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"Confronting monsters in the darkness."

Updated: Mar 23

How we stand as guardians against the shadowy specter of pediatric urological cancer.


Wilms' Tumour


A significant concern in paediatric urology is Wilms' tumour, also known as nephroblastoma, a cancer primarily affecting children. This tumour, which originates in the kidneys, is most prevalent in children aged three to four years. However, it can occasionally be diagnosed in older children and, in rare cases, adults. Wilms' tumour accounts for approximately 90% of all paediatric kidney cancers, emphasizing its significance in paediatric urology.


The exact cause of Wilms' tumour remains unclear, but it is believed to result from a mutation in the genes responsible for kidney development. This mutation allows immature kidney cells to proliferate uncontrollably, leading to the formation of a tumour. In a small percentage of cases, Wilms' tumour is associated with certain genetic syndromes, such as WAGR syndrome, Denys-Drash syndrome, or Beckwith-Wiedemann syndrome to name a few. This association suggests a genetic predisposition in some patients.


Wilms tumors are grouped into 2 major types based on how they look under a microscope (their histology):

  • Favorable histology: Although the cancer cells in these tumors don’t look quite normal, there is no anaplasia. More than 9 of 10 Wilms tumors have a favorable histology. The chance of cure for children with these tumors is very good.

  • Unfavorable histology (anaplastic Wilms tumor): In these tumors, the look of the cancer cells varies widely, and the cells’ nuclei (the central parts that contain the DNA) tend to be very large and distorted. This is called anaplasia. The more anaplasia a tumor has, the harder it is to cure.


Clinically, Wilms' tumour often presents as a painless, palpable abdominal mass. Other symptoms may include abdominal pain, blood in the urine, fever, nausea, or loss of appetite. However, these symptoms are common to many conditions, making it crucial for healthcare providers to maintain a high degree of suspicion when evaluating children with these complaints.


Diagnosis of Wilms' tumour involves a combination of physical examination, imaging studies, and biopsy. Imaging studies such as ultrasound or computed tomography (CT) can reveal the presence of a kidney mass. Still, a definitive diagnosis requires a biopsy, where a small sample of the tumour is removed and examined under a microscope. The biopsy not only confirms the diagnosis but also helps determine the tumour's stage and plan appropriate treatment.


The stage of a cancer is a term used to describe its size and whether it has spread beyond its original site. Knowing the particular type and the stage of cancer helps doctors decide on the most appropriate treatment. In the case of a Wilms’ tumor, the stage is finalized after surgery to remove the tumor. As most Wilms’ tumor patients receive chemotherapy before surgery, you may not know the exact stage of your child's tumor straight away. An exception is that babies under six months old usually have surgery straight away.


A commonly-used staging system for Wilms' tumor is described below.

Stage 1

The tumor is only affecting the kidney and has not begun to spread. It can be completely removed with surgery.

Stage 2

The tumor has begun to spread beyond the kidney to nearby structures, but it’s still possible to remove it completely with surgery.

Stage 3

The tumor has spread beyond the kidney; either because the tumor has burst before (or during) the operation, has spread to lymph glands (nodes), or has not been completely removed by surgery.

Stage 4

The tumor has spread to other parts of the body such as the lungs or liver. Tumors in other parts of the body are known as metastases.

Stage 5

There are tumors in both kidneys (bilateral Wilms' tumor).

If the tumor comes back after initial treatment, this is known as recurrent cancer or relapse.


Treatment for Wilms' tumour typically involves a combination of surgery, chemotherapy, and sometimes radiation therapy. The choice of treatment depends on the disease's stage and the patient's overall health. Surgery usually involves removing the affected kidney (nephrectomy), but in cases where the tumour affects both kidneys, a partial nephrectomy may be performed to preserve kidney function. Chemotherapy is used to shrink the tumour before surgery and to kill any remaining cancer cells after surgery. Radiation therapy may be used in advanced cases or when the tumour has spread to other parts of the body.


The prognosis for children with Wilms' tumour is generally good, with survival rates exceeding 90% due to advances in diagnosis and treatment. However, long-term follow-up is crucial as survivors are at risk for late effects of treatment, including heart and lung problems, growth retardation, and the development of secondary cancers.


Despite the favourable prognosis, Wilms' tumour poses significant challenges, both medical and emotional, for the affected children and their families. Therefore, a multidisciplinary approach involving paediatric urologists, oncologists, radiologists, pathologists, and mental health professionals is essential to provide comprehensive care.



Neuroblastoma



Neuroblastoma is a type of cancer that primarily affects children, usually under the age of five. It is the most common extracranial solid tumor found in children, accounting for approximately 8% of all pediatric malignancies. Despite its prevalence, this disease remains a complex and challenging condition for healthcare practitioners, particularly in the field of paediatric urology, due to its tendency to present with a wide range of symptoms and clinical manifestations.


Neuroblastoma originates in the adrenal glands, which are small organs that sit on top of the kidneys. It can, however, spread to other parts of the body, such as the chest, abdomen, spine, and bones. The adrenal glands are part of the body's endocrine system, which releases hormones into the bloodstream. They produce hormones that are essential for the regulation of heart rate, blood pressure, sugar levels and stress response. Consequently, a tumor in this area can disrupt these crucial bodily functions.


The symptoms of neuroblastoma are diverse and often non-specific. They can include abdominal pain, changes in bowel habits, unexplained weight loss, fatigue, and fever. Sometimes, a lump can be felt in the abdomen. If the disease has spread to the bones, the child may present with bone pain or pathological fractures. In some cases, the child may also exhibit signs of hormonal imbalance, such as high blood pressure or rapid heartbeat.


The diagnosis of neuroblastoma involves a series of tests. An initial physical examination is performed to check for lumps or other abnormalities. Blood and urine tests are conducted to look for substances that are often found in higher amounts in children with neuroblastoma. Imaging tests, such as X-rays, ultrasound, computed tomography (CT) scans, and magnetic resonance imaging (MRI), are used to visualize the tumor and determine its size and location. A biopsy, in which a small sample of tissue is removed for examination under a microscope, is usually necessary to confirm the diagnosis.


INSS STAGING FOR NEUROBLASTOMA:

Stage 1: The tumor can be removed completely during surgery. Lymph nodes attached to the tumor removed during surgery may or may not contain cancer, but other lymph nodes near the tumor do not.

Stage 2A: The tumor is located only in the area it started and cannot be completely removed during surgery. Nearby lymph nodes do not contain cancer.

Stage 2B: The tumor is located only in the area where it started and may or may not be completely removed during surgery, but nearby lymph nodes do contain cancer.

Stage 3: The tumor cannot be removed with surgery. It has spread to regional lymph nodes (lymph nodes near the tumor) or other areas near the tumor, but not to other parts of the body.

Stage 4: The original tumor has spread to distant lymph nodes (lymph nodes in other parts of the body), bones, bone marrow, liver, skin, and/or other organs, except for those listed in stage 4S, below.

Stage 4S: The original tumor is located only where it started (as in stage 1, 2A, or 2B) and has spread only to the skin, liver, and/or bone marrow in infants younger than 1. The spread to the bone marrow is minimal (usually less than 10% of cells examined show cancer).


The treatment of neuroblastoma is multifaceted and depends on several factors, such as the child's age, the stage of the disease, and the specific characteristics of the tumor. Surgery is often used to remove as much of the tumor as possible. Chemotherapy and radiation therapy are used to kill any remaining cancer cells. In some cases, stem cell transplant, immunotherapy or targeted therapy may be necessary.


Despite the severity of this disease, the prognosis for children with neuroblastoma varies widely. Some forms of the disease are very treatable, while others are more aggressive and resistant to treatment. Recent advances in medical research have led to the development of new treatment strategies that have significantly improved the survival rates for this devastating disease.



Rhabdomyosarcoma



Rhabdomyosarcoma is a rare but highly malignant tumour that can occur in the soft tissues of children and adolescents. This tumour, which originates from the skeletal muscle cells, is the most common soft tissue sarcoma in the paediatric population.


Rhabdomyosarcoma can occur anywhere in the body, but it is most frequently observed in the head and neck region, followed by the genitourinary tract. In the latter, the most common sites are the bladder and the prostate, but it can also affect the vagina, uterus, and testes. Around 20% of all paediatric rhabdomyosarcoma cases occur in the genitourinary system.


Symptoms of rhabdomyosarcoma are usually non-specific and can mimic other more common conditions, which can lead to a delay in diagnosis. Common symptoms include abdominal pain, blood in the urine, urinary tract infections, and a palpable mass. For tumours located in the bladder or prostate, there may be symptoms related to urinary obstruction or retention.


Diagnosis is typically confirmed by biopsy, where a small sample of the tumour is removed and examined under a microscope. Imaging studies, such as ultrasound, computed tomography (CT), or magnetic resonance imaging (MRI), are also important in determining the size and location of the tumour and whether it has spread to other parts of the body. This process, known as staging, is crucial for treatment planning.


RMS is staged differently from most other cancers. Doctors first determine 3 key pieces of information:

  • The TNM stage (Tumor, Node, Metastasis)

  • The clinical group

  • Whether the cancer cells have a PAX/FOX01 fusion gene


These factors are used to divide patients into risk groups, which then are used to guide treatment. The risk groups are low, intermediate and high risk.


Treatment of rhabdomyosarcoma usually involves a combination of surgery, chemotherapy, and radiotherapy. The goal of surgery is to remove as much of the tumour as possible while preserving the function of the affected organ. However, complete removal is often not possible due to the location and extent of the tumour. In these cases, chemotherapy and radiotherapy are used to shrink the tumour before surgery or to kill any remaining cancer cells after surgery.


The type of chemotherapy used depends on the type and stage of the rhabdomyosarcoma. Commonly used drugs include vincristine, actinomycin, and cyclophosphamide. Radiotherapy may be used in cases where the tumour is not completely resectable or in high-risk patients to improve local control and survival.


The prognosis of rhabdomyosarcoma varies widely and depends on several factors, including the location and size of the tumor, the stage of the disease, the histological subtype, and the patient's response to treatment. With modern treatment protocols, the overall survival rate for children with rhabdomyosarcoma is about 70%.


Survivorship and Long-term Effects



Children diagnosed and treated for urological malignant conditions often face long-term effects that extend into adulthood. While the primary focus of the paediatric urologist is on the immediate diagnosis and treatment, the survivorship and potential long-term effects of these conditions and their treatments cannot be overlooked.


Survivorship, in this context, refers to the health and life of a child post-treatment. It includes aspects such as the child's quality of life, long-term health, and the potential for recurrence of the condition. Survivorship is a critical part of paediatric urology as it helps determine the success of the treatment and the child’s future health status.


Many children who undergo treatment for urological malignancies experience long-term effects. These effects can be physical, psychological, or social and may last for years after the treatment has ended. They can also impact the child’s development and quality of life.


Physical long-term effects can include changes in urinary and sexual function. Others may require additional surgeries or procedures as they grow and develop.

Psychological effects can also be significant. Children may experience anxiety or depression related to their condition or treatment. They may feel different from their peers or struggle with body image issues, particularly if their condition or treatment has altered their physical appearance. These psychological effects can impact their self-esteem and social interactions, potentially leading to social isolation.


Long-term effects can also include social challenges. Children with chronic urological conditions like cancer may miss school or social activities due to hospital stays or medical appointments, which can affect their education and social development. They may also face stigma or bullying from their peers, further exacerbating their social challenges.


Despite these potential long-term effects, it's important to note that many children treated for urological malignancies lead healthy, fulfilling lives. Effective treatment in childhood can prevent or minimize many of these long-term effects. Furthermore, ongoing support and follow-up care can help identify and manage these effects early on, improving the child’s long-term outcomes.




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