Wilms’ tumour

About

Wilms’ tumour, also known as nephroblastoma, is a type of cancer that affects the kidneys. It is a rare condition that is most commonly diagnosed in children younger than 5 years old. The tumour usually appears as a lump in one of the kidneys and can spread to other parts of the body including the abdomen, lungs, and lymph nodes. Diagnosis of Wilms’ tumour is typically done through X-rays, CT scans, and biopsies. Treatment options include surgery, chemotherapy, and radiation therapy. The prognosis is usually good if the tumour is diagnosed and treated early.

Symptoms

The most common symptom of Wilms’ tumour is a swelling or lump in the abdomen. Other symptoms may include fever, weight loss, nausea, vomiting, abdominal pain, and anemia. The child may also develop hypertension, an enlarged liver, or have blood in their urine. As the tumour grows, it may cause compression of the surrounding organs and cause difficulty with urination.

Causes

The cause of Wilms’ tumour is unknown, but certain genetic changes that have been identified are thought to contribute to the development of the disease. These genetic changes include mutations in certain genes such as WT1, WT2, and CTNNB1. Other possible causes may include environmental factors such as exposure to certain types of radiation, certain medications, and/or certain infections, as well as a family history of the disease.

Risk factors

The risk factors for Wilms’ tumour include having a family history of kidney cancer, certain birth defects (including aniridia, hemihypertrophy, and Beckwith-Wiedemann syndrome), certain genetic syndromes (including Denys-Drash syndrome, isolated aniridia, and isolated hemihypertrophy), and certain other rare genetic mutations. Additionally, low birth weight and being born prematurely have been identified as potential risks.

Diagnosis

Wilms’ tumour is typically diagnosed with a combination of physical examination, imaging studies, and biopsy. The physician will use an ultrasound to check the abdomen, looking for a mass or any abnormal growths. An X-ray and CT scan may also be used to gain more detailed images and pinpoint the exact location of the tumour. Once the tumour has been identified, a biopsy will be performed to confirm the diagnosis. This procedure involves removing a sample of the tumour tissue and sending it to a laboratory for further analysis.

Sub-types

Wilms’ tumour is a type of cancer that affects the kidneys, typically found in children under the age of five. There are four different subtypes of Wilms’ tumour, each with their own distinct characteristics and associated prognosis.

The first is the classic Wilms’ tumour, which is the most common subtype, accounting for more than 90% of cases. This type of Wilms’ tumour is usually composed of two parts: a larger, solid component made up of embryonic cells, and a smaller component made up of mature kidney cells. These tumours tend to grow slowly and respond well to treatment.

Aggressive or anaplastic Wilms’ tumour is the second subtype, and it is rare and fast-growing, making up only 5-10% of cases. It is composed of only embryonic cells, and it doesn’t respond well to chemotherapy or radiation.

The third subtype is congenital mesoblastic nephroma (CMN). This type of Wilms’ tumour is not malignant, and it is typically found in newborns. It is composed of cells that have never developed into mature kidney cells. CMN is usually slow-growing and can be treated successfully with surgery and/or chemotherapy.

Finally, the fourth subtype is Anaplastic Wilms’ Tumour with Malignant Rhabdoid Features (AT/MR). This rare subtype is the most aggressive and has the poorest prognosis. It is composed of highly abnormal cells, and it usually does not respond to traditional treatments such as chemotherapy or radiation.

Overall, Wilms’ tumour is a rare cancer but it is important to be aware of the different subtypes in order to diagnose and treat it properly.

Treatments

The treatment for Wilms’ tumour depends on the size and stage of the tumour. The goal of Wilms’ tumour treatment is to remove the tumour and any affected lymph nodes as well as to prevent further spread. In most cases, this involves a combination of surgery, radiotherapy, and chemotherapy.

Surgery is typically used to completely remove the tumour, as well as any affected lymph nodes. Surgery can be performed using either an open procedure (with an incision) or a laparoscopic procedure (using a fibre-optic camera and small incisions).

Radiotherapy is typically used to kill any remaining cancer cells after surgery and to reduce the chances of recurrence. Radiotherapy is sometimes administered before surgery to shrink the tumour size and make it easier to remove.

Chemotherapy is typically used before and after surgery to reduce the chances of cancer recurrence and prolong survival. Chemotherapy may also be used to treat metastatic Wilms’ tumour or when surgery is not an option.

Other treatments may be used in some cases, such as hormonal therapy or stem cell transplantation.

It is important to talk to your doctor to learn about your treatment options for Wilms’ tumour and the potential risks and benefits associated with each.

Prevention

Wilms’ tumour is a rare form of kidney cancer that mainly affects children. The exact cause of Wilms’ tumour is not yet known, but there are some risk factors which can increase the chances of a child developing it. These include a family history of the disease, certain genetic syndromes, and certain congenital abnormalities.

To reduce the risk of Wilms’ tumour, it is recommended to:

  • Be aware of any family history and genetic syndromes that may put a child at higher risk
  • Monitor any congenital abnormalities in the child
  • Encourage children to be physically active, eat a healthy diet, and maintain a healthy weight
  • Encourage regular checkups with a doctor to monitor any signs of the disease
  • Provide any necessary genetic counselling.

Gender differences?

Yes, there are gender-specific differences in the presentation and management of Wilms’ tumour. The most significant difference is that Wilms’ tumour is much more likely to occur in boys than girls, with male patients accounting for approximately 2/3 of cases. Furthermore, there are differences in the pattern of metastasis for Wilms’ tumour between males and females. Male patients are more likely to develop metastasis to the lungs or abdominal lymph nodes, whereas female patients are more likely to develop metastasis to the liver. In terms of treatment, male patients are more likely to receive surgery, radiotherapy and chemotherapy than female patients and are also more likely to experience complications from the treatment such as abdominal wall necrosis, whereas female patients are more likely to experience recurrence of the disease.

Nutrition

Nutrition plays an important role in the management of Wilms’ tumour. Proper nutrition can help to provide the body with the energy, vitamins and minerals needed to fight the cancer and keep the body strong. Eating a balanced diet, including plenty of fruits, vegetables and proteins, can help to prevent malnutrition and other issues such as fatigue. Proper hydration is also important in managing Wilms’ tumour. Additionally, nutrition can help to reduce symptoms and can be used in combination with chemotherapy, radiation and surgery. Good nutrition helps to boost the body’s ability to fight the cancer, allowing the body to function more effectively and heal quicker.

Physical Activity

It is not currently known how physical activity may affect Wilms’ tumour, a rare kidney cancer typically found in young children. Studies have found a slight increase in the risk of Wilms’ tumour among physically active children, but the evidence is not conclusive. Therefore, more research is needed to determine the possible effects of physical activity on Wilms’ tumour.

Further Reading

  1. https://bestpractice.bmj.com/topics/en-us/934/references
  2. https://www.ncbi.nlm.nih.gov/books/NBK442004/
  3. https://www.nature.com/articles/s41572-021-00308-8
  4. https://www.mayoclinic.org/diseases-conditions/wilms-tumor/symptoms-causes/syc-20352655
  5. https://www.cancerresearchuk.org/about-cancer/childrens-cancer/wilms-tumour/about
  6. https://diagnosticpathology.biomedcentral.com/articles/10.1186/1746-1596-1-46

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