Types of Treatment for Wilms’ Tumor
Symptoms of Wilms’ Tumor include fever, fatigue, muscle weakness, and loss of appetite. There are several types of treatment for this disease. These include chemotherapy, surgery, and stem cell transplantation.
Surgery
Surgical treatment for Wilms’ tumor is usually a combination of chemotherapy and surgery. The first-line treatment is usually a combination of vincristine and actinomycin D. In some cases, radiation therapy may be used. The treatment usually varies according to the stage of cancer.
The UMBRELLA protocol provides comprehensive guidelines for the management of adult Wilms tumors. It is based on a consensus that reflects the best available international standards and recommendations. It includes specific recommendations for all age groups, including recommendations for localized and metastatic Wilms tumors. It also includes a new classification system for nonsyndromic unilateral (NSS) Wilms tumors. The protocol is intended as a backbone for future phase I/I trials and serves as an international research collaboration.
The protocol also includes recommendations for the diagnosis and treatment of infant and relapsed Wilms tumors. It specifies that patients with relapsed Wilms tumors should be divided into three groups. The first group will be comprised of patients with initial stage I-II low-risk tumors. The other two groups will be composed of patients with stage III or IV intermediate-risk tumors.
The UMBRELLA protocol also recommends preoperative chemotherapy. It aims to minimize the use of radiation and doxorubicin in the preoperative phase. It consists of two regimens: Regimen I, which consists of vincristine, and Regimen II, which consists of actinomycin D. The doses are adjusted according to age.
In addition, the UMBRELLA protocol recommends a fine-needle biopsy as a diagnostic tool for unusual imaging findings. It also recommends percutaneous cutting needle biopsy in difficult situations. It specifies that an absolute residual volume of the blastema is to be measured to improve the definition of blastemal-type histology.
The UMBRELLA protocol continues to recommend the preoperative chemotherapy of vincristine and actinomycin-D. It also includes recommendations for the treatment of bilateral and metastatic Wilms tumors.
Chemotherapy
Usually, the treatment for Wilms’ tumor is surgery followed by chemotherapy. The goal of treatment is to resect cancer as well as to treat any metastases. Depending on the stage of cancer, the patient may require radiation therapy. The aim is to reduce the symptoms of terminal illness while maximizing quality of life.
In children, the risk of developing a metachronous Wilms tumor is low. However, it has been reported that synchronous bilateral renal tumors are very rare. In this scenario, it is necessary to identify additional predictive factors.
Wilms tumors are classified according to their genetic characteristics. MLLT1 mutations are thought to play a role in the development of Wilms tumors. Patients with MLLT1-mutant tumors present at younger ages and have high rates of precursor intralobar nephrogenic rests. In addition, patients with a mutation of CTNNB1 show a higher prevalence of pulmonary metastases.
The risk of Wilms’ tumor survivors dying from cardiac-related causes is 25%, while it is 50% for those with malignant neoplasms. There is a trend that Wilms tumor survivors have a lower incidence of end-stage renal disease than the general population.
Using an unsupervised analysis of gene expression data, 75 Wilms tumor patients were clustered into six groups. The highest-risk subtype was focal anaplasia. The group was characterized by three patients who had local tumor recurrence and seven who had an intestinal obstruction.
Patients with WT1 missense mutations are the least likely to develop Wilms’ tumor. The group contains a small number of patients with mutations in the YEATS domain of MLLT1. This protein shows altered binding to acetylated histone tails, suggesting that it might contribute to the development of the disease.
The treatment of Wilms’ tumor in adults varies from patient to patient. In some cases, the patient will undergo a nephrectomy followed by chemotherapy. In others, cancer will be treated with a combination of drugs.
Stem cell transplantation
Generally, treatment for Wilms’ tumor involves surgery to remove as much of cancer as possible, followed by chemotherapy and radiation therapy. The majority of patients respond to these standard therapies, but a minority develop recurrence. Some children also develop metastatic disease, which can be treated with chemotherapy or radiation therapy.
Autologous hematopoietic stem cell transplantation has been used in the treatment of high-risk childhood solid tumors for the last two decades. Retrospective studies have shown that this treatment has a higher response rate than conventional chemotherapy for neuroblastoma, and is therefore considered a first-line treatment. In addition, it may be effective in reducing radiation dose in high-risk sPNET.
An extensive review of the literature on allogeneic and autologous hematopoietic and allogeneic and autologous bone marrow/stem cell transplantation has shown that there is no evidence that allogeneic transplantation has a greater benefit than autologous transplantation. Additionally, allogeneic stem-cell transplantation has a higher complication rate than autologous transplantation.
The use of tandem or sequential transplant protocols to treat high-risk neuroblastoma has been explored. A systematic review by Raghuram et al identifies risk factors for HDC with HSCR, as well as predictive and predictive/predictive factors for survival after HDC with HSCR.
A previous study by Dunkel and colleagues reported on the outcomes of 25 patients with recurrent medulloblastoma, ependymoma, and medulloblastoma. The patients were a median age of 11.5 years at diagnosis and were given high-dose thiotepa and carboplatin followed by autologous stem-cell transplantation. In addition, they underwent hyper-fractionated total body irradiation and myeloablative chemotherapy.
Another study by Zacharoulis and colleagues investigated the effect of an intensive chemotherapy induction regimen on the outcome of autologous hematopoietic. Twenty-two children had a primary tumor in the posterior fossa and were treated with five cycles of chemotherapy. They were also evaluated for residual tumor size.
Genetic mutations
Molecular studies in Wilms’ tumor are often complicated by the lack of target cell lines for the analysis. However, these studies have provided important insights into the genetic architecture of the disease.
In addition to the RB1 (614041) tumor suppressor gene, four other genes have been linked to Wilms’ tumor oncogenesis. These are the H19 locus, E7-associated cell-surface antigen, REST, and DICER1.
The H19 locus, which is located at 11p, was found to be hypermethylated in 58% of expansions. Similarly, the E7-associated cell-surface antigen was associated with an association region on 11p13.
FBXW7, which codes for the substrate recognition component of the E3-ubiquitin ligase SCF complex, frequently mutates in many cancers. However, its mutations in Wilms’ tumors are not reported.
Another gene known to predispose to Wilms’ tumor is the DIS3L2 gene. In one family, a boy developed the condition following a de novo balanced translocation of the intron 6 of this gene.
In another study, the presence of two independent intragenic mutations within the WT1 gene was noted. This provided the first instance of two somatic alterations restricted to the WT1 gene. It also reaffirmed the authenticity of the zinc finger gene as a disease locus.
In addition to known gene mutations, somatic truncation of the WT1 protein is found in a small percentage of Wilms’ tumor patients. These truncations account for 5% of the total Wt1 protein in the tumor. In addition, stromal-predominant histology is more likely in tumors with truncated WT1 protein.
A third genetic feature of Wilms’ tumor is a loss of heterozygosity at the 11p13 region. Interestingly, this loss of heterozygosity was found in 67% of stromal-predominant tumors.
The loss of heterozygosity at the WT1 gene is associated with renal dysfunction. This gene is known to play a key role in urogenital development.
Treatment options
Getting an accurate diagnosis is very important when choosing treatment options for Wilms’ tumor. After a diagnosis, a child will receive treatment that is customized to his or her condition. This includes surgery and chemotherapy.
In addition to surgery, children may also undergo radiation therapy. During radiation therapy, high-energy X-rays are used to destroy cancer cells. This type of therapy is most commonly applied to the later stages of Wilms’ tumor.
There are several imaging tests that can detect and treat kidney tumors. Some of the tests include computerized tomography, magnetic resonance imaging, and ultrasound. These are used to find tumors and other abnormalities near the kidney.
In many cases, a biopsy is performed before or after treatment. The sample is then sent to the lab for examination. Then, further tests are used to stage cancer and assign a stage to the tumor. This helps doctors decide which treatment option is best.
Various factors can increase the risk of having a Wilms’ tumor. For instance, certain birth defects, genetic syndromes, and maternal exposure during pregnancy all contribute to the likelihood of a child developing the disease.
Other risks can include hemihypertrophy and aniridia. A family history of the disease is also a risk factor.
Survivorship depends on the stage of the disease, as well as the overall health of the child. Children with a Wilms’ tumor are usually cured if they are diagnosed early. However, survival rates may vary greatly from one child to another.
In some cases, a patient may need to undergo a stem cell transplant. A stem cell transplant is a procedure where the child’s own stem cells are transferred to the body to fight the disease. This is a risky procedure and can be difficult to perform.
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