Download as pdf or txt
Download as pdf or txt
You are on page 1of 24

DEPARTMENT OF RADIOLOGY,

COLLEGE OF MEDICINE,

UNIVERSITY OF IBADAN

RADIOLOGY OF WILMS TUMOUR

(Being in partial fulfilment of the Elective Posting in the Department of Radiology)

BY

IGWE SOLOMON I.

190997

GROUP B, 2018

FEBRUARY 2022
CONTENTS

I. INTRODUCTION .............................................................................................................. 4

A. BRIEF OVERVIEW OF WILMS TUMOUR .................................................................... 4

B. IMPORTANCE OF RADIOLOGY IN THE DIAGNOSIS AND MANAGEMENT OF

WILMS TUMOUR .................................................................................................................... 5

II. PATIENT PRESENTATION AND CLINICAL HISTORY ............................................. 7

A. DEMOGRAPHIC INFORMATION (AGE, GENDER, ETC.) ......................................... 7

B. Presenting symptoms and duration ....................................................................................... 8

C. Family history (if applicable) ................................................................................................ 9

III. Radiological Findings ........................................................................................................ 11

A. Imaging Modalities Used: ................................................................................................... 11

B. Description of Radiological Findings: ................................................................................ 12

C. Radiological Staging of the Tumour: .................................................................................. 13

IV. Differential Diagnosis ........................................................................................................ 14

A. Discussion of other conditions that may present with similar radiological findings: ......... 14

B. Differentiating features of Wilms Tumour from other differential diagnoses: ................... 15

V. Treatment and Management................................................................................................ 16

A. Overview of Treatment Options for Wilms Tumour: ......................................................... 16

B. Role of Radiology in Treatment Planning and Monitoring: ............................................... 16

C. Description of Surgical Interventions, Chemotherapy, and Radiation Therapy: ................ 16

D. Follow-up Imaging and Surveillance Recommendations: .................................................. 17

VI. Prognosis and Outcome ..................................................................................................... 18

2
A. Discussion of Prognosis Factors and Survival Rates: ......................................................... 18

B. Long-term Outcomes and Potential Complications: ........................................................... 19

C. Impact of Radiological Findings on Prognosis and Treatment Decisions: ......................... 19

VII. Conclusion ........................................................................................................................ 20

REFERENCES ........................................................................................................................ 23

3
I. INTRODUCTION

A. BRIEF OVERVIEW OF WILMS TUMOUR

Wilms Tumour, also known as nephroblastoma, is a rare type of kidney cancer that primarily

affects children. It is the most common renal tumour in paediatric patients, accounting for

approximately 90% of all kidney tumours in children (1). Wilms Tumour usually occurs in

children aged 3 to 4 years, but it can be seen in infants and older children as well. The tumour

is named after Max Wilms, a German surgeon who first described it in 1899.

The exact cause of Wilms Tumour is still unknown, although several genetic and

environmental factors have been implicated. Genetic predisposition plays a significant role in

the development of Wilms Tumour, as about 10% of affected children have a family history of

the disease (2). Several genes have been associated with the development of Wilms Tumour,

including WT1, WT2, and WTX. Mutations or abnormalities in these genes can disrupt normal

kidney development and lead to tumour formation.

Clinically, children with Wilms Tumour typically present with a painless abdominal mass,

which is often discovered by parents or healthcare providers during routine physical

examination. Other signs and symptoms may include abdominal pain, haematuria (blood in the

urine), hypertension (high blood pressure), and fever. However, these symptoms are

nonspecific and can also be associated with other conditions, emphasizing the importance of

radiological evaluation for accurate diagnosis.

Radiology plays a crucial role in the diagnosis and management of Wilms Tumour. The initial

imaging modality of choice is typically an abdominal ultrasound (US), which can accurately

identify the presence of a renal mass and evaluate its characteristics (1). US features suggestive

of Wilms Tumour include a well-defined, predominantly solid mass with variable echogenicity.

4
US can also help determine the tumour's location within the kidney and assess its relationship

with adjacent structures.

Once a renal mass is identified on US, further radiological evaluation is warranted to

characterize the tumour and assess its extent. Computed tomography (CT) and magnetic

resonance imaging (MRI) are commonly used modalities for this purpose. CT provides

excellent spatial resolution and is particularly useful for evaluating the extent of tumour

involvement, detecting lymph node metastasis, and assessing the presence of distant metastases

(3). MRI, on the other hand, provides detailed soft tissue characterization and can aid in

differentiating Wilms Tumour from other renal tumours.

B. IMPORTANCE OF RADIOLOGY IN THE DIAGNOSIS AND MANAGEMENT OF


WILMS TUMOUR

Radiology plays a crucial role in the diagnosis and management of Wilms Tumour, offering

valuable insights into the tumour's characteristics, staging, and treatment planning. The

utilization of various radiological imaging modalities allows for accurate assessment of the

extent of disease, evaluation of potential complications, and monitoring of treatment response.

Imaging techniques, such as ultrasound (US), computed tomography (CT), and magnetic

resonance imaging (MRI), are essential in the initial diagnosis of Wilms Tumour. Ultrasound

is often the first-line imaging modality used to evaluate a suspected renal mass in children (1)

It allows for the identification and characterization of the tumour, assessment of its size,

location within the kidney, and involvement of adjacent structures. Ultrasound features

suggestive of Wilms Tumour include a well-defined, predominantly solid mass with variable

echogenicity (3).

Once a renal mass is identified, additional radiological investigations, such as CT and MRI,

are employed to further evaluate the tumour and its extent. CT provides excellent spatial

5
resolution, allowing for accurate staging and assessment of local invasion, lymph node

involvement, and distant metastases (3). CT scans aid in determining the precise location and

size of the tumour, which is crucial for surgical planning and identifying potential

contraindications to nephron-sparing surgery. Moreover, CT can detect small pulmonary

metastases that may be missed on chest X-rays (3).

MRI, with its superior soft tissue contrast resolution, is valuable for differentiating Wilms

Tumour from other renal masses, particularly those with cystic or complex solid components

(1). It helps evaluate the tumour's involvement of surrounding structures, such as the renal vein,

inferior vena cava, or adjacent organs. Additionally, MRI is beneficial for assessing the

tumour's response to neo-adjuvant chemotherapy and for monitoring treatment response during

follow-up (3).

The radiological findings obtained through these imaging modalities contribute significantly

to the staging of Wilms Tumour. Staging is crucial for determining the appropriate treatment

approach and predicting prognosis. The International Society of Paediatric Oncology (SIOP)

staging system is commonly used and takes into account the tumour size, local invasion, lymph

node involvement, and distant metastasis (3). Radiological imaging provides the necessary

information for accurate staging and facilitates appropriate treatment planning.

Furthermore, radiology plays an essential role in the evaluation of treatment response and

monitoring disease progression. Serial imaging assessments during and after chemotherapy

allow for the assessment of tumour regression, identification of residual disease, and detection

of recurrent or metastatic disease (1). These imaging studies aid in clinical decision-making

regarding the continuation of chemotherapy, adjustment of treatment regimens, or

consideration of surgical intervention.

6
II. PATIENT PRESENTATION AND CLINICAL HISTORY

A. DEMOGRAPHIC INFORMATION (AGE, GENDER, ETC.)

Demographic information, such as age and gender, is important in understanding the

epidemiology and clinical characteristics of Wilms Tumour. Wilms Tumour primarily affects

paediatric patients, with the majority of cases occurring in children aged 3 to 4 years (4).

However, it can also be seen in infants and older children. The incidence of Wilms Tumour is

relatively rare, accounting for approximately 6% of all childhood cancers (5).

Age is a significant factor in the diagnosis and management of Wilms Tumour. Younger age

at diagnosis is associated with a higher likelihood of favourable histology, which is correlated

with a better prognosis (4). Children diagnosed before the age of 2 years tend to have a higher

proportion of favourable histology tumours, while those diagnosed at an older age may have a

higher proportion of unfavourable histology tumours.

Gender differences have also been observed in Wilms Tumour. The condition is slightly more

common in girls than in boys, with a male-to-female ratio of approximately 1:1.2 (5). However,

the clinical significance of this gender disparity remains unclear, and further research is needed

to explore potential underlying factors contributing to this observation.

Apart from age and gender, other demographic factors can play a role in the incidence and

clinical presentation of Wilms Tumour. There is evidence suggesting a higher incidence of

Wilms Tumour in certain racial and ethnic populations. For instance, studies have reported

higher incidence rates among African-American and Hispanic children compared to children

of Caucasian descent (6). Socioeconomic factors and access to healthcare may also influence

the stage at diagnosis, treatment outcomes, and survival rates among different populations (6).

7
Understanding the demographic characteristics of Wilms Tumour patients is important for

several reasons. First, it helps identify specific populations at higher risk, allowing for targeted

screening and early detection efforts. Second, demographic information provides insights into

potential genetic and environmental factors that may contribute to the development of Wilms

Tumour. Family history and genetic predisposition, for example, are more prevalent in certain

populations and may influence tumour development (5). Lastly, demographic factors can

impact treatment outcomes and survival rates, as they may be associated with variations in

tumour biology, access to healthcare, and treatment compliance (6).

B. Presenting symptoms and duration

Wilms Tumour, also known as nephroblastoma, is the most common type of kidney cancer that

affects children. The disease often presents with various signs and symptoms, which can vary

depending on the stage of the tumour and its location within the kidney.

The most common presenting symptom of Wilms Tumour is the presence of an abdominal

mass or swelling. Parents or caregivers may notice a firm, non-tender mass in the child's

abdomen during routine physical examination or while bathing the child (7). The mass is

typically smooth and confined to one side of the abdomen, although it can occasionally involve

both kidneys. In some cases, the tumour may be large enough to cause abdominal pain or

discomfort.

Other symptoms that can occur in children with Wilms Tumour include haematuria (blood in

the urine), hypertension (high blood pressure), and fever. Haematuria, which may be visible or

microscopic, can occur due to tumour invasion into the renal pelvis or blood vessels within the

kidney (7). Hypertension can result from the release of renin by the tumour or the compression

of the renal arteries (7). Fever, although less common, can be a manifestation of tumour

necrosis or infection within the tumour (8).

8
The duration of symptoms before diagnosis can vary widely. In some cases, the mass may be

detected incidentally during routine physical examination or imaging for other reasons,

resulting in an early diagnosis. However, in many cases, the duration between the onset of

symptoms and diagnosis can be several weeks to months. Delayed diagnosis may occur due to

a variety of factors, including misinterpretation of symptoms, lack of awareness about Wilms

Tumour, and atypical clinical presentations (8).

Early diagnosis and prompt treatment of Wilms Tumour are crucial for optimal outcomes.

Imaging studies such as ultrasound, computed tomography (CT), or magnetic resonance

imaging (MRI) are commonly used to evaluate the extent and characteristics of the tumour.

These imaging modalities can help determine the size, location, and involvement of adjacent

structures, aiding in the staging of the tumour (7). Additionally, blood tests and urine analysis

may be performed to assess kidney function and identify any abnormalities.

It is important to note that while Wilms Tumour is typically asymptomatic or associated with

non-specific symptoms, there are rare instances where the tumour may present with more

severe manifestations. These can include acute abdominal pain, anaemia, weight loss, or even

metastatic spread to distant organs (8).

C. Family history (if applicable)

Family history plays a significant role in the context of Wilms Tumour, as there is evidence to

suggest a genetic predisposition to the disease. While the majority of Wilms Tumours occur

sporadically without a family history, a small proportion of cases are associated with inherited

genetic abnormalities that increase the risk of developing the tumour.

The most well-known genetic syndrome associated with Wilms Tumour is WAGR syndrome

(Wilms Tumour, Aniridia, Genitourinary anomalies, and mental Retardation). WAGR

9
syndrome is caused by a deletion or mutation in the WT1 gene on chromosome 11p13, which

predisposes individuals to Wilms Tumour, aniridia (absence of the iris), genitourinary

anomalies, and intellectual disabilities (9). Children with WAGR syndrome have a

significantly increased risk of developing Wilms Tumour, with estimates ranging from 30% to

50% (4).Therefore, a thorough evaluation of the family history should be performed in children

diagnosed with Wilms Tumour, especially in cases of bilateral or multifocal tumours.

Other genetic syndromes associated with an increased risk of Wilms Tumour include Denys-

Drash syndrome and Beckwith-Wiedemann syndrome. Denys-Drash syndrome is caused by

mutations in the WT1 gene and is characterized by early-onset nephropathy, genitourinary

anomalies, and a high risk of Wilms Tumour (9). Beckwith-Wiedemann syndrome, on the other

hand, is associated with abnormalities on chromosome 11p15 and is characterized by

macrosomia (large body size), macroglossia (enlarged tongue), and omphalocele (abdominal

wall defect) (4). Children with Beckwith-Wiedemann syndrome have an increased risk of

developing Wilms Tumour, particularly those with specific genetic abnormalities such as loss

of methylation at the H19 locus (9).

In addition to these specific syndromes, a positive family history of Wilms Tumour in first-

degree relatives is also associated with an increased risk of developing the disease. Siblings of

a child diagnosed with Wilms Tumour have a slightly higher risk of developing the tumour

compared to the general population (10). The risk is further increased if the affected sibling has

bilateral or multifocal tumours (9). Close monitoring and surveillance are recommended for

siblings of affected individuals; as early detection can significantly improve treatment

outcomes.

Genetic counselling and testing play a crucial role in families with a history of Wilms Tumour.

Identification of individuals carrying specific genetic mutations associated with Wilms Tumour

10
allows for appropriate surveillance and early detection. Furthermore, genetic testing can

provide valuable information for family planning and counselling regarding the risk of Wilms

Tumour in future generations.

III. Radiological Findings

A. Imaging Modalities Used:

Various imaging modalities are utilized in the diagnosis and management of Wilms Tumour,

including ultrasound, computed tomography (CT) scan, and magnetic resonance imaging

(MRI). Each modality provides valuable information about the location, size, characteristics,

and involvement of surrounding structures.

Ultrasound is often the initial imaging modality used to evaluate an abdominal mass in children.

It is non-invasive, readily available, and does not involve ionizing radiation, making it ideal

for paediatric patients. Ultrasound helps determine the size, location, and overall characteristics

of the tumour, such as its solid or cystic nature (3). Additionally, Doppler ultrasound can assess

the blood flow within the tumour and surrounding structures, aiding in the evaluation of

vascular involvement.

CT scan is another commonly employed imaging technique in Wilms Tumour. It provides

detailed cross-sectional images of the abdomen and can accurately determine the extent of the

tumour. CT scans offer precise information about the size and location of the tumour, as well

as its relationship to adjacent structures, including the renal vein and lymph nodes (11).

Intravenous contrast administration during CT scanning enhances the visualization of vascular

structures and aids in the detection of metastatic disease.

11
MRI is particularly useful in cases where further characterization of the tumour is required. It

provides detailed anatomical and soft tissue information, allowing for better assessment of

tumour extension and involvement of surrounding structures. MRI is especially valuable in

evaluating vascular invasion, as it can accurately depict the relationship between the tumour

and the renal vein (12). Moreover, MRI can help differentiate between solid and cystic

components of the tumour, providing important insights for treatment planning.

B. Description of Radiological Findings:

Location and Size of the Tumour:

Radiological imaging allows for precise determination of the location and size of the Wilms

Tumour within the kidney. Typically, the tumour arises from one kidney and is localized,

although bilateral cases do occur. Imaging findings can reveal the specific kidney involved

(right or left) and the precise location of the tumour within the renal parenchyma.

Characteristics of the Tumour:

Radiological studies provide information about the characteristics of the tumour, including

whether it appears solid, cystic, or demonstrates specific features such as calcifications. Solid

components of the tumour typically appear hypoechoic on ultrasound, while cystic areas may

appear anechoic or have internal fluid levels (3). CT scans and MRI can further elucidate the

solid and cystic components, aiding in tumour characterization and treatment planning.

Involvement of Surrounding Structures:

Imaging plays a crucial role in assessing the involvement of surrounding structures by the

Wilms Tumour. The renal vein is commonly evaluated for tumour invasion, which can have

implications for surgical planning and prognosis. Lymph nodes in the vicinity of the kidney

12
are also assessed for potential metastatic spread, as lymph node involvement is an important

factor in staging and treatment decisions.

C. Radiological Staging of the Tumour:

Classification According to the International Society of Paediatric Oncology (SIOP) Staging

System:

The International Society of Paediatric Oncology (SIOP) staging system is widely used for

Wilms Tumour. It categorizes the tumour into different stages based on specific criteria,

including tumour size, involvement of surrounding structures, and presence of distant

metastasis (12).

Description of Staging Criteria and Findings Specific to Each Stage:

The SIOP staging system for Wilms Tumour includes the following stages:

Stage I: The tumour is limited to the kidney and can be completely resected without rupture or

spillage. There is no involvement of the renal sinus, lymph nodes, or distant metastasis.

Stage II: The tumour extends beyond the kidney but can still be completely resected. It may

involve the renal sinus or invade the renal capsule. However, there is no lymph node

involvement or distant metastasis.

Stage III: The tumour extends beyond the kidney and cannot be completely resected. This stage

includes tumours with residual tumour nodules at or near the surgical margins, lymph node

involvement, or tumour rupture during surgery.

Stage IV: The tumour has spread to distant sites, such as the lungs, liver, bones, or other distant

organs. This stage may involve lymph node involvement as well.

13
Stage V: This stage includes bilateral renal involvement at the time of diagnosis, regardless of

other factors.

Radiological staging is based on the findings from imaging modalities such as CT scans, MRI,

and chest X-rays to detect distant metastasis. These findings guide treatment decisions and help

determine the prognosis for the patient.

IV. Differential Diagnosis

A. Discussion of other conditions that may present with similar radiological findings:

When evaluating a renal mass in a paediatric patient, it is important to consider other conditions

that may present with similar radiological findings to Wilms Tumour. These differential

diagnoses include:

Nephroblastomatosis: Nephroblastomatosis refers to the presence of multiple small nodules

within the kidney, which can mimic the appearance of Wilms Tumour on imaging studies.

However, unlike Wilms Tumour, nephroblastomatosis is not a neoplastic process but

represents a precursor lesion or persistence of embryonal tissue (13). Nephroblastomatosis

typically demonstrates multiple small, well-defined nodules throughout the kidney, and careful

evaluation is necessary to differentiate it from Wilms Tumour.

Renal Cell Carcinoma: Renal cell carcinoma (RCC) is a malignant tumour that can occur in

paediatric patients, although it is more commonly seen in adults. On imaging, RCC often

presents as a solid mass with varying enhancement patterns, including hypervascular,

hypovascular, or heterogeneous enhancement (14). The presence of calcifications, infiltrative

growth, or invasion into the renal vein suggests RCC rather than Wilms Tumour.

14
Multilocular Cystic Nephroma: Multilocular cystic nephroma is a rare benign renal tumour that

predominantly affects children. It typically presents as a multilocular cystic mass with well-

defined septations on imaging studies (15). Unlike Wilms Tumour, multilocular cystic

nephroma does not show solid components or enhancement.

B. Differentiating features of Wilms Tumour from other differential diagnoses:

Age of Presentation: Wilms Tumour is primarily a paediatric malignancy, with most cases

diagnosed before the age of 5 years. In contrast, renal cell carcinoma and multilocular cystic

nephroma are rare in children and typically occur in older individuals.

Location and Size of the Tumour: Wilms Tumour typically arises from one kidney, whereas

renal cell carcinoma can affect either kidney and is more frequently unilateral. Multilocular

cystic nephroma is usually unilateral as well. Additionally, the size of the tumour can provide

important clues, as Wilms Tumour is often larger than other renal masses seen in children.

Solid vs. Cystic Nature: Wilms Tumour can have a heterogeneous appearance on imaging, with

both solid and cystic components. However, the presence of significant solid components

favours Wilms Tumour, while predominantly cystic lesions suggest other differential

diagnoses, such as multilocular cystic nephroma.

Vascular Involvement: Invasion of the renal vein is more commonly seen in Wilms Tumour

than in other renal masses. The presence of tumour thrombus within the renal vein or inferior

vena cava strongly suggests Wilms Tumour.

Metastasis: The presence of distant metastasis, particularly to the lungs or liver, is more

commonly associated with Wilms Tumour than with other renal masses in children.

Accurate differentiation between Wilms Tumour and other differential diagnoses is crucial for

appropriate management and treatment planning.

15
V. Treatment and Management

A. Overview of Treatment Options for Wilms Tumour:

The management of Wilms Tumour typically involves a multimodal approach, including

surgery, chemotherapy, and radiation therapy in certain cases. The specific treatment plan

depends on various factors such as tumour stage, histology, and patient's age. The primary

goals of treatment are to achieve complete tumour resection, minimize the risk of recurrence,

and preserve renal function.

B. Role of Radiology in Treatment Planning and Monitoring:

Radiology plays a crucial role in the treatment planning and monitoring of Wilms Tumour.

Imaging modalities such as ultrasound, CT scan, and MRI help in assessing tumour

characteristics, determining the extent of disease, and identifying any associated vascular or

lymphatic involvement. These findings aid in surgical planning by guiding the surgeon to

achieve complete tumour resection while preserving as much healthy renal tissue as possible.

During the course of treatment, radiological imaging is used to monitor treatment response and

assess for any signs of disease recurrence or metastasis. Follow-up imaging allows for early

detection of any residual or recurrent disease, guiding further treatment decisions and ensuring

timely intervention.

C. Description of Surgical Interventions, Chemotherapy, and Radiation Therapy:

Surgical Interventions: Surgery is the primary treatment for Wilms Tumour and aims to achieve

complete tumour resection while preserving renal function. The extent of surgery depends on

the tumour stage and may involve partial nephrectomy (removal of the tumour while preserving

16
a portion of the kidney) or radical nephrectomy (complete removal of the affected kidney).

Lymph node sampling or dissection may also be performed to evaluate for any lymph node

involvement (16).

Chemotherapy: Chemotherapy plays a critical role in the management of Wilms Tumour. It is

typically administered both before and after surgery to reduce tumour size, control

micrometastatic disease, and improve overall survival rates. Commonly used chemotherapy

agents include vincristine, dactinomycin, and doxorubicin. The specific chemotherapy regimen

depends on the risk stratification of the tumour (16).

Radiation Therapy: Radiation therapy is utilized in specific cases of Wilms Tumour, primarily

when there is a high risk of local recurrence or in the presence of residual disease after surgery.

It is typically administered postoperatively and may involve external beam radiation targeting

the tumour bed and nearby lymph nodes. The dose and field of radiation depend on the

individual patient's risk factors and the treatment team's recommendations (16).

D. Follow-up Imaging and Surveillance Recommendations:

After completion of treatment, regular follow-up imaging is essential to monitor for disease

recurrence or late effects of treatment. The frequency and duration of follow-up imaging

depend on the individual patient's risk factors, treatment response, and specific protocols

followed by the treating institution.

Commonly employed imaging modalities for surveillance include abdominal ultrasound, CT

scan, or MRI. These imaging studies help evaluate the surgical site, assess for any residual or

recurrent disease, and monitor the health of the contralateral kidney.

17
In addition to imaging, close clinical monitoring, including physical examination and

laboratory tests, is an integral part of surveillance to detect any signs or symptoms of disease

recurrence or treatment-related complications.

VI. Prognosis and Outcome

A. Discussion of Prognosis Factors and Survival Rates:

The prognosis of Wilms Tumour depends on various factors, including tumour stage, histology,

age at diagnosis, and response to treatment. Overall, the prognosis for Wilms Tumour is

favourable, with high survival rates achieved through multimodal treatment approaches.

Tumour Stage: The stage of Wilms Tumour is a significant prognostic factor. The International

Society of Paediatric Oncology (SIOP) staging system is commonly used to stratify patients

into different risk groups. Generally, patients with lower stage tumours have better prognoses

and higher survival rates compared to those with advanced stages (17).

Histology: Histological subtype also influences prognosis. Favourable histology Wilms

Tumours, which represent the majority of cases, have excellent survival rates. However,

unfavourable histology subtypes, including diffuse anaplastic Wilms Tumour, are associated

with a higher risk of recurrence and poorer outcomes (17).

Age at Diagnosis: Age at diagnosis is an important prognostic factor. Younger children,

particularly those diagnosed before the age of 2 years, tend to have better outcomes than older

children. This age-related difference in prognosis may be attributed to differences in tumour

biology and response to treatment (17).

Survival rates for Wilms Tumour have significantly improved over the years due to

advancements in treatment protocols. The overall 5-year survival rate for Wilms Tumour is

18
approximately 90%, with higher rates observed in favourable histology and lower stage

tumours (18).

B. Long-term Outcomes and Potential Complications:

While the majority of patients with Wilms Tumour achieve long-term survival, it is important

to consider potential long-term outcomes and complications that may arise as a result of the

disease or its treatment.

Renal Function: Since Wilms Tumour primarily affects the kidneys, there is a risk of impaired

renal function following treatment. Depending on the extent of surgical resection and any

radiation therapy administered, some patients may experience long-term renal dysfunction or

require renal replacement therapy (18).

Cardiopulmonary Complications: Certain treatment modalities, such as radiation therapy, may

increase the risk of cardiopulmonary complications, including cardiac dysfunction and

pulmonary fibrosis. Long-term follow-up and monitoring of cardiac and pulmonary function

are important for timely detection and management of these potential complications (17).

Second Malignancies: Survivors of Wilms Tumour are at a slightly increased risk of developing

second malignancies later in life. This risk is influenced by various factors, including genetic

predisposition, previous radiation exposure, and certain chemotherapy agents. Regular

surveillance and screening for second malignancies are essential in long-term survivorship care

(17).

C. Impact of Radiological Findings on Prognosis and Treatment Decisions:

Radiological findings play a crucial role in assessing the extent of disease and guiding

treatment decisions for Wilms Tumour. They provide valuable information about tumour

19
characteristics, involvement of surrounding structures, and the presence of metastasis. These

findings help determine the tumour stage and guide the selection of appropriate treatment

modalities.

The radiological evaluation of Wilms Tumour also aids in prognostication by identifying high-

risk features, such as tumour rupture, vascular invasion, or lymph node involvement. These

factors may influence treatment decisions and intensification of therapy in order to optimize

outcomes.

Furthermore, radiological imaging during follow-up allows for early detection of disease

recurrence or treatment-related complications, enabling timely intervention and potentially

improving overall prognosis.

VII. Conclusion

A. Recap of Key Points Discussed in the Case Write-Up:

In this case write-up, we have explored various aspects of Wilms Tumour, a common paediatric

renal malignancy. The key points discussed include the overview of Wilms Tumour, its

epidemiology, presenting symptoms, diagnostic imaging modalities, radiological findings,

staging, treatment options, prognosis, and long-term outcomes. Additionally, we examined the

importance of radiology in the diagnosis, staging, and management of Wilms Tumour.

B. Importance of Radiology in the Diagnosis, Staging, and Management of Wilms Tumour:

Radiology plays a critical role in the diagnosis, staging, and management of Wilms Tumour.

Imaging modalities such as ultrasound, CT scan, and MRI provide essential information about

tumour location, size, characteristics, and involvement of surrounding structures. Radiological

findings help in the accurate diagnosis, staging, and risk stratification of Wilms Tumour,

thereby guiding treatment decisions.

20
Radiology also facilitates surgical planning by providing detailed anatomical information to

achieve complete tumour resection while preserving renal function. Furthermore, radiological

imaging is instrumental in monitoring treatment response, detecting disease recurrence, and

assessing long-term outcomes.

C. Future Directions and Potential Advancements in Radiological Techniques for Wilms

Tumour Evaluation:

The field of radiology is constantly evolving, and future advancements hold promise for further

improving the evaluation of Wilms Tumour. Some potential future directions and

advancements may include:

Advanced Imaging Techniques: Continued advancements in imaging techniques, such as

functional MRI, diffusion-weighted imaging, and molecular imaging, may provide additional

information about tumour biology, microstructural changes, and molecular markers, aiding in

precise diagnosis and treatment monitoring.

Image-Guided Interventions: Minimally invasive image-guided interventions, such as

percutaneous biopsies and ablative techniques, may play a role in obtaining tissue samples for

histopathological confirmation and providing targeted treatment options for localized disease.

Artificial Intelligence (AI) and Machine Learning: Integration of AI and machine learning

algorithms into radiological practice has the potential to enhance the accuracy and efficiency

of image interpretation, allowing for automated tumour segmentation, risk stratification, and

prediction of treatment response.

Personalized Medicine: With advances in genomics and molecular profiling, radiogenomics

may emerge as an area of interest, correlating radiological features with specific genetic

alterations, enabling personalized treatment strategies and prognostication.

21
It is important for radiologists to stay updated with the latest research and technological

advancements to optimize their role in the comprehensive management of Wilms Tumour.

In conclusion, Wilms Tumour is a paediatric renal malignancy where radiology plays a pivotal

role in its diagnosis, staging, and management. With the continued advancements in

radiological techniques and integration of innovative approaches, the field holds great potential

for improving outcomes and tailoring treatment strategies for patients with Wilms Tumour.

22
REFERENCES

1. Dome JS, Huff V. Wilms Tumor Overview. 2003 [cited 2023 Jun 2]; Available from:

www.genetests.org

2. Huff V. Wilms tumor genetics. Am J Med Genet. 1998;79(4):260–7.

3. Brisse HJ, Smets AM, Kaste SC, Owens CM. Imaging in unilateral Wilms tumour.

2008;18–29.

4. Breslow N, Olshan A, Beckwith JB, Green DM. Epidemiology of Wilms tumor. Med

Pediatr Oncol. 1993;21(3):172–81.

5. Meier CM, Furtwängler R, Mergen M, Welter N, Melchior P, Schenk JP, et al. Impact

of Time to Surgery on Outcome in Wilms Tumor Treated with Preoperative

Chemotherapy. Cancers (Basel). 2023;15(5).

6. Nakata K, Colombet M, Stiller CA, Pritchard-Jones K, Steliarova-Foucher E.

Incidence of childhood renal tumours: An international population-based study. Vol.

147, International Journal of Cancer. 2020. 3313–3327 p.

7. Charlton J, Irtan S, Bergeron C, Pritchard-Jones K. Bilateral Wilms tumour: a review

of clinical and molecular features. Expert Rev Mol Med. 2017;19.

8. Ng A, Griffiths A, Cole T, Davison V, Griffiths M, Larkin S, et al. Congenital

abnormalities and clinical features associated with Wilms’ tumour: A comprehensive

study from a centre serving a large population. Eur J Cancer. 2007;43(9):1422–9.

9. Ruteshouser EC, Huff V. Familial Wilms tumor. Am J Med Genet - Semin Med Genet.

2004;129 C(1):29–34.

10. Felgenhauer JL, Barce JM, Benson RL, Nan B, Olson JM, Breslow NE. No excess of

23
early onset cancer in family members of Wilms tumor patients. Cancer.

2001;92(6):1606–12.

11. Lowe LH, Isuani BH, Heller RM, Stein SM, Johnson JE, Navarro OM, et al. Pediatric

renal masses: Wilms tumor and beyond. Radiographics. 2000;20(6):1585–603.

12. McDonald K, Duffy P, Chowdhury T, McHugh K. Added value of abdominal cross-

sectional imaging (CT or MRI) in staging of Wilms’ tumours. Clin Radiol [Internet].

2013;68(1):16–20. Available from: http://dx.doi.org/10.1016/j.crad.2012.05.006

13. WALLIS CJ. Embryology and Development. Pract Biol. 1966;357–78.

14. Pavlica P, Derchi L, Martorana G, Brunocilla E, Bertaccini A, Manferrari F, et al.

Renal Cell Carcinoma Imaging. Eur Urol Suppl. 2006;5(8):580–92.

15. Silver IMF, Boag AH, Soboleski DA. Multilocular cystic renal Tumor: Cystic

nephroma. Radiographics. 2008;28(4):1221–7.

16. Szychot E, Apps J, Pritchard-Jones K. Wilms’ tumor: biology, diagnosis and

treatment. Transl Pediatr [Internet]. 2014;3(1):12–24. Available from:

http://www.ncbi.nlm.nih.gov/pubmed/26835318%0Ahttp://www.pubmedcentral.nih.g

ov/articlerender.fcgi?artid=PMC4728859

17. Kaste SC, Dome JS, Babyn PS, Graf NM, Grundy P, Godzinski J, et al. Wilms

tumour: Prognostic factors, staging, therapy and late effects. Pediatr Radiol.

2008;38(1):2–17.

18. Spreafico F, Fernandez C V., Brok J, Nakata K, Vujanic G, Geller JI, et al. Wilms

tumour. Nat Rev Dis Prim [Internet]. 2021;7(1).

24

You might also like