International Classification of Diseases for Oncology

(ICD-O, second edition, 1990)

Bone-forming

Benign

Osteoid osteoma

Osteoblastoma

Malignant

Osteosarcoma

Juxtacortical osteosarcoma

Malignant osteoblastoma (primary or secondary)

Cartilage-forming benign

Solitary

Chondroma central (enchondroma)

Juxtacortical (periosteal)

Osteochondroma (bone- cartilaginous exostosis, ecchondroma)

Chondroblastoma

Chondrodysplasia

Chondromyxoid fibroma

Multiple

Multiple chondromas

Juxtacortical (periosteal) enchondromatosis calcific and ossifying

Enchondromatosis is predominantly unilateral (Olier disease or dyschondroplasia, Maffucci syndrome)

Osteochondromatosis, congenital multiple exostoses, congenital deformities

Cartilaginous malignant

Chondrosarcoma (primary and secondary)

Juxtacortical chondrosarcoma

Giant cell tumor (osteoclastoma)

Malignant bone marrow tumors

Ewing's sarcoma

Malignant lymphoma of bone: reticulosarcoma and lymphosarcoma

Vascular tumors

Benign

Hemangioma

Lymphangioma

Glomus tumor

Intermediate and malignant

Hemangioendothelioma

Hemangiopericytoma

Angiosarcoma

Other connective tissue tumors

Benign

Desmoplastic fibroma

Malignant

Fibrosarcoma

Liposarcoma

Malignant mesenchymoma

Malignant fibrous histiocytoma

Leiomyosarcoma

Other tumors

"Adamantinoma" of long bones (angioblastoma)

Sarcomas developing against the background of previous processes

Sarcomas in Paget's disease

Sarcomas after irradiation

Tumor-like diseases

Solitary bone cyst (simple or unilocular cyst)

ACC (multilocular blood bone cyst)

Juxtacortical bone cyst (intraosseous ganglion)

Metaphyseal fibrous defect (non-ossifying fibroma)

Eosinophilic granuloma

Solitary eosinophilic granuloma

Fibrous dysplasia

Myositis ossificans (heterotopic calcifications)

"Brown tumors" in hyperparathyroidosis

Epidermoid cysts

Tumors and tumor-like diseases of synovial nature

Pseudotumor processes

Localized nodular synovitis

Pigmented villonodular synovitis

Osteochondromatosis

Chondroma

Synovial sarcoma

Features of diagnosing tumor lesions of bones

Diagnosis of tumor lesions of bones is quite difficult due to the significant diversity and lack of obvious early symptoms. The depth of the location of the process, the difficulty of assessing the patient’s condition and the lack of clear complaints limit the diagnostic possibilities. In the same time clinical diagnosis has features. Complaints, especially in children, arise only when the focus is in bone tissue reaches significant sizes. General symptoms may include increased body temperature, general weakness And sharp pain when the process reaches the periosteum. In dysplastic processes, the cause of the disease can often be trauma, which is not detected in the early stages. The leading place in this case is taken by the collection of anamnesis. The pain is vague, often radiating. During a malignant process, its intensity rapidly progresses, it becomes constant (disturbs even at night).

Local changes in malignant course tumor process- in the bones are detected in the form of swelling, often deformation, and skin changes due to difficulty in blood outflow and expansion of the saphenous veins.

With a benign process, pain syndrome is almost absent, but deformations appear, sometimes pathological fractures. With dysplasia, the pain is mild but constant, and gradual development of deformity is possible.

When collecting anamnesis, great attention should be paid to heredity.

The general condition of dysplasia and benign tumors remains largely unchanged. With a malignant process in young children, the condition resembles an acute inflammatory process with high temperature body, leukocytosis, increased ESR. The appearance of the patient does not correspond to the severity of the disease, and exhaustion and anemia occur mainly in advanced stages of bone sarcoma. In adults, this process is less intense.

By palpation it is possible to detect a bone tumor at an early stage of its development only in places where there is little soft tissue, or when the tumor is located periosteally or subperiosteally. Bone tumor metastases are never palpable.

In most cases, it is possible to clarify the consistency of the tumor by palpation in case of a shallow tumor, while paying attention to the color of the skin over the tumor, the mobility of soft tissues, the presence of an expanded venous network or pulsation of blood vessels.

With some types of dysplasia, it is possible to identify skin changes accompanying the tumor in the form of pigmentation, hyperkeratosis, angiomatous and varicose venous formations (for example, with Maffucci syndrome). When determining the growth of a tumor, especially a malignant one, over time, it is necessary to measure the circumference of the limb on the diseased and healthy sides at the same level.

Benign and dysplastic bone lesions progress very slowly. The degree of impairment of limb function complements information about the prevalence and nature of bone damage. Thus, tumors located in the diaphyseal region do not lead to movement disorders, but with epiphyseal localizations they can lead to neuro-reflex contractures, especially early in osteogenic sarcomas. With the metaphyseal location of the tumor, movement in the joint is not impaired and pain is not expressed. Most often, dysfunction of the joint is associated with a pathological fracture, which, as a rule, occurs in the late stage of development of the tumor process.

The patient's age is essential in determining the nature of the tumor. Thus, children are characterized by primary bone tumors; metastatic tumors are extremely rare, while in adults metastatic tumors are 20 times more common than primary ones malignant tumors.

In some cases, tumor or dysplastic processes have a characteristic localization. Thus, chondromas are often located in the phalanges of bones, eosinophilic granuloma is in the bones of the calvarium, and foci of dyschondroplasia are in the distal extremities. Benign tumors most often located within the tubular bone, metadiaphyseal region. Cartilaginous tumors in children they are almost always associated with epiphyseal growth cartilage; in the metaphyses of tubular bones they grow like ecchondromas, while in older people they grow centrally, in the form of enchondromas. Dysplasia affects the ends of the bones that form the knee joint, the proximal end of the femur, the upper jaw, etc.

Clinical and laboratory data (general blood and urine tests) for benign tumors and dysplasia do not change noticeably. At the same time, during malignant processes, especially such as osteogenic sarcoma, Ewing's sarcoma and reticulosarcoma, significant changes are detected in the blood in the form of leukocytosis, lymphocytosis, monocytosis, increased ESR; with multiple myeloma, leukopenia, anemia, thrombocytopenia and increased ESR are detected.

Biochemical studies are very important to determine the nature and stage of the tumor process. Thus, in myeloma, hyperproteinemia is expressed (up to 100-160 g/l) with an increase in the content α 2 -,β- and γ- globulins. A specific Bence-Jones protein is detected in the urine of such patients. In malignant tumors there is usually a sharp decrease in the content total protein due to a decrease in the amount of albumin with a slight increase in the content of globulins. The content of sialic acids is one of the indicators of tumor growth. Thus, with slowly growing tumors it is within normal limits, and with fast-growing benign tumors it increases, with a malignant process it increases sharply, especially with metastasis. The activity of proteolytic enzymes, in particular chymotrypsin, in blood serum can help with differential diagnosis malignancy of the process. Increased excretion of total hydroxyproline in the urine indirectly indicates malignancy of the process.

Elements such as calcium, phosphorus and sodium play an important role in the functioning of bone tissue. Studying the state of phosphorus-calcium metabolism (calcium, phosphorus content and alkaline phosphatase activity in the blood, calcium and phosphorus content in the urine) is very important when examining a patient. Alkaline phosphatase activity changes mainly in osteogenic sarcoma, osteoid osteoma, and in some cases in osteoblastoclastoma after a pathological fracture; The calcium content in the blood increases during a malignant process and during parathyroid osteodystrophy.

X-ray examination allows us to clarify the diagnosis and such indicators as the border of the lesion, its structure; this method is leading in the differential diagnosis of malignant, benign and dysplastic processes (Fig. 1). Tomography, radiography with contrast, radionuclide diagnostics and the use of magnetic resonance imaging make it possible to make a diagnosis with greater accuracy.

Rice. 1.

When differentiating dysplastic processes from inflammatory ones, it is necessary to compare the general signs of changes in bone tissue (Table 1).

Table 1. Differential diagnostic signs of a tumor and a number of other diseases (according to V.D. Chaplin)

Sign	Tumor	Chronic osteomyelitis	Tuberculosis	Fibrous dysplasia
Destruction
Periosteal reaction
Sequestration
Bone necrosis
Bone atrophy

The final diagnosis is made on the basis of clinical, radiological and morphological data.

It should be borne in mind that with age, a transition from one state of bone tissue to another is possible. Thus, juvenile cartilaginous exostosis can develop into chondroma; cartilaginous dysplasia in adulthood - into chondrosarcoma; fibrous osteodysplasia - into osteogenic sarcoma. Therefore, after identifying a tumor process and with appropriate indications, it is necessary to perform a radical operation - resection of the tumor and mandatory restoration of the ability to support and function of the limb. In some cases, combined treatment is used for a malignant process - surgery in combination with chemotherapy and radiation treatment.

Benign tumors

Lipoma- a benign tumor of subcutaneous fatty tissue, surrounded by a thin connective tissue capsule. Typically, lipomas develop upon reaching puberty, more often in women, they are single, less often multiple, and are located superficially under the skin of the neck, back, and axillary region.

Upon palpation, a painless formation of soft-elastic consistency, delimited, often lobular, is determined. Lipomas associated with nerves (neurolipomas) are painful. Lipomas containing fibrous inclusions connective tissue(fibrolipomas), are multiple, of varying sizes, and are also painful on palpation. There are intermuscular lipomas, which are most often localized in the axillary region, on the thigh, and forearm. They have a variety of shapes and grow deep down to the bone. On palpation, their elasticity, absence of sharp boundaries and pain are felt.

Lipomas in the synovial membranes of joints are quite rare, but in mucous bursae and tendon sheaths they are relatively common.

There are two forms of lipomas - simple and branched. Clinical symptoms include pain, swelling in the area, and clicking in the joint when moving. Often there is effusion in the joint. Their presence in tendons reduces the strength of the latter and promotes rupture. Lipomas are most often located in the tendon sheaths of the extensor muscles of the fingers, toes and ankle joints.

Macroscopically, a simple lipoma is a single round-shaped formation; branched lipoma has a roughly villous, nodular appearance due to diffuse proliferation of adipose tissue in the synovium.

Lipoma of the bone itself is extremely rare. It often develops in the form peripheral tumor- periosteal. There are no characteristic clinical and radiological signs. In the vertebrae it occurs in the form of small growths of adipose tissue; sometimes peripheral lipomas located subperiosteally are detected.

Surgical treatment for limited joint function or impaired cosmetic appearance.

Hemangioma- benign tumor growing from blood vessels, most often of congenital etiology. It grows in childhood; when the child grows, its growth stops. Tends to grow into tissue. There are the following types of hemangiomas: simple, cavernous and branched. A simple hemangioma is an expansion of the skin in the form of birthmark red-blue color. When pressed, it decreases until it subsides and disappears; after the pressure stops, it appears again. Cavernous hemangioma has a nodular structure with developed cavities filled with blood, the size of the nodes varies. When you press on the tumor, it turns pale or disappears. Branched hemangioma consists of highly dilated and thickened pulsating vessels. More often they are located on the hand with transition to the forearm. When listening, a double (arterial-venous) pulsating noise is noted, and there are often trophic changes on the skin.

Hemangiomas in the muscles of the extremities (thighs, legs) are rare in the form of small, dense, round-shaped vascular tumors (so-called angiofibromas). They are painful on palpation, have clear boundaries and do not subside.

Hemangiomas of synovial membranes and tendon bursae are also rare and are localized in the synovial membrane outside the joint and in the fascia. They are clinically manifested by pain, palpation is determined by a doughy swelling, which increases and decreases when the limb is raised and lowered. When hemangiomas are located inside joints, blockages of the latter often occur.

X-rays reveal hematomas only when phleboliths are formed or their stroma ossifies.

Surgical treatment is carried out strictly according to indications: with a simple form, more often with a joint blockade; with a branched form, the operation is much more complicated.

Fibroma- a benign tumor of connective tissue origin, the basis of which is fascia, aponeuroses and muscle tendons.

It is rare and grows slowly over a limited area. Upon palpation, it is revealed as a smooth elastic mobile formation. Rarely causes vascular compression and limb dysfunction.

A tumor of the connective tissue sheaths of nerves is called neurofibroma. It is located on the skin, which is usually brown, sometimes cyanotic in color. It is located (for example, in neurofibromatosis) projectionally along the intercostal nerves. It has a different shape: round, oblong, can hang on a leg or look like folds of skin (folded elephantiasis), located on the nerves of the limbs.

There are no movement disorders in neurofibroma, but there is a disorder pain sensitivity in the form of anesthesia, hyperesthesia, paresthesia. Palpation and pressure on the neurofibroma are painless, but can cause paresthesia along the nerve.

Benign soft tissue tumors also include fibromatosis, in particular palmar fibromatosis (Dupuytren's contracture), plantar fibromatosis (Lederhose disease). The first is expressed by the appearance of several nodules and cords in the palmar aponeurosis of the hand, which grow slowly.

It occurs mainly in the elderly, progresses slowly and leads to flexion contracture of the fourth and fifth fingers. The course is long, benign, the process is limited only by the aponeurosis. After surgical removal, relapses are possible.

Lederhose disease is a fibromatosis lesion of the plantar aponeurosis of the foot, often involving subcutaneous tissue and skin. Occurs at any age, most often after 30 years.

Treatment is predominantly non-operative. After surgical removal of the altered plantar aponeurosis, relapses are possible.

Ganglion refers to tumor-like cystic diseases of the tendons or joint capsule.

The tumor-like formation has a spherical shape, often the size of a hazelnut, mobile, slightly fluctuating. The most typical location is on the back surface wrist joint(between i.e. extensor indicts And t. extensor carpi radialis brevis), less often located at the level of the wrist and main phalanges. The patient sometimes experiences neuralgic pain in the hand or throughout the arm, increased fatigue and aching in the muscles of the forearm. The skin over the ganglion can be folded, it is mobile, the consistency of the tumor is tight-elastic.

Treatment: rest, physical activity, limitation of physical activity. In chronic cases it is indicated surgical treatment- deletion.

Osteoblastoclastoma(giant cell tumor of bone) is distinguished by a unique clinical course, a polymorphic X-ray picture and a special type of blood circulation, giving a specific histological picture. There are benign and malignant variants. Osteoblastoclastoma was first described in 1818 as a malignant form, and in 1853 by J. Paget as a benign giant cell form.

In accordance with the II edition International classification diseases for oncology (ICD-O) there are four forms of the disease:

1) giant cell tumor of bone;

2) malignant giant cell tumor of bone;

3) giant cell tumor of soft tissues;

4) malignant giant cell tumor of soft tissue.

Clinical forms of the disease. Juvenile, or solitary, bone cyst is a form of osteoblastoclastoma. Depending on the nature of growth and clinical and radiological data, three forms of the tumor are distinguished:

1) lytic, with rapid growth and destruction of a lytic nature;

2) active cystic, with an active increase in the cystic focus;

3) passive-cystic - in fact, this is the outcome of the tumor without its obvious growth.

Osteoblastoclastoma is observed in people under 30 years of age and affects the metaphysis of long tubular bones - humerus, femur, tibia, etc. Diagnosis of the disease is difficult in the early stage. The onset of the lytic form of osteoblastoclastoma is characterized by rapid bone growth and pain. Later, the local temperature rises, the tumor is palpable, and the saphenous veins dilate.

As a result of thinning of the cortical layer of the bone tumor, pain during palpation is added to pain at rest, and then painful contracture occurs in the nearest joint.

In the cystic form, the tumor is asymptomatic and is detected accidentally after injury. A pathological fracture in this form is one of the first symptoms of the disease.

In patients with the active cystic form of the disease (Fig. 2), pain and gait disturbance are observed due to the reaction of the joint closest to the tumor. A fusiform swelling of the bone is determined by palpation.

Rice. 2.

X-ray picture. A peculiarity of osteoblastoclastoma is its localization in the metaphyses of long tubular bones in children; in adults, in lytic forms, it moves to the epiphysis. The type of tumor is predominantly oval enlightenment with clear boundaries within the bone; with the active cystic form the boundaries are not clear everywhere, but with the lytic form they are clear on all sides. Initially, the tumor is located eccentrically, and then centrally. The cortical layer swells up to the periosteum. An important radiological sign of osteoblastoclastoma is the absence general osteoporosis. The exception is the lytic form of the tumor with prolonged immobilization.

At the moment of breakthrough of lytic osteoblastoclastoma beyond the periosteum, a bone “visor” is visible on the radiograph, simulating osteogenic sarcoma (Fig. 3).

Rice. 3. Lytic form of osteoblastoclastoma of the femur: a - radiographs in frontal and lateral projections; b - macroscopic specimen of a removed tumor

These tumors behave differently in relation to the epiphyseal cartilage. In the lytic form, the epiphyseal cartilage is damaged, causing growth retardation; the lesion extends to the articular cartilage, but the latter is not affected. In the active cystic form, osteoblastoclastoma does not penetrate the epiphysis, but “stops” near it, disrupting nutrition and, as a consequence, the function of the growth zone, which causes significant shortening of the limb.

Clinical and radiological observations of fractures in osteoblastoclastomas indicate good bone healing, however, in the active-cystic form, tumor growth increases, and in the passive-cystic form it is inhibited. The bone deformations that existed before the fracture remain and are difficult to eliminate.

Laboratory research. In lytic forms of tumors there are increase in ESR, leukocytosis, phosphorus-calcium metabolism changes in the presence of a fracture due to its consolidation. In some cases, alkaline phosphatase activity increases.

Morphologically macroscopic examination reveals foci of the lytic form of osteoblastoclastoma, which are brown blood clots filling the tumor; in the active-cystic form, the cortical layer is not damaged, the bone seems to be stretched circularly and contains a significant number of bone partitions; inside there is a jelly-like mass resembling blood. In the passive-cystic form, the lesion consists of serous fluid enclosed in a dense bone box or fibrous membrane.

Treatment surgical. The tactics of tumor resection for osteoblastoclastoma depends on the form of the disease. Thus, with the lytic form, it is necessary to remove the tumor while preserving part of the periosteum for osteogenesis. In the cystic form, subperiosteal resection is performed, preserving the cortical layer along one edge. The surgeon's tactics in the presence of a pathological fracture due to cystic forms of osteoblastoclastoma should be wait-and-see, but the operation is performed no earlier than a month after the formation of a good callus. It should be especially noted the need to comply with ablastics during surgery.

The prognosis for benign osteoblastoclastomas is favorable; with the lytic form, an unfavorable outcome is possible.

Chondroma- a benign tumor of cartilage tissue, its frequency is 4% of all primary bone tumors and dysplasias in children. According to the nature of growth, they are distinguished: 1) ecchondromas (Fig. 3, a, b), growing exophytically; 2) enchondromas growing inside the bone (Fig. 3, c).

Rice. 3. Chondromas: a - chondroma of the upper third of the tibia; b - ecchondroma of the pelvis; c - enchondroma of the main phalanx of the first toe

Along with primary chondromas, secondary ones develop - based on dysplasia or a benign tumor. Chondromas are true tumors and differ from osteochondral exostoses in that the latter have autonomous growth regardless of skeletal growth.

Clinical picture. Chondroma is characterized by a solitary focus in one bone, while multiple chondromas are secondary neoplasms of a dysplastic nature (Fig. 3, c).

Most often, chondroma is localized in the metatarsals, metacarpals, bones of the phalanges of the fingers and toes, then in the ribs and sternum, i.e. in the bones with the largest number cartilage tissue. Pain syndrome with ecchondroma is usually caused by swelling of the bone tissue and periosteum. Enchondroma remains asymptomatic for a long time. Deformities as a result of the growth of chondroma develop mainly on the fingers and toes.

Radiologically, ecchondromas are characterized by clear boundaries in the form of a cortical “shell”, clearly visible at the base of the bone. The cartilaginous tissue of ecchondroma is an oval and spherical accumulation with bone inclusions.

Enchondromas are located centrally in the metaphysis and diaphysis. On an x-ray, they look like cysts - an oval or round sparse space with calcareous inclusions in the center and a small area of ​​sclerosis around.

Morphological picture. Chondroma with macroscopic examination It is a dense tumor with a bumpy, pearlescent surface.

Treatment surgical - partial marginal bone resection; segmental resection is performed only in doubtful cases. Tumor recurrences are extremely rare.

Malignant tumors

An exclusively malignant tumor, its frequency is 18% of all tumors and 62% of malignant bone tumors in children.

The term “osteogenic sarcoma” was proposed in 1920 by I. Ewing. This primary neoplasm originates from the bone itself and is composed of undifferentiated mesenchymal cells.

Based on the histological picture (based on the predominance of a particular tissue), osteoblastic, chondroblastic and fibroblastic sarcomas are distinguished. Osteogenic sarcomas are localized predominantly in long tubular bones (Fig. 4), especially those forming the knee joint (75% of cases).

Rice. 4.

Osteogenic sarcomas typically involve only one bone, even with metastasis. Metastases can be to the lungs, liver, The lymph nodes.

The clinical picture at the onset of the disease is uncertain. Pain syndrome comes first. These are intense independent pains, even at rest. The tumor grows rapidly, causing swelling of the soft tissues and skin. The skin becomes blue due to venous stagnation, the saphenous veins dilate, and the skin over the tumor becomes thinner.

By the 3-4th month from the onset of the disease, a painful contracture occurs in the nearest joint, due to which the limb becomes non-supporting.

Palpation of the tumor has a dense consistency, in places there are areas of softening as a result of tissue breakdown. The crunching sound of a tumor when pressure is applied to it is late symptom diseases. Regional lymph nodes do not enlarge. The general condition of a patient with osteogenic sarcoma does not change, and by the 3-4th month, when the tumor becomes large, the body temperature rises to 38...39 °C.

Laboratory research. Changes in the blood are detected in the form of leukocytosis, increased ESR, and anemia in an advanced stage.

Increased activity of alkaline phosphatase in the blood and urine indicates a malignant course of the process.

X-ray picture the initial stage of sarcoma is characterized by focal osteoporosis, blurred and unclear contours of the tumor, which usually do not spread to the epiphysis; destruction of the bone structure is determined in the form of defects (Fig. 5, a-d). When the tumor reaches the periosteum and peels it off, the pain increases sharply. When the detached periosteum is destroyed, a typical “visor” is visible on the radiograph. In the first months, the “visor” and periostitis are small, then the tumor spreads along the medullary canal to the center of the diaphysis, and the detached periosteum is determined in the form of a “swollen” periostitis. Areas of diffuse osteoporosis of the bone can be combined with sclerotic foci, the so-called osteoid bundles. A characteristic radiological sign appears - a symptom of needle periostitis.

Rice. 5. Osteogenic sarcoma: a - osteogenic sarcoma of the lower third of the femur; b - appearance removed tumor; c - osteogenic sarcoma of the fibula; d - osteogenic sarcoma of the femur

According to M.V. Volkov, there are three stages of osteogenic sarcoma.

Stage I characterized by pain without external manifestations of the tumor. On x-ray, it appears as a poorly defined lesion of osteoporosis with sclerotic bone inclusions within the normal boundaries of the bone.

Stage II - swelling of the soft tissues appears in the tumor area, some expansion of the network of saphenous veins, the first radiological signs sarcoma in the form of destruction of the periosteum exfoliated by the tumor (the “visor” symptom).

Stage III- visible and palpable tumor. The skin over it is thinned, the venous pattern is expanded. The radiograph shows a large “visor” and spicule-like spotty deposition of bone substance in the soft tissues. The pain is unbearable. The function of the limb is impaired (static and dynamic).

The examination should include radiography and magnetic resonance or computed tomography of the affected segment, radiography and computed tomography chest, scintigraphy of the skeleton and liver, ultrasound of the abdominal cavity and retroperitoneal space, trephine biopsy of the tumor with cyto- and histological verification of the diagnosis.

Treatment Osteogenic sarcoma remains one of the difficult problems of bone oncology. Currently, the method of choice is combination treatment, including a combination of cyclic chemotherapy and surgery (for damage to long tubular bones). Most of the used neoadjuvant (preoperative) and adjuvant (postoperative) drug therapy regimens contain various combinations methotrexate, phosphamide, cisplatin, doxorubicin. Surgical organ-preserving treatment consists of ablastic removal of the tumor within healthy tissues, followed by replacement of the resulting defect (endoprosthetics, bone grafting, arthrodesis). If it is impossible to perform organ-preserving surgery, amputation or disarticulation of the limb is performed, followed by prosthetics. Survival of patients after combination treatment is 60-70%, and the frequency of local tumor relapses after organ-sparing operations is 5-8%.

Bone hemangioma. Bone hemangioma is a tumor of non-osteogenic origin. Primary bone hemangioma arises from the vascular elements of the red bone marrow.

Pathogenesis. Blood sinuses in the bone communicate with bone capillaries, push bone elements apart and thereby cause osteoporosis, atrophy of bone beams, and the bone tissue elements themselves become calcified. The most common location of hemangioma is the spine, then the skull bones and the humerus (Fig. 6). It is observed at any age.

Rice. 6.

Clinical picture hemangiomas of the long tubular bone, indeterminate. The first sign of a tumor is pain, constant in intensity. The swelling occurs quite late, when the hemangioma extends beyond the bone. The diagnosis is difficult to make even radiographically; X-ray vasography is the most informative. X-ray examination of spinal hemangioma reveals a fine-mesh pattern of the affected vertebra and a radiant structure of protruding bone trabeculae.

An early sign of vertebral hemangioma is osteoporosis, local, without destruction. The second sign is the absence of changes in the intervertebral discs.

With hemangioma of the long tubular bone, there are no characteristic radiological signs. Primary hemangioma, growing from bone vessels, has a outlined appearance, while the cortical layer of the bone can be narrowed, and the bone itself is thinned. With secondary hemangioma, the tumor borders are fringed.

Treatment for spinal hemangioma - radiotherapy. For hemangioma of the tubular bone - marginal or complete resection of the bone with plastic replacement of the defect.

Multiple myeloma. Myeloma is a malignant bone tumor of non-osteogenic origin that develops from proliferating plasma cells of the bone marrow. Multiple myeloma was described in 1873 by O. A. Rustitsky. There are four forms of the disease:

1) multifocal;

2) diffuse osteoporotic without localized tumor nodes - generalized myelomatosis of the red bone marrow;

3) osteosclerotic;

4) solitary.

The disease is most often observed at the age of 50-60 years and is extremely rare in children. Mostly flat bones are affected - ribs, skull, pelvis.

Clinical picture. Manifestations of the disease begin with a general loss of strength, loss of ability to work, aching pain in bones such as rheumatic, weight loss. Quite often pathological fractures occur, especially of the ribs, and radicular disorders are possible. Patients may experience a decrease in blood pressure.

Laboratory data: Bence Jones protein is detected in the urine in 70% of cases, and at the same time the calcium content in the urine increases. In the blood - hyperproteinemia, often anemia.

X-ray in the initial stage shows diffuse osteoporosis, then multiple foci of osteolysis are detected with the absence of periosteal and endosteal reactions (Fig. 7).

Rice. 7.

Subsequently, the process spreads to other bones, pathological fractures, compression of the vertebral bodies, and cachexia occur. Myeloma metastasizes to parenchymal organs: spleen, liver and rarely to the lungs.

Treatment. Patients with single and multiple myeloma require radiotherapy; Chemotherapy and administration of corticosteroids are indicated. The effect of treatment is short-lived.

The prognosis for life is unfavorable.

Traumatology and orthopedics. N. V. Kornilov

- a group of malignant and benign neoplasms arising from bone or cartilage tissue. Most often, this group includes primary tumors, but a number of researchers also call bone tumors secondary processes that develop in the bones during metastasis of malignant neoplasms located in other organs. To clarify the diagnosis, radiography, CT, MRI, ultrasound, radionuclide methods and tissue biopsy are used. Treatment of benign and primary malignant bone tumors is usually surgical. For metastases, conservative methods are more often used.

ICD-10

C40 C41 D16

General information

Bone tumors are malignant or benign degeneration of bone or cartilage tissue. Primary malignant bone tumors are rare and account for about 0.2-1% of the total number of tumors. Secondary (metastatic) bone tumors are a common complication of other malignancies, such as lung cancer or breast cancer. Primary bone tumors are more often detected in children, and secondary ones in adults.

Benign bone tumors are found less often than malignant ones. Most neoplasms are localized in the area of ​​tubular bones (in 40-70% of cases). The lower extremities are affected twice as often as the upper extremities. Proximal localization is considered a prognostically unfavorable sign - such tumors are more malignant and are accompanied by frequent relapses. The first peak of incidence occurs at 10-40 years (during this period, Ewing's sarcoma and osteosarcoma develop more often), the second - after the age of 60 years (fibrosarcomas, reticulosarcomas and chondrosarcomas occur more often). Orthopedists, traumatologists and oncologists treat benign bone tumors; only oncologists treat malignant ones.

Benign bone tumors

Osteoma– one of the most favorable benign bone tumors. It is a normal spongy or compact bone with elements of restructuring. Osteoma is most often detected in adolescence and adolescence. It grows very slowly and can be asymptomatic for years. Usually localized in the bones of the skull (compact osteoma), humerus and thigh bones(mixed and spongy osteomas). The only dangerous localization is on the inner plate of the skull bones, since the tumor can compress the brain, causing an increase in intracranial pressure, seizures, memory disorders and headaches.

It is a stationary, smooth, dense, painless formation. On an x-ray of the skull bones, a compact osteoma appears as an oval or round dense homogeneous formation with a wide base, clear boundaries and smooth contours. On radiographs of tubular bones, spongy and mixed osteomas are revealed as formations with a homogeneous structure and clear contours. Treatment is removal of the osteoma in combination with resection of the adjacent plate. In asymptomatic cases it is possible dynamic observation.

Osteoid osteoma– a bone tumor consisting of osteoid as well as immature bone tissue. Characterized by small sizes, a clearly visible zone of reactive bone formation and clear boundaries. It is more often detected in young men and is localized in the tubular bones of the lower extremities, less often in the area of ​​the humerus, pelvis, wrist bones and phalanges of the fingers. As a rule, it manifests itself with sharp pain, in some cases it may be asymptomatic. On radiographs it appears as an oval or round defect with clear contours, surrounded by a zone of sclerotic tissue. Treatment is resection along with the focus of sclerosis. The prognosis is favorable.

Osteoblastoma– a bone tumor, similar in structure to osteoid osteoma, but differing from it in its larger size. Typically affects the spine, femur, tibia and pelvic bones. Manifests itself as a severe pain syndrome. In the case of a superficial location, atrophy, hyperemia and swelling of soft tissues may be detected. X-ray reveals an oval or rounded area of ​​osteolysis with unclear contours, surrounded by a zone of slight perifocal sclerosis. Treatment is resection along with the sclerotic area located around the tumor. At complete removal the prognosis is favorable.

Osteochondroma(another name for osteochondral exostosis) is a bone tumor that is localized in the cartilage zone of long tubular bones. It consists of a bone base covered with cartilage. In 30% of cases, osteochondroma is detected in the knee area. It can develop in the proximal humerus, head of the fibula, spine and pelvic bones. Due to its location near the joint, it often causes reactive arthritis and impaired limb function. X-ray examination reveals a clearly defined, tuberous tumor with an inhomogeneous structure on a wide stalk. Treatment is resection; if a significant defect is formed, bone grafting is performed. In case of multiple exostoses, dynamic observation is carried out; surgery is indicated when rapid growth or compression of adjacent anatomical structures. The prognosis is favorable.

Chondroma– a benign bone tumor that develops from cartilage tissue. Can be single or multiple. Chondroma is localized in the bones of the foot and hand, less often in the ribs and tubular bones. May be located in the medullary canal (enchondroma) or along outer surface bones (ecchondroma). It becomes malignant in 5-8% of cases. Usually asymptomatic, mild pain is possible. On x-rays a round or oval focus of destruction with clear contours is determined. There is uneven expansion of the bone; in children, deformation and growth retardation of a limb segment is possible. Surgical treatment: resection (if necessary with endoprosthetics or bone grafting), when the bones of the foot and hand are affected, amputation of the fingers is sometimes required. The prognosis is favorable.

Malignant bone tumors

Osteogenic sarcoma– a bone tumor arising from bone tissue, prone to rapid development and rapid formation of metastases. Develops mainly between the ages of 10 and 30, men are affected twice as often as women. Usually localized in the metaepiphyses of the bones of the lower extremities, in 50% of cases it affects the femur, followed by the tibia, fibula, humerus, ulna, bones of the shoulder girdle and pelvis. On initial stages manifests itself as dull, unclear pain. Then the metaepiphyseal end of the bone thickens, the tissues become pasty, a visible venous network is formed, contractures form, the pain intensifies and becomes unbearable.

Manifests intense pain, difficulty moving in the adjacent joint and swelling of the soft tissues. When located in the vertebrae, lumbosacral radiculitis develops. The flow is usually slow. Radiographs reveal a focus of destruction. The cortical layer is destroyed, the periosteal overlays are not clearly expressed, they look like spicules or a visor. To clarify the diagnosis, MRI, CT, osteoscintigraphy, open and penetrating needle biopsy may be prescribed. Treatment is often complex – chemosurgical or radiosurgical.

Ewing's sarcoma is the third most common malignant bone tumor. More often it affects the distal parts of the long tubular bones of the lower extremities, less often detected in the area of ​​​​the bones of the shoulder girdle, ribs, pelvis and spine. Described in 1921 by James Ewing. Usually diagnosed in teenagers, boys are affected one and a half times more often than girls. It is an extremely aggressive tumor - even at the stage of diagnosis, metastases are detected in half of the patients, detected using conventional research methods. The incidence of micrometastasis is even higher.

In the early stages, it manifests itself as vague pain, intensifying at night and not subsiding with rest. Subsequently, the pain syndrome becomes intense, disrupts sleep, interferes with daily activity, and causes restriction of movements. On late stage x pathological fractures are possible. General symptoms are also characteristic: loss of appetite, cachexia, fever, anemia. Upon examination, dilation of the saphenous veins, pasty soft tissue, local hyperthermia and hyperemia are revealed.

To clarify the diagnosis, radiography, CT, MRI, positron emission tomography, angiography, osteoscintigraphy, ultrasound, trephine biopsy, tumor biopsy, molecular genetic and immunohistochemical studies may be prescribed. X-ray images reveal a zone with areas of destruction and osteosclerosis. The cortical layer is indistinct, stratified and disintegrated. Needle-shaped periostitis and a pronounced soft tissue component with a homogeneous structure are revealed.

Treatment – ​​multicomponent chemotherapy, radiation therapy, if possible radical removal tumors (including the soft tissue component), and in recent years organ-sparing operations have often been used. If it is impossible to completely remove the tumor, non-radical intervention is performed. All operations are performed against the background of pre- and postoperative radiation and chemotherapy. The five-year survival rate for Ewing's sarcoma is about 50%.

Bone tumors

What are Bone Tumors -

Bone tumors- a relatively rare occurrence. Their share among other tumors is about 1%. Bone tumors are characteristic mainly of young people.

The main proportion of bone tumors are osteosarcoma (50–60%), Ewing's sarcoma, chondrosarcoma and fibrosarcoma. In general, about 30 types of bone tumors are known.

What provokes / Causes of bone tumors:

The causes of bone tumors are still poorly understood. Trauma plays a role.

The causes of primary bone neoplasms are generally unknown. Persons suffering from chronic inflammatory disease bones (Paget's disease) are at higher risk of developing osteosarcoma in adulthood or old age, but this disease occurs in a very small number of people.

Secondary bone tumors always develop as a result of primary tumors in other organs. Sometimes a primary tumor may be the first manifestation of a primary cancer that is completely asymptomatic.

Symptoms of Bone Tumors:

Both primary and secondary bone tumors cause pain, which may be the first noticeable and significant symptom. The pain, as a rule, is localized deeply and is aching, constant in nature, disturbing both at rest and during physical activity. At first, the pain is not constant and worries more at night or when walking in case of damage lower limb. As the tumor grows, the pain becomes constant.

The pain intensifies with movement and, if the lower limb is affected, can lead to the development of lameness. A visible swelling may appear at the location of the pain, and it can also be detected by touch. Fractures are not very common, but can occur both in the area where the tumor is located and in its immediate vicinity. General symptoms occur during a widespread process and are expressed in a decrease in body weight, increased fatigue, weaknesses.

When classifying bone tumors, in addition to histological forms, the following are used:
- TNM and pTNM classification. They are applicable to all primary malignant bone tumors with the exception of malignant lymphoma, multiple myeloma, juxtacortical osteo- and juxtocortical chondrosarcoma.
- histopathological grade of malignancy. There are two-, three- and G four-stage systems for assessing malignancy.
- R-classification (based on the absence or presence of residual tumor after treatment),
- Classification according to the stage of the disease (stage Ia, stage Ib, stage IIat1, stage IIb, stage III, stage IVa, stage IVb).

Diagnosis of bone tumors:

Diagnosis of bone tumors, in addition to examination, history taking and standard blood tests, involves:
Clinical examination
Use of medical imaging techniques:
o magnetic resonance imaging,
o X-ray computed tomography,
o ultrasound examination,
o radiographic methods,
o radioisotope methods,
o positron emission tomography)
morphological diagnosis of the tumor with establishment of the tumor type
cytological examination strokes and smears-imprints

X-rays in mutually perpendicular projections help determine whether a tumor is benign or malignant.

Radiological signs of malignancy:
Tumor large sizes
Bone destruction
Tumor growth into soft fabrics.

X-ray signs of benignity:
Well-defined, small lesion
Thick rim of sclerosis around the tumor
No tumor growth into soft tissue.

Ancillary studies - CT, MRI and TCT scanning of the affected bone help determine the stage, extent of the tumor and its anatomical relationship with adjacent tissues.

If a malignant tumor is suspected, a chest CT scan is performed to exclude metastases in the lungs (the most common location of metastases in sarcomas).

Biopsy. If necessary, it is advisable to immediately perform a radical resection or amputation of the limb.

Treatment of Bone Tumors:

Surgery- the main method of treating bone tumors.

- Benign tumors. Intralesional or intracapsular excision of the tumor is performed. It is possible to replace the defect with a bone graft
- Malignant tumors. Resected within healthy tissue(at least 2 cm away from the visible border of the tumor) Metastases. Single (1-2) isolated pulmonary metastases of sarcoma (especially osteo- or chondrosarcoma) are subject to resection.

Adjuvant therapy for malignant tumors:
- Radiation therapy. Some tumors (eg Ewing's sarcoma) are very sensitive to radiation therapy. In other cases, radiation therapy is an auxiliary treatment method
- Chemotherapy. For osteosarcoma - doxorubicin (doxorubicin hydrochloride), cisplatin (intra-arterial or intravenous), methotrexate, thiophosphamide, cyclophosphamide (cyclophosphamide), dactinomycin, bleomycin). For Ewing's sarcoma - cyclophosphamide (cyclophosphamide), vincristine, actinomycin D (dactinomycin), adriamycin (doxorubicin hydrochloride).

Treatment of bone tumors may include surgery, chemotherapy and also radiation. The choice of treatment tactics depends on many reasons. In the case of well-differentiated tumors, it is often sufficient surgical intervention. As a rule, organ-preserving operations are performed. Only in some cases amputations are provided. For some types of tumors, combined or complex treatment using chemotherapy and radiation therapy, but usually involves surgical excision of the tumor as the primary method. For certain types of tumors (Ewing's sarcoma, for example) good effect gives radiation therapy.

Chemotherapy usually used in addition to surgical methods and may precede surgery. Due to the possible effects of chemotherapy and radiotherapy on the reproductive system, young men are usually offered the option of using a sperm bank.

Forecast. In recent years, the survival rate of patients with primary bone cancer has greatly improved, thanks to the emergence of new multidisciplinary approaches that include adjuvant and neoadjuvant chemotherapy and radiotherapy in addition to surgical interventions. In addition, thanks to the development of gentle methods, it is now possible to perform less radical operations than before. It is very important that patients with bone cancer are treated in specialized centers, where they can be prescribed the optimal combination of treatment methods according to their specific disease.

Prevention of bone tumors:

It is important to regularly conduct preventive examinations and timely seek professional and specialized advice. medical care when alarming symptoms. Medical observation and treatment of potentially precancerous diseases/conditions.

Which doctors should you contact if you have bone tumors:

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Other diseases from the group Oncological diseases:

Pituitary adenoma

Adenoma of the parathyroid (parathyroid) glands

Thyroid adenoma

Aldosteroma

Angioma of the pharynx

Angiosarcoma of the liver

Brain astrocytoma

Basal cell carcinoma (basal cell carcinoma)

Bowenoid papulosis of the penis

Bowen's disease

Paget's disease (nipple cancer)

Hodgkin's disease (lymphogranulomatosis, malignant granuloma)

Intracerebral tumors of the cerebral hemispheres

Hairy polyp of pharynx

Ganglioma (ganglioneuroma)

Ganglioneuroma

Hemangioblastoma

Hepatoblastoma

Germinoma

Giant Buschke-Levenshtein condyloma

Glioblastoma

Brain glioma

Optic nerve glioma

Chiasmal glioma

Glomus tumors (paragangliomas)

Hormonally inactive adrenal tumors (incidentalomas)

Mycosis fungoides

Benign tumors of the pharynx

Benign tumors of the optic nerve

Benign pleural tumors

Benign tumors of the oral cavity

Benign tumors of the tongue

Malignant neoplasms of the anterior mediastinum

Malignant neoplasms of the mucous membrane of the nasal cavity and paranasal sinuses

Malignant tumors of the pleura (pleural cancer)

Carcinoid syndrome

Mediastinal cysts

Cutaneous horn of the penis

Corticosteroma

Bone-forming malignant tumors

Bone marrow malignant tumors

Craniopharyngioma

Leukoplakia of the penis

Lymphoma

Burkitt's lymphoma

Thyroid lymphoma

Lymphosarcoma

Waldenström's macroglobulinemia

Medulloblastoma of the brain

Peritoneal mesothelioma

Mesothelioma malignant

Pericardial mesothelioma

Pleural mesothelioma

Melanoma

Conjunctival melanoma

Meningioma

Optic nerve meningioma

Multiple myeloma (plasmocytoma, multiple myeloma)

Pharyngeal neuroma

Acoustic neuroma

Neuroblastoma

Non-Hodgkin's lymphoma

Balanitis xerotica obliterans (lichen sclerosus)

Tumor-like lesions

Tumors

Tumors of the autonomic nervous system

Pituitary tumors

Frontal lobe tumors

Cerebellar tumors

Tumors of the cerebellum and fourth ventricle

Adrenal tumors

Tumors of the parathyroid glands

Pleural tumors

Spinal cord tumors

Brain stem tumors

Tumors of the central nervous system

Pineal tumors

Osteogenic sarcoma

Osteoid osteoma (osteoid-osteoma)

Osteoma

Osteochondroma

Genital warts of the penis

Pharynx papilloma

Oral papilloma

Paraganglioma of the middle ear

Pinealoma

Pineoblastoma

Squamous cell skin cancer

Prolactinoma

Anal cancer

Anal cancer (anal cancer)

Bronchial cancer

Thymus cancer (thymus cancer)

Vaginal cancer

Extrahepatic bile duct cancer

Cancer of the vulva (external genitalia)

Maxillary sinus cancer

Pharynx cancer

Brain cancer

Laryngeal cancer

Bone tumors develop when bone marrow cells begin to divide uncontrollably. They may be unchanged and pathological.

Differential diagnosis depends on factors such as:

• morphology of the bone lesion, as determined by conventional radiographs;
• tumor structure and boundaries (well or poorly defined);
• laboratory histological examination of tumor tissue, which makes it possible to identify the features of the neoplasm;
• establishing a more precise type of formation, depending on the specifics of the tumor and the results of previous data.

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Types and classification of bone tumors

There are the following main types of tumor masses of bone tissue:

• Benign:

The main feature is clear localization. Such formations are more common in people under 30 years of age, as well as in children. They are influenced by hormones that stimulate growth. Therefore, many tumors of this type stop growing when the patient reaches adulthood. Some benign formations can be locally aggressive and cause harm to the patient's health.

• Malignant:

Such formations are prone to metastasis and severe destruction of surrounding tissues. There are the following types of bone cancer:

1. Primary, initially arising in any place of bone or cartilage tissue.
2. A secondary formation involves the bone as a distant site for invasion by another type of malignant tumor.

Benign tumors of bone tissue

Osteochondroma:

Represents 35-40% of all benign formations. Consists of both bone and cartilage. Mostly develops in adolescents. Localized in the actively growing ends of long bones (femur, tibia, upper bone shoulder).

Chondroma:

Occurs in the tubular bones of the arms and legs, and can be either single or multiple.

Chondroblastoma:

A rare species, usually occurring in the epiphysis of long bones (hip, shoulder, knee). The formation is accompanied by joint pain and muscle atrophy. Treatment involves curettage of the bone plate.

Osteoblastoma:

Locally destructive progressive lesion occurring in the vertebrae. To exclude malignancy it is necessary.

Fibroma:

More often observed in children and adolescents.

Fibrous dysplasia:

This is a gene mutation that makes the fibrous structure of bone vulnerable to destruction.

Aneurysmal bone cyst:

A vascular abnormality that begins in the bone marrow. The disease is dangerous because it affects the growth of the plate.

Cartilaginous formation:

Occurs inside the bone marrow. It affects children and adults equally.

Osteoclastoma (giant cell tumor):

Quite aggressive formations that occur in adults at the rounded ends of the bone, rather than in the growth plate. Very rare.

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Malignant bone tumors

Multiple myeloma:

Multiple myeloma () is a bone marrow cancer that develops inside the bone. The onset and progression of the formation is usually multicentric. Imaging studies show limited damage or diffuse demineralization. Rarely, the tumor may manifest as sclerotic or diffuse osteopenia in the vertebral body.

Osteosarcoma:

One of the most common primary cancers in children aged 15-19 years. Osteosarcoma produces malignant osteoid from the bone tumor. Occurs in the metaphyses of long bones. Common locations are around the knee (75%) or proximal humerus. Prone to metastasize to the lungs.

Fibrosarcoma:

It has similar characteristics to osteosarcoma but occurs in fibrous tissues of tumor cells.

Malignant fibrous histiocytoma:

Differs in histological characteristics. Typically occurs in children and adolescents, but can also occur in older adults as a secondary lesion.

Chondrosarcoma:

Represents the oncological formation of cartilage. In 90% of cases, these are primary tumors that arise in the presence of benign education. They often develop in flat tissues (eg, pelvis, scapula), but can also implant in surrounding soft tissues. affects older people.

Ewing's sarcoma of bone:

Neuroectodermal bone tumor, arising from mesenchymal stem cells. Affects young people under 25 years of age. Tends to be expansive. Typically presents with masses and swelling in the long bones of the arms and legs, pelvis, chest, as well as in the skull and flat bones torso.

Bone lymphoma:

The tumor consists of round small cells, often with a mixture of lymphoblasts and lymphocytes. Imaging studies show patchy or penetrating bone destruction. At a later stage, the tumor can completely replace the bone.

Malignant giant cell tumor:

Typically located at the very end of a long bone. Mostly, it develops from previously benign formations.

Chordoma:

It occurs extremely rarely and develops from the remains of a primitive notochord. Tends to form at the end of the spine, usually in the middle of the sacrum or near the base of the skull.

Spindle cell sarcoma:

This is a mixed group of malignant bone tumors that includes fibrosarcoma, malignant fibrous histiocytoma, leiomyosarcoma and undifferentiated sarcoma.

Treatment tactics

Therapeutic modalities depend on type, location and advancedness malignant process. Treatment options are:

1. Surgery – the most adequate way to combat a malignant tumor of bone tissue and the only possible method for the treatment of benign lesions. The specialist removes cancerous tumor and part of the healthy area around the outbreak.
2. Chemotherapy – envisions use to destroy abnormal cells. Currently, the method is not applied to chondrosarcoma.
3. Radiation treatment: Primarily complements surgical treatment or is the primary therapeutic option for patients who cannot or refuse resection.
4. ‒ modern technology, in some cases allowing to replace conventional surgery.

Bone tumor prognosis

Survival depends on educational characteristics. The result of treatment of benign formations is positive. Forecast for malignant forms tumors depends on the class, stage of spread of the process and the treatment used:

1. In the initial stages of the disease, treatment is quite successful. Almost 60 out of 100 people will live at least 5 years after the diagnosis is confirmed.
2. Treatment with help increases survival rate from 20% to 65%.
3. With complete necrosis of the abnormal tissue, the 5-year prognosis indicates a 90% survival rate.
4. With metastatic cancer, survival rate decreases to 25%.

Bone tumors quite common cancers. However, timely diagnosis also contributes to the destruction of accumulated masses and a positive result in general.