primary lymphoma of the bone
Basic overof the primary lymphoma of bone marrow cells that originated in the round cell sarcoma, the histological features, clinical features, course and prognosis of Ewing''s sarcoma was significantly different, but the boundaries between the two is not always obvious , primary lymphoma of bone and systemic lymphoma is even more obvious difference between the. Primary lymphoma of bone is not as systemic lymphoma as a morphological and immunological classification. Treatment of primary lymphoma of bone sensitive to radiotherapy and radiotherapy for primary lesions, metastases, multi-center bone lesions, radiation dose of 50 ~ 80Gy (5000 ~ 8000rad) between the illuminated area on the image to be compared wide, often including the entire risk of bone. Must be combined with chemotherapy, radiotherapy, chemotherapy for a variety of chemotherapy drugs combined with chemotherapy, long-term chemotherapy, periodic applications. Primary lymphoma of bone lesions such as spinal nerve root compression, can be decompression laminectomy and biopsy at the same time, and then radiotherapy; also possible needle biopsy, you can then radiotherapy. Such as the long bones were severely damaged, and associated with pathological fractures or impending pathologic fracture, viable tumor resection, functional reconstruction is available or artificial joint prosthesis can also be combined with bone cement to bone surgery, radiotherapy and chemotherapy after surgery should be performed . For the sca
pula, ribs, pelvis anterior arch of the disease, tumor resection can be used, in parallel with radiation and chemotherapy. Amputation should be strictly limited to a few cases, the scope of its application to large lesions, not suitable for the line limb surgery and radiotherapy are reserved; radiotherapy has not inhibit tumor development in persons; extensive local recurrence after radiotherapy. Pathogenesis 1. Naked eye tumor tissue was gray, soft texture, liquid and more like for the marrow, it is difficult and malignant lymphoma, Ewing''s sarcoma and identification of any rich cell sarcoma. With Ewing''s sarcoma is similar to primary lymphoma of bone cortical bone to the abuse or the spin the bone can be quite far away from the primary lesion of bone found in multiple tumor nodules and / or extensive tumor infiltration. As with Ewing''s sarcoma, primary lymphoma of bone within the tumor tissue often hemorrhage, necrosis and liquefaction zones, surgical exploration can be mistaken for osteomyelitis of the liquefied area. Sometimes regional lymph nodes can be enlarged, the quality tough and flexible, occasionally confluent, in section, the lymph node medulla may be part or all of the white medullary swelling replaced by tumor tissue. 2. Endoscopic findings are rich in tumor cells, less pale cytoplasm, some of the cytoplasm of some rich, clear boundaries, cytoplasmic processes can be combined with each other to form fine cell network. Ewing''s sarcoma cell nucleus is usually large compared with the size ranging polymorphism significantly, the shape can be round or oval, can also have a notch or pear-shaped or lobulated. Some cells may have a larger core or dual-core, but no giant cells. Finely granular chromatin can also block for larger groups. Nucleus often hollow, may have one or more nucleoli volume can be great. Split-phase common. Often in the middle of the reticular cells and lymphocytes lymphoblasts. Seen in tumors with a thickness ranging from collagen, the tumor is divided into irregular lobules. Silver staining often but not always able to show a thick mesh of fibrous tissue, the fibrous tissue surrounding the small network of cells, the base to the surrounding single cells. PAS were negative and found no glycogen within cells. Immunohistochemistry (not decalcified specimen) to show cell markers were positive. Better in the differentiation of primary lymphoma of bone, the cells are smaller, the performance of the lymphocyte samples, compared with Ewing''s sarcoma, the nucleus small, dark in color, cytoplasmic boundaries more clearly. Clinical manifestations of malignant lymphoma is a group of systemic lymphoid tissue of the tumor, two of which often have bone cancer lesions, a total of Hodgkin''s disease and non-Hodgkin''s disease. Systemic lymphoma may be generated through the three channels of bone lesions: the adjacent lymph node involvement of bone, which could explain the disease more common in the spine, pelvis, ribs and sternum reasons; derived blood-borne and lymphatic metastasis; occurrence of bone marrow itself. First symptom of Hodgkin''s disease for bone, and lymph node lesions may not be significant, or even hidden, but it will eventually develop significant lesions, bone lesions are part of systemic Hodgkin''s disease. Non-Hodgkin''s disease of bone lesions can be formed, sooner or later may have systemic spof lymph nodes and internal organs, but can also be indefinitely confined to the skeletal system. Therefore lymphoma can be divided into two types, one type involving the lymph nodes, internal organs, often involving the skeletal system, but the bone lesion is part of systemic malignant lymphoma; another type involving only the skeletal system, bone lesions are not systemic lymphoid part of tumor, primary bone tumors. Histological examination can not distinguish between these two lymphoma, its clinical manifestations may be different for the two types of lymphoma during or intermediate state, therefore, must be considered of primary lymphoma of the possibility of systemic malignant lymphoma. It should be noted that only a few years later followed up in order to obtain primary bone lymphoma and non-systemic diagnosis, in the performance of primary lymphoma of bone cases, required detailed examination of the body lymph nodes, neuropathy bones, internal organs, bone marrow and peripheral blood. Primary lymphoma of bone than the low incidence of Ewing''s sarcoma more. Occurs in men. Compared with Ewing''s sarcoma, primary lymphoma of bone in adults or the elderly, most cases after the age at onset of 25 to 30, rarely before the age of onset at 20, 15, were rare before the age of onset. Primary lymphoma of bone, compared with Ewing''s sarcoma, primary lymphoma of bone occurs in the trunk better bone and craniofacial bone, accounting for 50% of all cases, the remaining 50% can be found in long bones, usually femur, tibia and humerus. In the long bones, metaphyseal or backbone occurred in each half of the lesions, because lymphoma often occurs after the disappearance of the growth cartilage, the epiphyseal end of violations of a lower incidence of Ewing''s sarcoma of the high. Primary bone lymphoma can involve two or more adjacent or distant bone, which is not uncommon. Such as lymphoma in the spinal epidural space, either imaging or intraoperative findings laminectomy, are difficult or impossible to confirm the origin of the bone. Compared with Ewing''s sarcoma, primary lymphoma of bone most notable feature is the general good of patients, at least in the longer period of time to maintain this, its no fever, anemia, weight loss, increased ESR and WBC increased. The main symptoms are pain in the long time performance of intermittent mild pain, other symptoms may appear localized swelling and pathological fracture, such as the spinal nerve root compression, there may be pain and neurological dysfunction radioactive. Compared with Ewing''s sarcoma, the incidence of regional lymph nodes lower. When suspected or diagnosed as primary lymphoma of bone, should be careful to check all of the bone (bone scan), lymph nodes, liver, spleen, bone marrow and peripheral blood smears. X ray findings auxiliary examination of primary lymphoma of bone imaging findings and imaging features of Ewing''s sarcoma was no significant difference, not the typical image performance. The difference with Ewing''s sarcoma is its slow growth, usually adult-onset, reactive periosteal bone less. Primary lymphoma of bone imaging showed osteolytic lesions based, usually for the point of osteolytic, fuzzy boundaries, worm-eaten bone destruction usually presents like. In rare cases, the osteolytic osteolysis can be integrated into a uniform image. Next to the osteolytic areas may have increased bone mineral density, bone formation for the reaction, the diffuse infiltration of tumor tissue was not being re-absorbed. Small number of cases the image may be mainly for high-density bone sclerosis. Cortical bone is usually broken, but sometimes may remain relatively intact. In the advanced tumor may spto the soft tissues, the formation of lumps through the X line, fuzzy boundaries, may have some reactions of bone of the stripes. No or very few outside the periosteal reactive bone, such as young patients, and the tumor is located in the backbone, outside the periosteal bone formation may be active, but few can produce onion skin-like images. Pathology of primary lymphoma of bone fractures common in the anatomical extent of disease than is usually seen on the image much larger extent of disease, and similar to Ewing''s sarcoma, in this sense, the bone scan and MRI is more accurate . Primary lymphoma of the bone and the lack of symptoms of slow progression, a number of cases of disease at diagnosis has existed a long time, the image on the extent of disease can also be great. Sometimes, the tumor in the image, which half the violations of the bone, or even the entire backbone. Lymphangiography can check whether the involvement of regional lymph nodes, bone scan than other bone lesions, but also for liver and spleen scintigraphy. Differential diagnosis of primary lymphoma of bone in some clinical and imaging findings may be similar to certain non-neoplastic diseases, such as osteomyelitis, eosinophilic granuloma, but in most cases, the imaging performance is neoplastic change but specific to one example, may be associated with fibrosarcoma, malignant fibrous histiocytoma, osteosarcoma, Ewing sarcoma, or confused. Small number of cases seen in patients tumor tissue was wrapped in a clear liquid, may be misdiagnosed as osteomyelitis. Diagnosis relies on pathology diagnosis, but sometimes difficult to determine the pathological diagnosis. If you have the histological features of tumor tissue from the lymph nodes, can be sure diagnosis, up to and confused with Hodgkin''s sarcoma, but subjects from the bone tissue when it is difficult to identify with Ewing''s sarcoma. Finally, in individual cases, primary lymphoma of bone to be undifferentiated epithelial carcinoma with bone metastasis, cell proliferation disorders of bone lesions in X phase identification, the reason is of primary lymphoma of bone lesions of the edge inflammatory reaction. Prognosis of primary lymphoma of bone disease variable, slow and insidious, even 10 years after treatment is also difficult to judge the prognosis. 5-year survival rate of primary lymphoma of the bone without much sense, even before the application of chemotherapy, the 5-year survival rate can reach 40% to 50%, but its 10-year survival rate of only about 30%, the United with chemotherapy, its 10-year survival rate has increased to 60% to 80%, the prognosis is better than Ewing''s sarcoma. Tip indicates negative factor is the range of bone lesions, located in the trunk or pelvis bone. Sometimes, primary lymphoma of bone relapse in the radiation zone. Small number of cases several years after the radiation therapy may be on the same site radioactivity sarcoma.