Primary malignant tumor of the spleen
Introduction
Introduction to spleen primary malignant tumor Primary spleen malignant tumors are clinically less common than spleen benign tumors. Krumbhar's primary spleen malignant tumors account for only 0.64% of all malignant tumors. The primary malignant tumors of the spleen are sarcomas, such as lymphomas, angiosarcomas, etc. According to the different origin tissues, the domestic literature currently divides them into three major categories, namely spleen primary malignant lymphoma, spleen angiosarcoma and spleen. Malignant fibrous histiocytoma. basic knowledge The proportion of illness: 0.011% Susceptible people: no special people Mode of infection: non-infectious Complications: intestinal obstruction, urinary tract obstruction
Cause
The cause of primary malignant tumor of the spleen
(1) Causes of the disease
The cause of spleen tumors has not yet been fully elucidated, but nearly 30 years of research have found some possible factors related to the occurrence of spleen tumors, such as infectious factors (some viruses, mycobacteria, malaria parasites, etc.), genetic factors and other chronic diseases of the spleen Et al., studying a group of cases, that 57% of spleen lymphomas are associated with infection, especially with the prevalence of mycobacteria, that is, their B-ultrasound performance is partially nodular, and the other part is atypical. Wakasugi reports a case of outbreak of B-cell lymphoma in patients with chronic hepatitis C virus infection; Ozaki et al also confirmed that hepatitis B virus infection is associated with spleen T/ T cell lymphoma; Kraus reports a case of lymph node infection in EB virus infection in heart transplant patients T/ T cell lymphoma occurs after abnormal hyperplasia; Bates et al reported that there are many clinical and immunological commonalities in spleen lymphoma with villusous lymphocytes in West Africa and highly reactive malaria splenomegaly, which is the onset of lymphoma The study of the mechanism provides clues. After the analysis of these documents, the spleen is infected by pathogens such as viruses and bacteria. The heterosexual immune response stimulates the accumulation and proliferation of B lymphocytes or T lymphocytes in the inflammatory zone of the spleen. In the case of some imbalances in the body, this proliferation may become unrestricted and develop into a tumor. In addition, genetic factors and some chronic diseases of the spleen may also have a certain relationship with the onset of spleen tumors.
(two) pathogenesis
According to the different tissues of origin, spleen malignant tumors are divided into three categories:
1. Spleen Primary malignant lymphoma The spleen is the human body and the largest lymphoid organ. More than half of the malignant lymphomas originating in other parts involve the spleen, while the malignant lymphoma that is actually primary in the spleen is relatively rare. The rate is less than 1% of the total number of malignant lymphomas. However, the primary malignant lymphoma of the spleen is still the highest in the primary malignant tumor of the spleen, accounting for more than 2/3 of the spleen malignant tumors. The primary malignant lymphoma of the spleen Refers to a malignant tumor originating from the lymphoid tissue of the spleen. The diagnosis of lymph nodes and other organs should be excluded at the time of diagnosis. Malignant lymphoma derived from lymph nodes and other organs involving the spleen does not fall into this category. The staging of spleen malignant lymphoma is generally Abmann. Method: Stage I means that the tumor tissue is completely confined to the spleen; Stage II refers to the lymph node that has affected the splenic portal; Stage III refers to the liver or distant lymph nodes.
2. Spleen angiosarcoma spleen malignant lymphoma, spleen often increases, and the degree of increase is related to the course of the disease, spleen weight ranging from 100 to 4500g, an average of 160g, the gross specimen can be divided into three types:
1 diffuse and enlarged, the spleen is evenly enlarged, no nodules are visible to the naked eye;
2 miliary nodular type, splenomegaly, the cut surface is scattered with 1 ~ 5mm size gray-white nodules, like spleen miliary tuberculosis;
3 nodular type, the spleen is significantly enlarged, the nodule is 2~10cm in size, and some adjacent nodules can be fused into a huge mass. Under the microscope, it is easy to ignore the early stage of small cell lymphoma under low magnification. The lesion, but carefully observed, found that almost every center of Malpighi is composed of diagnostic angular cells, and the pathological type of primary lymphoma of the spleen is basically the same as that of lymphoma from other sites. Hodgkin's lymphoma can find special large cells (Reed-Sternbergscell) and its variants in the lesions. Because the changes in tissue structure and cell ratio are difficult to determine, subtype diagnosis is generally not done, non-Hodgkin's lymphoma. There are B cell type and T cell type, there is no significant difference in pathological histomorphology, mainly relying on immunohistochemical differentiation. The clinical significance of pathological typing of non-Hodgkin's lymphoma is to choose the appropriate chemotherapy. Careful and careful identification of the difference between spleen reactive hyperplasia and malignant lymphoma in the examination, because the former is often misdiagnosed as malignant lymphoma, carefully study the spleen Cellular components nodules help confirm the diagnosis.
Splenic angiosarcoma is rare, and it can hardly be diagnosed before surgery. There are no characteristic symptoms and signs in the clinic. If the lesion is located on the upper pole of the spleen, it is less noticeable and detectable. Like other spleen malignant tumors, its main clinical manifestation is spleen swelling. Large, pain in the left upper abdomen, sometimes with fever, weight loss.
Splenic angiosarcoma is a malignant tumor that occurs from the spleen sinus endothelial cells. Some scholars call it spleen vascular endothelial cell tumor, but a few scholars believe that the two are two different tumors. Even so, these scholars agree with spleen angiosarcoma. The tissue characteristics and biological behavior of spleen vascular endothelial cell tumor are very similar. Extramedullary hematopoiesis in tumor tissue is characteristic of spleen angiosarcoma.
The spleen angiosarcoma is mostly characterized by giant spleen, weighing 420-5300g, with an average of 1500g. Most or part of the spleen tissue is destroyed by the tumor tissue, and there are multiple hemorrhagic sarcoma nodules of different sizes. The tumor is grayish white and fine. Microscopically, the malignant endothelial cells with high atypicality expand along the spleen and sinus. The tumor cells divide like many, often forming flower buds. Multinucleated giant cells protrude into the cystic dilated sinus cavity, and multiple hemorrhagic diseases of different sizes appear. Tumor nodules, electron microscopy showed that Weiber-Palade bodies were visible in the cytoplasm of the tumor.
3. Malignant fibrous histiocytoma (MFH) or fibroblastic sarcoma , tissue cells and malformed giant cells, malignant tumors that occur in the spleen capsule or spleen trabecular fibrous tissue, called spleen primary malignant fibrous histiocytoma. In recent years, scholars have noted that FHS is an independent type of malignancy. Tumors, including the etiology, are still to be studied.
Malignant fibrous histiocytoma consists of neoplastic tissue cells and fibroblasts, which may be derived from undifferentiated mesenchymal cells, which in different cases differentiate into different directions such as tissue cells, fibroblasts, and myofibroblasts, exhibiting complex tissues. The morphology can be divided into six histological types: polymorphic cell type, car-like type, bundle-shaped type, inflammatory fiber type, hemangioma-like type and mucinous type. The spleen enlargement is lobulated and hard. The cut surface is different in gray, the center is necrotic or cystic, and there may be ossification.
Prevention
Spleen primary malignant tumor prevention
1. Develop good habits, stop smoking and limit alcohol. Smoking, the World Health Organization predicts that if people no longer smoke, after five years, the world's cancer will be reduced by 1/3; secondly, no alcohol. Smoke and alcohol are extremely acidic and acidic substances. People who smoke and drink for a long time can easily lead to acidic body.
2. Don't eat too much salty and spicy food, don't eat food that is overheated, too cold, expired and deteriorated; those who are frail or have certain genetic diseases should eat some anti-cancer foods and high alkali content as appropriate. Alkaline foods maintain a good mental state.
Complication
Spleen primary malignant tumor complications Complications, intestinal obstruction, urinary tract obstruction
The swollen spleen can cause compression symptoms around the dirty mouth. For example, compression of the intestine can cause intestinal obstruction. Compression of the left ureter can cause upper urinary tract obstruction. In some cases, spontaneous rupture of the cancer is caused by intra-abdominal hemorrhage. symptom.
Symptom
Symptoms of primary malignant tumor of the spleen Common symptoms Chronic facial bloating, liver enlargement, low fever, high heat, fatigue, toxicity, loss of appetite, loss of appetite
Primary spleen malignant tumors often have no special symptoms in the early stage, and patients often present advanced cancer status at the time of treatment.
1. The performance of the spleen itself: The swollen spleen is mostly below the umbilical level. It has been reported in the literature that the maximum is 7.5cm below the umbilicus, which is progressively enlarged, hard, uneven surface, poor mobility and obvious tenderness.
2. Local compression symptoms caused by the mass: such as stomach area fullness, loss of appetite, abdominal distension, palpitations and shortness of breath.
3. Toxicity of malignant tumors: such as low fever, fatigue, anemia, weight loss, etc., some cases can show high fever, nearly one-four cases may be accompanied by liver enlargement, and the spleen is irregularly swollen, no long-term fever, no spleen Hyperfunction, etc., is a characteristic of primary malignant tumors of the spleen.
Examine
Examination of primary malignant tumor of the spleen
Patients with hyperactivity may have peripheral blood leukocytes and thrombocytopenia as well as hemolytic anemia.
Imaging examination plays an important role in the diagnosis of spleen tumors. X-ray examination can find signs of spleen enlargement and local compression, but it is not specific. B-ultrasound can determine whether the spleen has a mass, which is parenchymal or cystic, but not Differentiated between benign and malignant, percutaneous biopsy, the risk is high, and the puncture site is difficult to determine. CT and magnetic resonance not only show the lesion of the spleen itself, but also show the relationship between the mass and the adjacent organs, lymph node or liver invasion and abdominal cavity and Other lesions in the thoracic cavity, selective splenic angiography can show signs of spleen parenchymal defects.
Diagnosis
Diagnosis and differential diagnosis of primary malignant tumor of spleen
Diagnostic criteria
According to the medical history, irregular swelling of the spleen, unexplained fever, no swelling of the superficial lymph nodes, and laboratory and imaging examinations, it is generally possible to diagnose malignant tumors of the spleen; There is a splenic lymphoma with villous lymphocyte (SLVL) in the blood circulation, which should be differentiated from chronic lymphocytic leukemia and hairy cell leukemia. The diagnosis of SLVL mainly depends on the morphology and immunophenotyping of circulating villous lymphocytes. We believe that the diagnosis of spleen malignant tumors still requires pathological confirmation.
Differential diagnosis
In view of the fact that early signs of malignant tumors are not obvious, and even some advanced cases have no specific manifestations, differential diagnosis is more important and often needs to be distinguished from the following diseases:
1. Systemic diseases accompanied by splenomegaly: such as congestive splenomegaly caused by portal hypertension, malignant lymphoma and chronic leukemia invading the spleen.
2. Benign diseases of the spleen itself: such as spleen abscess, spleen tuberculosis, spleen cyst and other benign tumors of the spleen.
3. Diseases of adjacent organs of the spleen: such as retroperitoneal tumors, kidney tumors, pancreatic tumors, and the like.
These diseases are often identified by means of medical history, physical examination, laboratory examination and imaging diagnosis, and lymph node biopsy.
