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Causes of Wilms' Tumor
Causes of Wilms' Tumor Disease
In the occurrence of this disease, it may be related to developmental abnormalities and many chemical and biological factors. Wilms tumor may be the result of abnormal proliferation due to the failure of metanephric embryo base to differentiate into renal tubules and glomeruli. Bove and McAdams (1976) suggested that the nephroblastomosis complex may transform into nephroblastoma. Knudson and Strong (1972) believed that Wilms tumor can be divided into two types: genetic type and non-genetic type according to whether germ cells have mutations. If it belongs to the genetic form, the tumor occurs earlier, and it is more likely to occur in a bilateral and multicentric form. All bilateral Wilms' tumors and 15% to 20% of unilateral lesions are genetically related. In addition, the probability of tumors in the offspring of hereditary bilateral Wilms tumor can reach 30%, while that of unilateral lesions is about 5%. Hoffinan (1989) and Koufos (1984) found that there is a recessive tumor suppressor gene deletion on 11p13 (WT1), while in patients with Beckwith-Wiedemann syndrome, they found that the end of 11p13 and 11p15 (WT2) are allelic heterozygous. Interestingly, WT1 plays an important role in the formation of normal kidney embryos, and the deletion of alleles of this gene will lead to genitourinary dysplasia (Kreidberg, 1993). About 15% of children with Wilms tumor have other congenital malformations. A study by the US Wilms Tumor Research Group (NWTS) showed that 1.1% of children with Wilms tumor had absent iris, which was much higher than the incidence in the normal population (0.2%), and often accompanied by urogenital malformations (cryptorchidism, urethra) Hypospadias and renal fusion or ectopic), external ear deformities, mental retardation, head and facial deformities, inguinal or umbilical hernia (Haicken and Miller, 1971). 2.9% had unilateral limb hypertrophy (Janik and Seeler, 1976), often combined with embryonic carcinoma, adrenal cortical carcinoma, and hepatoblastoma. In addition, children with Wilms tumor can be combined with malignant tumors (sarcoma, adenocarcinoma, and leukemia) in other parts of the body.
From the embryological point of view, the persistent metanephric embryonic base failed to differentiate into glomeruli and tubules and showed abnormal proliferation and developed into Wilms tumor. Tumors can appear in hereditary or non-hereditary forms. If it belongs to the genetic form, the tumor occurs earlier, and it is more likely to be bilateral and multicentric. All bilateral Wilms tumors and 15% to 20% of unilateral lesions are genetically related, but in the etiological review of Belasc0, genetic factors are not important, and only 1% to 2% of patients have a family history.
The pathogenesis of this disease is unknown, and it may be related to the abnormal development of the embryonic kidney. This tumor is a malignant solid tumor composed of mesenchyme, embryo and epithelium, including epithelial tissue, connective tissue, muscle tissue, skeletal tissue, and nerve tissue. It has a capsule outside and a gray-yellow cut surface with cystic degeneration. Necrotic bleeding area. Adjacent kidney tissue is compressed and shrunk, and the renal pelvis and calyces can be deformed. Hematological metastasis can occur in the early stage, and the common metastasis sites are lung, liver, brain, etc. 10%-45% of cases have tumor thrombi in the renal vein and inferior vena cava. Lymphatic metastasis is of secondary importance. The prognosis of Wilms tumor is related to the structure of the tumor. Typical Wilms tumor and partially differentiated cystic Wilms tumor have a good prognosis, but the undifferentiated type has a poor prognosis. NWTS divides it into 5 phases. Stage I: The tumor is confined to the kidney and can be completely removed; Stage II: The tumor infiltrates the perrenal fat or tumor thrombus in the renal vein, but there is no obvious tumor remaining after the operation; Stage III: The tumor cannot be completely removed, and there is still remaining after the operation. But limited to the abdomen, there is no blood metastasis; stage IV: blood metastasis to lung, liver, bone, brain, etc.; stage V: bilateral Wilms tumor.
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