Nephroblastoma

Rahul Dhaker M.Sc. N. II year

Introduction

Nephroblastoma is a type of cancer which develops in the

kidneys.

This cancer is also called Wilms tumor, and is the most

common type of kidney cancer to develop in children.

In the 1950s and 1960s, most children with nephroblastoma had a very poor prognosis, but treatment for the cancer has advanced significantly in the intervening decades.

The success rate for Wilms tumor treatment is now

approximately 90%, largely due to the practice of combining

surgery with radiotherapy or chemotherapy.

Cont.

Wilms' tumour was named after Dr Max Wilms, who first

described it. It's a type of kidney cancer.

It's thought to come from very specialised cells in the embryo known as metanephric blastema.

These cells are involved in the development of the child's kidneys while they're in the womb.

These cells usually disappear at birth, but in many children

with Wilms' tumour, cells called nephrogenic rests can still be

found.

Definition

A Wilms tumor is a

malignant tumor (cancer)

which means it has the ability to grow and spread. The places it usually spreads to include

the lungs, liver and

nearby lymph nodes.

Causes

In most children the causes of Wilms' tumour are unknown. In 1 in 100 people with Wilms' tumour, another family member will also have Wilms' tumour.

Nephroblastoma has a global incidence rate of 0.8 cases per

100,000 people.

Most children with this cancer are diagnosed between three and four years of age. Children commonly have tumors in one kidney only; in around 6% of cases tumors grow in both kidneys.

Signs & Symptoms

Signs and symptoms of neuroblastoma vary depending on what part of the body is affected.

Neuroblastoma in the abdomen Neuroblastoma in the chest

Cont

Neuroblastoma in the
abdomen :

Neuroblastoma in the
chest : Wheezing Chest pain Changes to the eyes,

Abdominal pain A mass under the skin that isn't tender when touched

Changes in bowel habits, such as diarrhea

including drooping
eyelids and unequal

Swelling in the legs

pupil size

Other signs and symptoms that may indicate neuroblastoma include

Lumps of tissue under the skin Eyeballs that seem to protrude from the sockets (proptosis)

Dark circles, similar to bruises, around the eyes Back pain Fever Unexplained weight loss Bone pain

history and physical Urinalysis:

blood, sugar, protein and bacteria infection

Blood tests:-

Radiology studies

Ultrasound CT scan X-ray of the abdomen

Biopsy

Treatment

In general, this type of cancer is curable. If the tumor is only in the

kidney (typical), it can be removed along with the whole kidney (a

nephrectomy). During the operation, the surgeon checks if the other kidney has a tumor.

If there are tumors in both kidneys, a piece of the tumor will be removed. After the surgery, the child is given some chemotherapy drugs like Dactinomycin (trade name Cosmegen).

Cont

Children 16 years old or older have higher mortality rates

within their stages. This is due to them being treated less

aggressively and consistently.

Adjuvant chemotherapy is sometimes used.

Surgery Chemotherapy Radiotherapy

Stem cell transplant

Surgery

vincristine and actinomycin D are recommended for all stages

Stage

Stage I

Tumor is limited to the kidney and is completely excised. The surface of the renal capsule is intact.

Treatment: Nephrectomy +/- 18 weeks of chemotherapy depending on age of patient and weight of tumor.

Stage II

Tumor extends beyond the kidney but is completely excised.

Tumor involvement of the blood vessels of the renal sinus and/or outside the renal parenchyma.

Treatment: Nephrectomy + abdominal radiation + 24

weeks of chemotherapy

Stage III

Unresectable primary tumor. Lymph node metastasis.

Tumor is present at surgical margins.

Tumor spillage involving peritoneal surfaces either before or during surgery, or transected tumor thrombus.

Treatment: Abdominal radiation + 24 weeks of chemotherapy + nephrectomy after tumor shrinkage

Stage IV
Stage IV Wilms' tumor is defined as the presence of hematogenous metastases (lung, liver, bone, or brain), or lymph node metastases outside the abdomenopelvic region.

Treatment: Nephrectomy + abdominal radiation + 24 weeks of chemotherapy + radiation of metastatic site as appropriate

Stage V

bilateral renal involvement at the time of initial diagnosis

Treatment: Individualized therapy based on tumor burden

Depending on the stage of the tumour at diagnosis,

radiotherapy may also be given to the area of the affected

kidney or to the whole abdomen.

Radiotherapy treats cancer by using high-energy rays to

destroy the cancer cells, while doing as little harm as possible to normal cell.

Children with high-risk neuroblastoma may receive a transplant using their own blood stem cells (autologous stem cell transplant). The bone marrow produces stem cells, which mature and develop into the red and white cells and platelets that make up the blood.

Child undergoes a procedure that filters and collects stem cells from
his or her blood. Then high doses of chemotherapy are used to kill any remaining cancer cells in child's body. child's stem cells are then injected into child's body, where they can form new, healthy blood cells.

Side effects

feeling sick (nausea) and being sick (vomiting),

hair loss,
bruising and bleeding, tiredness, diarrhoea and an increased risk of infection.

Nursing Management

Prognosis

Effective diagnosis, staging, and multimodality therapy has dramatically reduced the morbidity and mortality in children with nephroblastoma

Poor prognostic factors in children include the presence of

bone metastases and tumor spillage during surgery