fibrosarcoma of bone
Introduction
Introduction to osteofibrosarcoma Osteofibrous sarcoma is a fibroid malignancy. Those who originate in the connective tissue of the bone marrow cavity, called fibrosarcoma of the central bone, are more common. Fibrous tissue originating from the periosteum, a fibrosarcoma called peripheral bone, is less common. Secondary osteofibrosarcoma is often secondary to the original bone disease. Such as malformation osteitis, osteofibrosis, aneurysmal bone cyst, chronic osteomyelitis, recurrent bone giant cell tumor. In imaging, clinical, and histological features, osteofibrosarcoma is similar to other bone tumors, and it is often difficult for an experienced pathologist to identify the disease. Surgical resection is the main measure of local control. basic knowledge The proportion of illness: 0.003% Susceptible people: no specific population Mode of infection: non-infectious Complications: fracture
Cause
Osteofibrosarcoma etiology
(1) Causes of the disease
The cause is unknown.
(two) pathogenesis
1. Gross examination of osteofibrosarcoma is a destructive, invasive lesion with a size of 1.5-20 cm. It is related to the size and differentiation of the tumor by the naked eye. The differentiated tumor is more collagen-rich than the poorly differentiated tumor. The content can be white or gray-white solid rubber, poorly differentiated fibrosarcoma is soft, the content is fish-like and mucus lesions, most fibrosarcoma tumors are uniform, but very large tumors can have In the area of hemorrhage and necrosis, there may be a pseudo-envelope that separates it from the diseased bone.
2. Microscopy Histological examination can classify fibrosarcoma into: well differentiated, moderately differentiated, poorly differentiated tumors, or divide them into grades I to IV. Higher grades indicate poorer differentiation, tumor grade, classification The criteria are objective. They are comprehensively evaluated by cell number, mitotic activity, collagen production, nuclear morphology and all tissue types. Most fibrosarcoma are moderate or poorly differentiated, and patients with high malignancy have a survival rate. low.
The well-differentiated fibrosarcoma is composed of rectangular and fusiform cells. The nucleus is slender and fat, the staining is light, the cell morphology and size are consistent, and they are arranged in a bundle, sometimes in a spiral shape, and the cells are loosely distributed and rich in cytoplasm. There are more collagen fibers in the interstitial. Nuclear fission is only seen occasionally. In poorly differentiated osteofibrosarcoma, the number of cells increases, the cells are arranged closely, and the corresponding collagen content decreases. The nucleus is round or oval. Irregular, chromatin is coarse, clustered and distributed, common nucleoli are more obvious, mitotic activity increases, and necrosis and hemorrhage often occur in poorly differentiated fibrosarcoma.
Prevention
Osteofibrosarcoma prevention
There is no effective preventive measure for this disease. Early detection and early diagnosis are the key to the prevention and treatment of this disease.
Complication
Osteofibrosarcoma complications Complications
And more patients with rational fractures.
Symptom
Osteofibrosarcoma Symptoms Common Symptoms Bone pain grows slowly in the wrist and blood...
The incidence of osteofibrosarcoma is lower than that of osteosarcoma and chondrosarcoma. The incidence rate of men and women is the same. It is common in adults. Most of them are between 30 and 60 years old. Most patients have slow onset. The main symptoms are pain. The degree of pain is not as good as flesh. The tumor was heavy, and there were swelling, joint activity limitation, mass and other symptoms, and there were more cases of rational fractures. About 33% of the patients were diagnosed with fractures for the first time.
Predilection site: osteofibrosarcoma in the skeletal site, similar to osteosarcoma and malignant fibrous histiocytoma, about 70% of cases invaded the long tubular bone, the lesions were followed by: femur (40%), tibia (16 %), tibia (10%), tibia (3%), tibia (1%), ulna (0.5%), bone fibrosarcoma around the knee is counted as 33% to 80%, small bones in the hands and feet are rare, pelvic bone The incidence is about 9%. If the skull has no previous Paget disease or has not been treated with radiation, the incidence is extremely rare.
In the long tubular bone, the metaphyseal end is a predilection site, and it is common for the metaphyseal tumor to extend to the ankle. Although the osteofibrosarcoma is a single intramedullary tumor, there are also multiple or focal lesions located in the periosteum.
Examine
Examination of osteofibrosarcoma
X-ray examination: X-ray features of osteofibrosarcoma are: 1 ground pattern, worm-type, piercing osteolytic bone destruction, generally the conversion bandwidth from normal bone to diseased bone, 2 rarely bone Hardening and periosteal reaction, if there is periosteal reaction, can be lamellar, radial and Codman triangle, and can have cortical bone destruction and soft tissue mass.
In the tubular osteofibrosarcoma can be located in the center or eccentric, in the tubular bone fibrosarcoma, if the eccentric position, the lesion is map-like destruction, and only invade part of the cortical bone, the prognosis is better than those without these characteristics.
In fact, X-ray abnormalities of osteofibrosarcoma are not particularly specific, and generally give the impression of an invasive or malignant lesion. Because there is no calcification and ossification in the lesion, it can be combined with osteosarcoma and chondrosarcoma. Identification, but fibrosarcoma, such as malignant fibrous histiocytoma, telangiectasia, sarcoma, lymphoma, plasma cell myeloma and bone metastasis, have certain difficulties, and sometimes other imaging methods can provide more information. But there is no special diagnostic significance.
Diagnosis
Diagnosis and diagnosis of osteofibrosarcoma
According to the medical history, predilection site, clinical manifestations, X-ray examination, the disease should be considered, and the final diagnosis will rely on biopsy and pathological examination results.
The following tumor types should be considered when histologically identifying spindle cell sarcoma.
1. The primary malignant fibrous histiocytoma of the bone is mainly composed of neoplastic fibroblasts, but the malignant fibrous histiocytoma is characterized by the presence of neoplastic fibroblasts and tumorous histiocytes. The composition is significantly different and the differentiation is worse.
2. Primary leiomyosarcoma (LMS) and rhabdomyosarcoma (RMS) These two tumors are more common in soft tissues. The primary bone is extremely rare, and its shape is fusiform, but LMS cells are short-fertilized and the nucleus is more ovoid. Both ends are flush like cigars, Masson trichrome stains myoplasma red stain, while fibrosarcoma collagen fibers are green stainable, Mallory PTAH special dye, smooth muscle (-), striated muscle visible blue-black horizontal stripes, with electron microscopy and immunity The grouping method can further help identify.
3. Fibrosarcoma with better differentiation of osteofibrofibromas sometimes needs to be differentiated from osteofibrofibromas, but the former can still find heteromorphic cells and mitotic phases, the latter being benign tumors, although clinically showing local invasive organisms Behavior, but histologically benign.
