What Is Nephroblastoma?
Wilms tumor is an embryonic malignancy. Rance first reported the disease in 1814, and Wilms made a detailed pathological description of the disease in 1899. Therefore, it was named Wilms tumor. In 2004, "Tumour Pathology and Genetics of Tumors of the Urinary System and Male Reproductive Organs" in the classification of renal tumors believed that the disease originated from renal basal cells.
- Also known as
- Wilms tumor, nephroblastoma
- English name
- Wilmstumor; nephroblastoma
- Visiting department
- Multiple groups
- Common symptoms
- Abdominal mass
Epidemiology and etiology of nephroblastoma
- Wilms tumors occur in children and are the second most common abdominal malignancy in children. 98% of cases occur under 10 years of age, most commonly seen in children under 3 years of age, the incidence is significantly reduced after 3 years of age, rare after 5 years of age, rare in adults, about 3% occur in adults, and are called adult nephrocytes tumor. 20% of adult nephroblastomas occur at the age of 15-20 years, and 80% occur at the age of 30-70 years. There is no significant difference in the incidence of men and women, most of them are on one side, 3% to 10% are bilateral, and both patients occur simultaneously or sequentially.
- The exact etiology of nephroblastoma is unknown, and may be related to the loss or mutation of the WT-1 gene on chromosome 11 (located at 11p13), or it may be due to the differentiation of mesenchymal blastoblasts into the posterior kidney tissue. And caused by continuous proliferation. The disease also has a certain familial tendency, so some people think that the disease is also hereditary.
Clinical manifestations of nephroblastoma
- Most children with nephroblastoma are inadvertently found with a lump in the abdomen, such as touching the lump while bathing, changing clothes, or touching the abdomen of the child. Usually the surface of the mass is smooth and flat, with hard texture and no tenderness. The mass is usually relatively fixed. In some children, the abdomen is swollen or asymmetric on both sides. A few children have symptoms of abdominal pain or nausea, vomiting, and anorexia. There are also a few children with hematuria, fever, and hypertension. Late-stage children may appear pale, wasted, debilitated, and even show metastatic symptoms such as hemoptysis and headache. Congenital malformations are present in 12% to 15% of children, such as congenital iris choroidal defects, repetitive kidneys, horseshoe kidneys, polycystic kidneys, ectopic kidneys, visceral hypertrophy, umbilical bulge, giant tongue, and hypertrophy. The clinical manifestations of adult nephroblastoma are similar to those of patients with renal cell carcinoma. They are asymptomatic, hematuria, low back pain, and abdominal mass.
Diagnosis of nephroblastoma
- The clinical diagnosis of the disease is based on imaging studies, including plain radiographs, excretory urography, abdominal ultrasound, abdominal CT or MRI. Among them, the simplest examination method is abdominal ultrasonography, and plain CT and enhanced scans of the abdomen are the most important examination items. The accuracy of diagnosing nephroblastoma is over 95%. However, patients with renal insufficiency and inferior vena cava tumor emboli should have an MRI scan of the abdomen. For patients who cannot be surgically removed, tumor biopsy should be considered for pathological examination to confirm the diagnosis and guide the treatment plan based on the results of pathological examination.
Treatment and prognosis of nephroblastoma
- Children's nephroblastoma is sensitive to radiotherapy and chemotherapy. Due to the comprehensive treatment, the cure rate of children is higher, which is a model for successful treatment of malignant tumors. Before the 1950s, the only treatment for nephroblastoma was surgery, and the 5-year survival rate of children was about 20%. By the 1950s and 1960s, surgery combined with radiotherapy was used, and the 5-year survival rate of children reached 45% to 50%. After that, the 5-year survival rate of children with surgery combined with radiotherapy and chemotherapy reached more than 85%. Postoperative radiotherapy should be performed as soon as possible if needed. Commonly used chemotherapeutic drugs are actinomycin D (A), cyclophosphamide (C), doxorubicin (D), vincristine (V), etoplatin (E), and 2 mercaptoethyl sulfonate ( M). Commonly used schemes are A + V scheme, A + V + D scheme, V + D + E + C + M scheme. Adult patients with early stage nephroblastoma are mainly treated by surgery, but the treatment effect of adult patients with advanced stage nephroblastoma is far inferior to that of children. Except for surgical treatment, no comprehensive treatment plan with good curative effect has been explored so far.