw seem to play the greatest role, reducing the body's resistance. The most common cause of death in these patients is aspiration pneumonia.

The rate of tumor growth and the progression of general exhaustion of the body depends on the type of tumor and its location. Intraosseous giant cell tumors develop slowly over years. Often, squamous epithelial cancer has a long, slow course. The most intense malignant growth appears to be found in pigmented tumors, which generalize very far in a short period of time, and some sarcomas. A sharp acceleration of growth and germination into surrounding tissues can occur after mechanical trauma to the tumor. If until this moment the tumor did not give any signs, then it is sometimes extremely difficult to recognize the presence of blastomatous growth against the background of clinical phenomena after injury.

Pathological fractures of the lower jaw, due to infiltration of the entire thickness of the bone by tumor elements, are observed much more often with cancer than with sarcomas.

Enlargement of regional lymph nodes in malignant tumors of the jaws for the most part has a dual origin. On the one hand, according to anatomical conditions, there is the possibility of early and extensive damage to nodes through metastatic transfer of tumor elements. Their damage is especially pronounced in cancer of the lower jaw. On the other hand, the tendency of jaw tumors to early decay with severe inflammatory phenomena entails a reactive enlargement of the lymph nodes (of an inflammatory nature).

Lymph nodes affected by a cancerous tumor, in advanced cases, are soldered together into huge conglomerates and grow together with the surrounding tissues. When metastases break through the skin, deep, crater-shaped ulcers with purulent-ichorous discharge are formed. In slow-growing forms of sarcomas (for example, intraosseous giant cell tumor), inflammatory processes prevail in the lymph nodes.

Tests and diagnostics

• X-ray of the affected area in two projections. X-ray examination covers the entire affected bone and joints nearby. This is necessary in order not to lose sight of the “step-by-step” metastases. With osteosarcoma, the x-ray picture may be different. A large tumor node with unclear outlines. The cortex and medulla of the bone are affected. The tumor destroys the periosteum, often revealing a “cloud-shaped” component, which is associated with uneven ossification of the tumor. In the newly formed bone, osteosclerotic and osteolytic (sparse) areas alternate. However, areas of bone change become visible on x-ray when the tumor grows to a certain size. Therefore, to avoid diagnostic errors, you need to use MRI and CT.
• MRI and CT provide layer-by-layer visualization of bone, bone marrow, soft tissue and connective tissue. They are carried out with contrast. These methods clarify the size of the lesion, the exact location and spread of the tumor beyond the bone.
• To detect distant metastases, a CT scan of the chest and abdominal pelvis is performed. Chest X-ray also reveals metastases in the lungs.
• To identify lesions in other bones, skeletal scintigraphy (radionuclide scanning) is prescribed.
• The main method to confirm the disease is a biopsy and histological examination of the obtained material. The patient's bone tissue is collected and the type of tumor is determined. Tissue collection is carried out by excision of the bone, which makes it possible to obtain sufficient material. If the lymph nodes are enlarged, a biopsy (puncture) is also performed.
• Histological examination. To make a diagnosis, detection of neoplastic tissue (osteoid) is mandatory. The amount of osteoid is not important because it is different in different fields of view. Osteoid is produced by tumor cells and may be found adjacent to or surrounding the cells.
• Angiography. Detects vascular damage.
• Molecular genetic diagnostics. It determines the presence of mutations in the genome. This is necessary to determine the need for targeted therapy (biological).

Specific markers for osteosarcoma are currently unknown. Sometimes patients are prescribed a CEA test. Let's find out how necessary this is. Carcinoembryonic antigen , what is it? This is a tissue marker of cancer. What does this marker show? This type of protein is detected in minute quantities in a healthy person, but during the oncological process its level increases.

Determination of this antigen is used to diagnose the following tumors:

• colon and rectum;
• stomach;
• mammary gland;
• pancreas;
• lungs;
• metastases to the liver and bones (the sensitivity of the method in the metastatic process is much lower).

This test is not specific for malignant tumors, since an increase in antigen is observed in inflammatory and autoimmune diseases, benign tumors (intestines, liver, pancreas), liver cirrhosis , hepatitis , Crohn's disease , tuberculosis , pneumonia , cystic fibrosis . During a malignant process, the level increases throughout the entire period of the disease. CEA analysis is used to monitor the effectiveness of treatment of malignant neoplasms. A decrease in its level during the course of treatment indicates a positive result. The norm of carcinoembryonic antigen for non-smokers: 0-3.8 ng/ml, the norm for women who smoke is slightly higher than 0-5.5. A negative result does not exclude cancer.

Diagnostics

Pathological examination of a piece of tumor is very widely used in clinical practice. The general principles of the technique for taking a piece of a tumor are entirely applicable when examining tumors of the jaws. If possible, without anesthesia, a piece of tissue is cut out at the border of the healthy and tumor-invaded area. The material is processed according to certain rules of histological technique.

Puncture of the tumor with a syringe with a wide needle often (for small tumors) makes it possible to obtain a cylindrical piece of tissue suitable for making sections.

The presence of tumor infiltration, the nature of the inflammatory infiltration, the degree of differentiation of the formed elements, the number of dividing cells, the presence of special formations (pearls, giant cells, foci of necrosis, hemorrhages) are the main signs that serve as criteria for making a pathological-histological diagnosis for suspected sarcoma of the jaw .

Blood and urine testing should be performed in all cases; these studies are auxiliary diagnostic techniques that make it possible to find out, for example, the degree of anemia, the intensity of inflammatory processes, and side diseases.

X-ray examination provides a very important service in diagnosing sarcoma of the jaw. The main importance of radiographs is in determining the boundaries of the tumor. When reading a radiograph, it is necessary to find out, if possible, the area of ​​primary localization of the tumor, its shape, relationship to neighboring organs and the degree of bone damage. Malignant tumors are characterized by vagueness (“blurring”) of the tumor boundaries. It is necessary to pay attention to the loss of bone structure, the condition of the cortical layer, and whether there is a pathological fracture. It is imperative to find out the relationship of the root tips to the tumor, which is important in differential diagnostic terms.

Diagnosis of sarcoma of the jaw is relatively simple when it is localized in accessible parts of the mucosa. Considerations regarding the age of the patient and the detection of factors causing irritation of certain parts of the oral cavity and jaws should also be taken into account in the clinical recognition of tumors.

It is much more difficult to recognize deeper tumors. In terms of symptomatology, there is a fundamental difference in the localization of sarcoma on the lower or upper jaw. In the lower jaw, the presence of a tumor can be established quite early, deformation of the dental arch, displacement of organs and formations of the floor of the oral cavity can be detected, especially with periosteal sarcomas.

If periosteal sarcomas are recognized early due to the certain availability of external examination, then central tumors developing in the thickness of the lower jaw may not show any external signs for a long time. Quite strong pain, but uncertain in localization, causeless local loosening of teeth, and often spontaneous loss of teeth - these are the most characteristic signs of a tumor developing in the central parts of the lower jaw.

With sarcomas of the upper jaw emanating from the wall of the maxillary cavity, sometimes only indirect signs can sometimes be stated, for example, neuralgic pain, darkening of the maxillary cavity, serous-purulent exudation from the nose, repeated bleeding, etc. However, these symptoms, without directly indicating the development of a tumor can give rise to diagnostic errors over a long period of time, and the true nature of the disease becomes clear only after the tumor, having occupied the entire maxillary cavity, begins to either infiltrate the nasal wall, or spread towards the alveolar process, causing loosening of the teeth, or grows outer wall of the jaw; in this case, a noticeable deformation of the face is formed with successive ulcerations through the skin.

Removal of loose teeth soon entails infiltration and germination of the walls of the free socket with tumor elements.

With cysts, the mucous membrane covering the protrusion has a normal appearance and is not inflamed; with sarcoma, the mucous membrane is often changed - swollen and hyperemic. With sarcoma, the bone wall is softened and gives in when pressed, even with a large thickness of the bone; with a cyst, the bone springs due to its thinning (softening of the cyst).

Tendency to bleeding, causeless loosening of teeth and symptoms of bone rarification are the main clinical signs that must be used to guide the differential diagnosis. Data from X-ray diagnostics and histological examination allow us to finally understand the essence and distribution of the process.

Difficult in diagnostic terms should be considered cases of development of sarcoma of the jaw from the wall of a periodontal cyst: but even here there are signs that suggest a malignant tumor: the absence of clear boundaries of the cyst, the softened spongy part of the bone and a strong tendency to bleeding during surgery and during dressings.

In conclusion, it is necessary to touch upon the differential diagnosis between a periodontal cyst and a central sarcoma of the mandible. Radiographs, physical examination and mechanical examination findings may sometimes be very similar. Anamnesis and objective data regarding the connection with the dental system do not always clarify the matter. In any case, a mixture of these completely different forms of diseases is observed.

Recognition in doubtful cases is most accurately determined by a test puncture and suction of the contents: with a cyst, a light yellow transparent liquid with cholesterol crystals (or pus) is obtained, with a tumor - blood. In cases of cysts and sarcomas, puncture sometimes fails due to the strength and thickness of the outer bone wall. It must be borne in mind that the lingual wall may also be thinned, making it difficult to touch and puncture. If the suspicion of a cyst cannot be completely eliminated, then an operative test trepanation of the bone wall is necessary.

In children

Among all tumors in children, 5% are bone sarcomas : 3% osteosarcoma and 2% Ewing sarcoma . The lack of alertness among doctors and the atypical nature of the course greatly complicate early diagnosis. Osteosarcoma in children in half of the cases is localized in the knee joint (the femur and tibia are affected). In second place is damage to the metadiaphysis of the humerus. The most common symptom of osteosarcoma is constant, dull pain with a gradual increase. Most children have a soft tissue component - swelling and an increase in the volume of the limb. Weight loss and fever at the onset of the disease are rare.

Ewing's sarcoma occurs before the age of 17 years, but the peak incidence occurs between 12 and 15 years of age. Boys are most often affected. Ewing's sarcoma, unlike osteosarcoma, does not synthesize osteoid . Often this tumor is located in the scapula, ribs, vertebrae, pelvic bones, as well as the femur, fibula, and humerus. Unlike osteosarcoma, flat bones are affected. Signs of Ewing's sarcoma are increasing pain, tissue swelling, dysfunction, and fever. The soft tissue component is more pronounced than bone destruction. An increase in local temperature due to the appearance of hemorrhages and necrosis in the tumor complicates diagnosis, since osteomyelitis is suspected. Neurological symptoms are associated with nerve damage.

Combined treatment for Ewing's sarcoma:

• Chemotherapy courses (standard drugs for this pathology).
• Surgery (similar to osteosarcoma).
• Radiation therapy. It is carried out on the area of ​​the primary tumor and on distant metastases.

Solitary form of peripheral osteoma on the lower jaw

Osteoma is a rare, slow-growing, benign osteogenic tumor that most often occurs in the maxillofacial region. These types of neoplasms can be found both on the surface of the bone (formed from the periosteum - peripheral, periosteal or exophytic form), and in the structure of the bone marrow space (formed from the endosteum - endosteal or central form). The case of verification of osteoma in the structure of muscle tissue is rare.

The pathogenesis of peripheral osteoma remains a completely unresolved clinical and scientific issue: there are opinions that the neoplasm can be attributed to true tumors or to reactive lesions provoked by trauma, constant traction of the muscles attached to the periosteum, as well as the action of infectious factors. Endocrine changes, in turn, can be a direct etiological factor initiating the occurrence of a neoplasm. Depending on the action of endocrine factors on the tumor, they can be classified as central and peripheral, or extraosseous.

Osteoma is the most common benign tumor of the nose and paranasal sinuses and the most common neoplasm in the frontal sinus. Single peripheral osteomas that occur in the lower jaw are quite rare lesions and are diagnosed in people of different age categories (from 4.8 months to 50 years), equally often in both men and women. Typically, clinically the tumor grows asymptomatically, and radiographically it manifests itself as a distinct contrasting area of ​​​​a round or ovoid lesion. Depending on the histological picture, compact, spongy and mixed forms of osteoma are distinguished. The presence of many small osteomas in the structure of the lower jaw is diagnosed as Gardner's syndrome, which is also characterized by the presence of perirectal polyposis and associated skeletal abnormalities. These symptoms can be used to differentiate between Gardner's syndrome and multiple dental impaction.

Treatment of osteomas is carried out through surgical excision of the tumor, but only in those patients who complain of accompanying uncomfortable symptoms of the tumor. Until now, only 26 clinical cases of verification of single peripheral osteomas of the mandible not associated with Gardner's syndrome have been reported in the literature.

Based on the extreme rarity of the disease, the purpose of this article is to illustrate an unusual clinical case of a single peripheral osteoma found in the structure of the mandible, as well as to conduct a retrospective analysis of the literature data regarding the pathogenesis, differential diagnosis and choice of treatment for this type of benign tumor.

Clinical case

A 30-year-old patient applied to the Department of Dentistry with the main complaint of swelling on the inside of the posterior part of the lower jaw, which she had noticed since the age of 8. 8 years ago, the formation that had been present since childhood increased to 5 mm in diameter, after which it began to grow progressively. Although the swelling was asymptomatic, due to its large size it provoked a feeling of discomfort when eating. The latter motivated the patient to seek dental care. The patient did not have any hereditary diseases in her own or family history, as well as a recent injury to the problem area of ​​the jaw. Based on the fact that no other symptoms other than a tumor in the mouth were found, Gardner's syndrome was excluded from the list of possible diagnoses. Clinically, a single pedunculated oval node was detected on the lingual side of the jaw in the area of ​​the 36th and 37th teeth. The tumor was more than 1 cm in diameter (Figure 1), and the overlying mucosa showed no signs of change or inflammation. Upon palpation, the tumor was painless, and it was also possible to manually establish its lobular shape and hard consistency.

Photo 1: View of a tumor in the oral cavity.

On a cross-sectional radiograph of the lower jaw, a clear radiopaque area of ​​irregular shape is visualized, connected to the cortical plate of the jaw in the area of ​​the 37th tooth through a pedicle (photo 2). The radiographic density of the formation is similar to the density of the body of the lower jaw. In the process of differential diagnosis, possible variants of bone exostosis, peripheral osteogenic fibroma, osteoid osteoma, osteoblastoma and osteosarcoma were analyzed. After carefully explaining to the patient the essence of the future surgical procedure and the possible risks of manipulation, complete excision of the tumor was performed using rotary instruments and a bone chisel. During surgery, an excisional biopsy of the tumor was also performed under local anesthesia.

Figure 2: Cross-sectional radiograph of the mandible.

Histopathological analysis of the lesion helped to establish that the tumor consists of dense bone tissue, in which concentric layers of bone plates are observed, as well as osteocyte cells in the lacunae (photo 3). The results of histopathological examination along with clinical and radiological findings helped confirm the diagnosis of compact osteoma.

Photo 3: Hematoxylin-eosin staining (10x magnification).

Discussion

According to the World Health Organization classification, osteoma belongs to benign neoplasms, consisting of differentiated mature bone tissue cells with a predominance of laminar structure, and with a very slow growth pattern.

It is not yet clear whether osteomas are true benign neoplasms or hamartomas. Although, on the other hand, the latest WHO classification of diseases defines osteoma as a truly benign tumor of bone tissue, clearly differentiating it from exostoses and other types of hamartomas.

The true etiology of the lesion has not been thoroughly elucidated, although Fetissof and Varboncoeur argue that the tumor develops either from embryonic cartilaginous remnants or from persistent embryonic cells contained in the periosteal structure. According to the embryological theory, osteomas originate from the area of ​​sutures of bone structures filled with tissues of different embryonic origin (membranous or enchondral). Other possible etiological factors are inflammation, trauma or endocrine pathologies. Kaplan et al suggested that the combination of trauma and persistent muscle traction in the periosteal region plays a key role in the development of the peripheral type of neoplasm. But the above-mentioned theories of osteoma formation do not explain the mechanism of its development in the described clinical case. Regezi and Sciubba, as a result of a comprehensive analysis, came to the conclusion that none of the proposed etiological theories fully explains the cause and mechanism of development of the neoplasm.

Histologically, three types of osteomas can be distinguished: hard or compact (consisting of a dense, ivory-like compact substance with a small amount of spongy bone, this type of pathology is quite difficult to resect), cancellous (trabecular), or mature (consisting of soft spongy bone and bone marrow) and mixed. Single peripheral osteomas (PO) of the jaws are quite rare lesions and are most often diagnosed in young people with the same frequency in both men and women. The patient described in the clinical case mentioned above was 30 years old. Typically, osteomas are asymptomatic tumors and can often go undetected until they cause visible facial asymmetry or functional malocclusion. In this case, the pathology must be confirmed by x-ray examination. The most common topography of osteomas is the area of ​​the body of the mandible behind the premolar on the lingual surface and the area of ​​the condylar process. Less often, osteomas occur in the area of ​​the angle, coronoid process and ramus of the mandible, or in the area of ​​the external auditory canal, orbit, temporal bone, maxilla, zygomatic arch and pterygoid processes. Often osteomas can be verified as part of Gardner's syndrome, characterized by the presence of several tumors in the jaw structure, pararectal polyps with a high degree of malignancy, cutaneous fibromas, congenital retinal hyperpigmentation, tooth retention, enostosis, epidermal cysts and supernumerary teeth. Gardner syndrome is an autosomal dominant disease caused by a mutation in the structure of the APC suppressor gene, which is responsible for the occurrence of adenomatous polyposis of the colon. It is extremely rare, but detection of osteomas is also possible in Haberland syndrome (encephalocraniocutaneous lipomatosis).

Radiologically, a peripheral osteoma may have a mushroom-shaped or oval-shaped lesion that is in close contact with the adjacent cortical plate of the mandible. Moreover, the bone density in the jaw area and in the area of ​​the neoplasm is almost identical. Traditional radiography methods, such as occlusal radiographs, are usually sufficient to diagnose osteoma, as in our case. When the tumor is located deeper in the tissues of the maxillofacial region, other highly effective imaging methods can be used to identify it: panoramic radiography, occipitomental projection radiography of the sinuses, computed tomography (CT), magnetic resonance imaging (MRI) or cone -radiation computed tomography (CBCT). Spiral computed tomography provides opportunities for three-dimensional reconstruction of osteoma: using radionuclide imaging, it is possible to distinguish between actively growing neoplasms (“hot”) and stable lesions (“cold”). In the structure of spongy osteoma, the vascular component predominates, while in the compact type of tumor there are almost no vascular elements observed. Differential diagnosis of different types of formation can be carried out using intravenous angiography.

Differential diagnosis of osteoma is carried out with bone exostoses, osteochondroma, osteoid osteoma, periosteal osteoblastoma, peripheral osteosarcoma, peripheral osteogenic fibroma, Paget's disease, fibrous dysplasia and odontoma. Exostoses are hamartomas that are usually located on the lower jaw or in the palate. Typically, exostoses stop growing after puberty, while osteomas continue to slowly increase in size, occupying a limited volume and forming a lobulated radiopaque structure. Osteochondromas consist of areas of endochondral ossification, calcified cartilage, and adipose or bone marrow tissue within the lumen of the trabecular space. Osteoid osteomas, on the contrary, are characterized by rapid growth, are often painful and contain a large number of vessels and an osteoid component. Periosteal osteoblastoma is also a rapidly growing, painful, round or oval tumor attached to a specific area of ​​the cortex. Parosteal osteosarcoma is often diagnosed in the posterior region of the mandible, representing a homogeneous or heterogeneous formation with a poorly verified lobular sclerotic structure. Telangiectasias are often observed at the periphery of the tumor, and Codman's triangle can be seen on the radiograph. Peripheral osteogenic fibroma can be found only in the gum area; it is a reactive lesion of dense consistency, often found in the anterior part of the upper jaw. Symptoms of Paget's disease are more typical for the anatomical areas of the femur, skull and vertebrae, while in the jaw area, bone damage is observed much less frequently and more often in the older age group. In this case, bone damage, as a rule, is always bilateral, and more often affects the upper than the lower jaw. Bone tissue in Paget's disease becomes deformed and increases in size. Fibrous dysplasia is usually seen in the posterior maxilla and radiographically appears as a diffuse, contrast-enhancing mass that may resemble ground glass, orange peel, or cotton wool. Odontomas often complicate the physiological eruption of permanent teeth, while their radiological density is almost identical to that of enamel and dentin, and the affected area is always surrounded by a radiopaque capsule.

conclusions

During the clinical examination of patients with osteomas, significant attention should be paid to the family history to exclude a possible diagnosis of Gardner's syndrome. Asymptomatic osteomas should be under constant observation, and pathologies that cause uncomfortable symptoms should be surgically removed, having previously organized a complex of clinical and radiological studies to accurately make the final diagnosis.

By Rohit Agrawal Shipra Agrawal Shitij Bhargava Mahesh Motlani Rahul Agrawal

Diet

Dietary table No. 1a

• Efficacy: therapeutic effect after a week
• Terms: 3-10 days
• Cost of food: 900-1100 rubles per week

Diet table No. 5a

• Efficacy: therapeutic effect after 5-7 days
• Time frame: 2-6 weeks
• Cost of products: 1300 - 1400 rubles per week

Diet 11 table

• Efficacy: therapeutic effect after a month
• Terms: 2 months or more
• Cost of products: 1800-1900 rubles. in Week

of diarrhea while undergoing chemotherapy . In this regard, a diet excluding spicy, fatty foods containing fiber (vegetables, nuts, seeds, bran bread) is recommended.

Fried foods and simple carbohydrates, which increase intestinal motility, are also excluded. A balanced diet during this period should contain a sufficient amount of protein (boiled meat, fish, steamed cutlets, meat soufflé). Dishes must be pureed - soups, cereals, meat. During this period, you can use Diet No. 1B and No. 5P . When diarrhea disappears and the patient’s condition improves, the patient is transferred to Table No. 11 . The diet of this diet is suitable for the recovery period. The patient’s diet is complete, containing the necessary vitamins, nutrients and minerals. Daily calorie content is increased (3000 kcal). Food is taken in 5 divided doses. The goal of the diet is to normalize weight while reducing it and improve the function of all body systems.

All foods and cooking methods are allowed, but nevertheless, it is better to adhere to a healthy diet, in which preference is given to stewed, boiled or baked dishes, vegetable and cereal soups that are not fried. To replenish vitamins, the diet should contain fresh seasonal vegetables, fruits and berries. It is important to choose healthy and natural products that do not contain dyes, flavor enhancers or preservatives. The diet must contain dairy products: natural milk, kefir, cottage cheese, cheeses, yogurt. Dairy products are important for replenishing calcium, which is constantly lost from bones. The calorie content of the diet should not be increased by fatty foods, cakes, cream pies, semi-finished products, beef and lamb fat, fatty sausages, ready-made sauces, pastes, mayonnaise, and sweet carbonated water.

Prevention

There is no specific prevention. However, you can perform non-specific activities:

• Observation and treatment of precancerous diseases of the skeletal system. Processes that tend to become malignant include chondroma, fibrous degeneration, Paget's disease, and osteochondral exostoses. The malignancy rate of these benign formations reaches 15%. It is important to undergo follow-up examinations annually to detect malignancy at an early stage. Healthy lifestyle. Balanced diet. Avoiding injuries and occupational hazards.

Secondary relapse prevention includes:

• The use of drugs that restore the immune system after chemotherapy and radiation therapy .
• Complete nutrition, rich in vitamins, antioxidants, proteins.
• Rejection of bad habits.
• Prevention of viral infections.
• Preventive examinations with an oncologist. Local relapses can be detected visually. It is important to have examinations during the first 4 years. After this, local relapses are rare.
• Carrying out diagnostic procedures according to the protocol. Metastases develop in 95% of cases within the first 5 years and most often affect the lungs. Bone metastases present with pain, and they appear simultaneously with pulmonary metastases.

Characteristic signs of osteosarcoma

The triad of clinical manifestations characteristic of osteosarcoma:

• 
  impaired mobility of the affected limb;

• the appearance of swelling;
• pain that gets worse over time.

These signs can be attributed to many diseases of the musculoskeletal system, so there is a possibility of misdiagnosis. The Meir Clinic in Israel conducts examinations using advanced programs and uses the latest equipment, so errors in diagnosis are excluded in the hospital.

Depending on the clinical signs, the disease is divided into two types:

• rapidly developing osteosarcoma - the disease begins suddenly, is accompanied by severe pain and rapid progression; if the patient does not go to the hospital in time, the outcome will be unfavorable;
• slowly developing osteosarcoma - the disease manifests itself poorly; if you consult a doctor in a timely manner, bone cancer treatment abroad will be successful.

Tumors that grow rapidly metastasize to the lungs. Such metastases are detected during the first diagnostic procedures. Often aggressive forms of the disease are found in children.

Pain is a cardinal symptom of the disease. At first, the pain is moderate and intermittent, often occurring during physical activity. Pain appears when the disease spreads to the periosteum. As the disease progresses, the intensity of the pain increases and it is constantly present.

A high degree of intensity is characterized by osteosarcoma, which develops in the bones near the lower leg. The pain often occurs at night, and there is no sign of a deterioration in the person's general health. Some patients note a slight increase in body temperature.

The main diagnostic indicator of osteosarcoma is swelling in the affected area. It occurs against the background of tumor progression, while the soft tissues located above the tumor become tense, a shine appears on the skin, and the subcutaneous veins dilate. As a result, the mobility of the affected limb is limited - it bends poorly, and the patient begins to limp. Gradually, the muscles of the limb atrophy.

General clinical signs of osteosarcoma occur in the later stages of the disease and are manifested:

• general malaise;
• losing weight;
• weakness;
• insomnia;
• Pathological fractures often occur in 10% of patients.

To ensure a good prognosis for osteosarcoma, it is necessary to visit the clinic at the first signs of pathology and undergo advanced cancer treatment in Israel.

Consequences and complications

The course of the disease itself is complicated by fractures and infection of soft tissues. All bone cancers after treatment have consequences:

• Secondary cancer , which develops as a result of exposure to radiation.
• Chronic heart failure (exposure to doxorubicin ).
• Osteoporosis.
• Hearing and vision impairments.
• Renal dysfunction and renal failure .
• Endocrinopathies.
• Impaired fertility ( infertility ). Reproductive functions suffer after chemotherapy with alkylating drugs ( Ifosfamide , Carboplatin , Cyclophosphamide ) and after the use of radiotherapy in the pelvis, abdomen, and spine. Menstrual irregularities are always observed after high-dose chemotherapy and stem cell transplantation.
• The consequences of the operation are significant - after amputation, limb prosthetics and long-term rehabilitation are necessary.

Forecast

The prognosis of osteosarcoma is determined by many factors: age, size, localization, stage, “purity” of the resection margin, histological conclusion.

How long do people live with bone cancer? With complex treatment of osteosarcoma, 70% of patients live 5 years or more, with Ewing's sarcoma - 60% of patients.

Some authors give a higher survival rate. If there is a localized distal lesion, radical resection is performed and the result of chemotherapy treatment is more than 90% tumor necrosis, then 5-year survival is observed in 80% of cases.

The prognosis is worse in case of proximal localization, large tumor size, poor response to chemotherapy before surgery and metastases. Poor prognosis for tumors in the pelvic bones and weight loss at diagnosis. It is known that the intensity of tumor cell apoptosis also affects prognosis. Also unfavorable factors are early relapse, multiple lesions in the lungs and pleural involvement. If patients have stage 4, how long do they live in this case? When removing metastases in the lungs, 12-20% occur; if metastasectomy is not performed, 2.5% of patients survive. Life spans are calculated in months.

List of sources

• Clinical guidelines for bone sarcomas, 2022, 51 p.
• Practical recommendations for the treatment of primary malignant bone tumors (osteosarcoma, Ewing's sarcoma) / Malignant tumors. Russian Society of Clinical Oncology. 2022, pp. 283-295.
• Clinical guidelines for the diagnosis and treatment of patients with primary malignant bone tumors. 2014, 25 p.
• Clinical protocol for diagnosis and treatment. Bone sarcoma, 2015, 19 p.
• Belokon O.V., Nechval V.N. Surgical treatment of children with osteogenic sarcoma with metastases to the lungs/Health of Ukraine, 2012, pp. 34-35.