Multiple hereditary osteochondromas

Introduction to multiple hereditary osteochondroma The incidence of multiple osteochondromatosis or multiple exostosis is smaller than that of isolated osteochondroma. It is a skeletal dysplasia, and bone bulges of varying sizes can be formed on the bone. Multiple hereditary osteochondroma has three characteristics: 1 has a hereditary 2 bone shortening or deformity 3 evil into a high incidence of peripheral chondrosarcoma, multiple osteochondroma disease or multiple exostosis Isolated osteochondroma is small. It is a kind of skeletal dysplasia, which can form bone ridges of different sizes on the bone. It is an autosomal dominant hereditary disease, and most patients have a family history [3]. The name of this disease is many, sometimes called hereditary malformation of cartilage dysplasia, or continuation of the backbone. The latter mainly refers to the abnormal shape of the entire affected bone. In severe cases, the bones of all cartilage internalized bones have different degrees of abnormalities. The most common site is the longest tubular bone adjacent to the knee and ankle, which is bilateral and symmetrical. basic knowledge The proportion of illness: 0.003% Susceptible people: no specific people Mode of infection: non-infectious Complications: hypercalcemia

Multiple hereditary osteochondroma causes

The cause of the disease is not fully understood.

(1) Due to congenital plaque defects.

(2) Due to the misplacement of the epiphyseal plate.

(3) The residual immature cells or metaplastic chondrocytes from the inner layer of the periosteum gradually grow to form osteophytes.

(4) due to incomplete periosteal growth, can not restrain the proliferation of cartilage, causing abnormal cartilage formation.

(5) Due to the ability of the bones to lose co-formation during the long process, the cognac is widened and continuously proliferated to form osteophytes. In recent years, it has been suggested that this disease is related to the metabolic disorder of acid mucopolysaccharides, Solomon [9] It was found that the amount of acid mucopolysaccharide excreted in the urine of children with multiple osteochondroma disease increased, but the excretion of adult patients was normal. He believed that the increase in excretion of children was due to the large amount of cartilage in the body. With the increase of age, the osteochondroma is mature, the cartilage component is reduced, and the excretion of acid mucopolysaccharide is bound to decrease.

Multiple hereditary osteochondroma prevention

Some adjustments can be made in the diet:

1. Maintain an ideal weight.

2. Ingest a variety of foods.

3. The diet includes a variety of vegetables and fruits.

4. Ingest more high-fiber foods (such as whole grain cereals, beans, vegetables, and fruits).

5. Reduce total fat intake.

6. Limit the intake of alcoholic beverages.

7. Limit the intake of marinated, smoked, and nitrite-containing foods.

Multiple hereditary osteochondroma complications Complications hypercalcemia

Chondromatosis is often accompanied by hypercalcemia.

Multiple hereditary osteochondroma symptoms Common symptoms Chondroital dysplasia and... Endochondral ossified myalgia

More common in men, there is a significant family history of genetic disease, all of the bones of cartilage internalization can occur, of which the common metaphyseal end of the long bones of the extremities is most common, often symmetry occurs, the lower extremity is more than the upper limbs, the lower limbs are knees The bones around the joints are the most, and the early tumors are often asymptomatic. Generally, in children and adolescence, the masses are enlarged due to osteophytes, deformed, and the nearby soft tissues are compressed. When the pain is noticed, the tumors can affect the joint function and oppress the gods. When the diameter (especially the common peroneal nerve) can cause the corresponding symptoms, multiple osteochondromatosis can be found in early childhood. When adolescents, the forearm can have a bow deformity, the hand is skewed to the ulnar side, and sometimes the pelvis and Thoracic deformation.

Examination of multiple hereditary osteochondroma

X-ray findings are similar to solitary osteochondroma, with osteochondromas of different sizes on many bones, bones near the knee, such as the lower end of the femur, the lower end of the humerus, and the upper end of the humerus with osteochondromas of different sizes and even knees. The upper and lower joints can be interlocked due to the growth of osteochondroma, even similar to bone fusion. In addition to the long tubular bone, the spine of the scapula is also a common predilection, followed by the metacarpal, humerus, phalanx, phalanges, ribs, spine. And the pelvis, the humerus at the junction with the cartilage, there may be countless small osteochondromas, the skull has never had such a lesion, after the malignant transformation, ECT showed a malignant metabolically active site.

Generally seen in the longitudinal section of the affected bone osteochondroma is not covered in the cortex, but an irregular bag-like cortical bone, this bag-like cortical bone is pure bone tissue, under the cortex is cancellous bone, in the bone There is a layer of cartilage on the outside of the cortex. Osteochondroma usually has no obvious symptoms. It is only a local mass formation. Sometimes the mass compresses the surrounding tissue, causing pain and discomfort. If the volume of the mass increases rapidly and the cartilage cap is thickened to more than 1cm, malignant transformation should be considered. Possibly, this is more common in multiple patients than in single-shot (20% more, 1% [10] single), Ochsner [11] reported an average age of 31 years for malignant transformation of multiple osteochondroma chondrosarcoma. It mainly occurs around the pelvic girdle.

Microscopic examination showed that the cartilage is hyaline cartilage. If the cartilage grows actively, the chondrocytes are arranged in a columnar shape. The periosteum covering the surface of the osteochondroma is connected with the periosteum of the normal bone. In these periosteal growth layers, focal cartilage metaplasia can be seen. Under the osteochondroma, there are occasionally different granulated calcified substances. The histological examination is mainly calcification or most necrotic cartilage, calcified cartilage matrix and calcification debris, which indicates that the normal process of cartilage internalization occurs. The localized disorder causes the calcified cartilage matrix that should be destroyed and replaced by bone to accumulate. This phenomenon cannot be regarded as the presence of endogenous chondroma. These sand-like calcifications do not originate in the bone but originate from the proliferation of the surface. Cartilage.

Diagnosis and diagnosis of multiple hereditary osteochondroma

Diagnosis can be based on medical history, clinical symptoms, and laboratory tests .

Differential diagnosis

Should be differentiated from chondrosarcoma, fibrosarcoma:

The development of chondrosarcoma on x-ray photographs is quite inconsistent. Sometimes the metaphysis of the long bone is destroyed by the tumor tissue and it is a transparent pseudocyst. The capsule is scattered with a little calcification and ossification. Sometimes the tumor tissue produces a large amount of cotton-like The calcified block obscures the damaged bone defect and forms a dense calcification shadow. The malignant transformation of osteochondroma begins in the cartilage cap of the tumor and its fibrous envelope (see osteochondroma), chondrocyte division and cartilage calcification suddenly Increased, so that a large number of irregular calcifications such as cotton-like plaques are found on the x-ray photographs, scattered in the soft tissue mass around the distal end of the osteochondroma, and the radiation-like periosteal reactive new bone hyperplasia is rare, sometimes found Periosteal triangle, when the chondromatosis is malignant, the osteolytic shadow in the bone increases, the calcification point increases, and the calcification points increase, and the tumor tissue sometimes penetrates the cortical bone, develops in the soft tissue and forms a calcification shadow, and sunlight may occur outside the cortical bone. Radiation-like periosteal new bone reaction.

Fibrous sarcomas show calcification in individual cases, lesions may erode nearby bones, and angiography, CT and MRI examinations show similar features to most other types of sarcomas.

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