Wilms Tumor — MCQs

10 questions

The ideal timing of radiotherapy for Wilms' tumor after surgery is -

A mother notices a swelling in the abdomen of her 3-year-old child while bathing him. He had a history of hematuria two weeks back, which resolved spontaneously. On examination, a right-sided reniform ballotable mass was found. What is the most appropriate initial investigative approach?

A 3 week old child presents with an abdominal mass. What is the most common congenital renal cystic abnormality causing this presentation?

Wilms' tumor: What is the most common presenting symptom?

What is the most common malignant tumor of the kidney in children?

Most common renal tumor in infants?

Which of the following malignant diseases of children has the best prognosis -

A 2 year old child is seen on a routine visit in the pediatric clinic. Abdominal examination demonstrates a palpable, non-tender mass on the left side of the abdomen. The mother had no idea the mass was present and the pediatrician did not note the presence of the mass at the child's 18-month's visit for immunisation. Physical examination is otherwise unremarkable. If a CT guided biopsy of the mass were performed, which of the following histological patterns would be most suggestive of the likely diagnosis?

A 1-year-old child presented with a swelling in the left flank with episodes of flushing, diarrhea, sweating and bone pain. The diagnosis is -

Q10

Which of the following statements regarding Wilms tumor is false?

Wilms Tumor Indian Medical PG Practice Questions and MCQs

Question 1: The ideal timing of radiotherapy for Wilms' tumor after surgery is -

A. Any time after surgery
B. Within 2 months
C. Within 2 weeks
D. Within 10 days (Correct Answer)

Explanation: ***Within 10 days*** - Postoperative radiotherapy for Wilms' tumor should ideally be initiated within **10 days** of surgery to optimize outcomes and minimize the risk of recurrence. - This **early initiation** helps to control potential microscopic residual disease and is associated with improved outcomes. - **Children's Oncology Group (COG)** protocols recommend starting radiotherapy within **10-14 days**, with 10 days being the preferred timeline. *Any time after surgery* - Delaying radiotherapy beyond the recommended 10-14 day window can significantly increase the risk of **disease progression** and **local recurrence**. - The efficacy of radiotherapy is highest when administered promptly after surgical resection. *Within 2 months* - A delay of two months is considered **suboptimal** and significantly exceeds the recommended timeframe. - This extended delay could lead to progression of any residual tumor cells and compromise treatment outcomes. *Within 2 weeks* - While 2 weeks (14 days) falls within the acceptable **10-14 day window** recommended by most protocols, **10 days is preferred** as the most optimal timing. - The distinction is that initiating within 10 days is considered **ideal**, whereas the full 14-day period represents the outer limit of the acceptable timeframe for standard cases.

Question 2: A mother notices a swelling in the abdomen of her 3-year-old child while bathing him. He had a history of hematuria two weeks back, which resolved spontaneously. On examination, a right-sided reniform ballotable mass was found. What is the most appropriate initial investigative approach?

A. Ultrasound, CT scan with contrast, CBC, and Bone marrow biopsy
B. Ultrasound, CT scan with contrast, and CBC (Correct Answer)
C. Ultrasound and CT scan with contrast
D. CT scan with contrast and Bone marrow biopsy

Explanation: ***Ultrasound, CT scan with contrast, and CBC*** - This combination allows for comprehensive **assessment of the kidney mass**, its extent, and helps differentiate it from other abdominal masses, while a CBC checks for paraneoplastic anemia or polycythemia. - **Ultrasound** is typically the initial imaging modality due to its non-invasiveness and ability to characterize the mass as solid or cystic, followed by a **CT scan with contrast** for detailed anatomical information and staging. *Ultrasound, CT scan with contrast, CBC, and Bone marrow biopsy* - While ultrasound, CT scan with contrast, and CBC are appropriate, a **bone marrow biopsy** is usually reserved for suspected metastasis or specific types of tumors like neuroblastoma, not as an initial step for a primary renal mass presentation in a 3-year-old child. - The initial focus is on characterizing the renal mass and systemic workup, and bone marrow biopsy would be considered later if there's evidence or high suspicion of metastatic disease to the bone marrow. *Ultrasound and CT scan with contrast* - While these imaging studies are crucial for diagnosing and staging the renal mass, neglecting a **complete blood count (CBC)** would be an oversight. - A CBC can reveal important information such as anemia (common with renal tumors), thrombocytosis, or other hematological abnormalities that might influence treatment decisions or indicate prognosis. *CT scan with contrast and Bone marrow biopsy* - Relying solely on a **CT scan with contrast** as the initial imaging can be less efficient than starting with ultrasound, especially in children, and lacks the preliminary information provided by a CBC. - A **bone marrow biopsy** is not typically part of the initial workup for a suspected Wilms' tumor unless there is specific concern for bone marrow involvement or to rule out a neuroblastoma.

Question 3: A 3 week old child presents with an abdominal mass. What is the most common congenital renal cystic abnormality causing this presentation?

A. Distended bladder
B. Wilms tumor
C. Neuroblastoma
D. Multicystic dysplastic kidney (Correct Answer)

Explanation: ***Multicystic dysplastic kidney*** - This is the **most common cause of an abdominal mass detected in the neonatal period** due to its congenital nature. - It results from abnormal renal development in utero, leading to multiple non-communicating cysts and virtually no functioning renal tissue. *Distended bladder* - While a distended bladder can present as an abdominal mass in an infant, especially with **posterior urethral valves**, it is typically symptomatic with difficulty urinating or urinary tract infections. - It is not the most common overall cause of an abdominal mass at this age. *Wilms tumor* - **Wilms tumor**, originating from the kidney, is the **most common renal malignancy in children**, but it typically presents in toddlers (2-5 years of age) rather than at 3 weeks old. - Presentation at 3 weeks would be exceptionally rare, as it is an embryonal tumor that grows over time. *Neuroblastoma* - **Neuroblastoma** is a common extracranial solid tumor in infancy, often originating in the adrenal gland or sympathetic chain. - However, it is generally outranked by multicystic dysplastic kidney as the *most common* cause of an abdominal mass this early in life, and it can present with various systemic symptoms depending on tumor location and metastasis.

Question 4: Wilms' tumor: What is the most common presenting symptom?

A. Haematuria
B. Abdominal pain
C. Headache
D. Asymptomatic abdominal mass (Correct Answer)

Explanation: ***Asymptomatic abdominal mass*** - Wilms' tumor, or **nephroblastoma**, commonly presents as an **abdominal mass** discovered incidentally by a parent or during a routine physical examination. - This mass is typically firm, smooth, and located in the **flank**, and is usually not painful in its early stages. *Haematuria* - While haematuria (blood in the urine) can occur in Wilms' tumor, it is a **less common** initial symptom compared to an abdominal mass. - Its presence often suggests a more advanced stage or involvement of the **pelvic collecting system**. *Abdominal pain* - Abdominal pain may be present in children with Wilms' tumor, but it is **not the most common initial presenting symptom**. - When present, it can indicate rapid tumor growth, hemorrhage, or obstruction, and is usually a **secondary complaint** to the palpable mass. *Headache* - Headache is **not a typical presenting symptom** of Wilms' tumor. - If a headache were present, it would more likely suggest **metastatic disease** involving the brain, which is rare at initial presentation, or an unrelated issue.

Question 5: What is the most common malignant tumor of the kidney in children?

A. Wilms tumor (Correct Answer)
B. Clear cell sarcoma of the kidney
C. Rhabdoid tumor of the kidney
D. Neuroblastoma

Explanation: ***Wilms tumor*** - Wilms tumor, or **nephroblastoma**, is the most common type of kidney tumor in children, typically presenting with an **abdominal mass** [2]. - Most patients are diagnosed between the ages of **2 and 5** [3], and it is often associated with syndromic conditions like **WAGR syndrome** [1]. *PCKD* - **Polycystic Kidney Disease (PCKD)** is a genetic disorder that leads to kidney enlargement but is not classified as a tumor. - While it can lead to complications like hypertension, it does not typically present as a **neoplastic mass** in children. *Angioliposarcoma* - This is a rare tumor that occurs primarily in adults, characterized by **fatty tissue** and vascular infiltrations, making it an uncommon kidney tumor in children. - Typically, renal tumors in children are **Wilms tumor** rather than **sarcomas** like angioliposarcoma. *Neuroblastoma* - Neuroblastoma is an **extrarenal tumor** that commonly arises from the adrenal glands or sympathetic nervous tissue [3], rather than directly from the kidney. - It presents with **abdominal masses** but is distinct from kidney tumors, which makes it less relevant in the context of renal tumors in children. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 487-488. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 488-490. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-484.

Question 6: Most common renal tumor in infants?

A. Neuroblastoma
B. Rhabdoid tumor
C. Congenital mesoblastic nephroma (Correct Answer)
D. Nephroblastoma

Explanation: ***Congenital mesoblastic nephroma*** - This is the **most common renal tumor in infants less than 3 months of age**, often detected antenatally or presenting shortly after birth. - It is a **benign tumor** originating from mesenchymal tissue, frequently seen as a large, solitary renal mass. *Neuroblastoma* - While common in infants and young children, **neuroblastoma** usually originates from **neural crest cells** in the adrenal gland or sympathetic ganglia, not primarily the kidney itself. - Though it can present as an abdominal mass, it is not primarily a renal tumor. *Rhabdoid tumor* - This is a rare, highly aggressive renal tumor that typically occurs in infants and young children, but it is **less common** than congenital mesoblastic nephroma or Wilms tumor. - Characterized by mutations in the **SMARCB1 gene** and a very poor prognosis. *Nephroblastoma* - Also known as **Wilms tumor**, nephroblastoma is the **most common primary malignant renal tumor** in children, but it typically peaks between **2 and 5 years of age**. - While it can occur in infants, congenital mesoblastic nephroma is more prevalent in the *infant* age group, especially in the first few months of life.

Question 7: Which of the following malignant diseases of children has the best prognosis -

A. Primitive neuroectodermal tumor
B. Wilms tumor (Correct Answer)
C. Neuroblastoma
D. Rhabdomyosarcoma

Explanation: ***Wilms tumor*** - **Wilms tumor**, also known as **nephroblastoma**, has one of the best prognoses among childhood malignancies, with a **survival rate exceeding 90%** for localized disease. - Its high curability is attributed to its **chemosensitivity**, surgical resectability, and advancements in multimodal therapy. *Primitive neuroectodermal tumor* - **Primitive neuroectodermal tumors (PNETs)** are highly aggressive and often associated with a **poor prognosis**, particularly when they are large or metastatic. - These tumors arise from neuroectoderm and can occur in the central nervous system (e.g., medulloblastoma) or peripherally, making treatment challenging due to their infiltrative nature. *Neuroblastoma* - **Neuroblastoma** is a common extracranial solid tumor in children, originating from neural crest cells, and its prognosis varies widely based on age, stage, and biological features, but it often has a **guarded prognosis**, especially in older children or with unfavorable genetic markers. - High-risk neuroblastomas frequently exhibit **amplification of the MYCN oncogene** and often require intensive, multi-modal treatment, still leading to significant mortality. *Rhabdomyosarcoma* - **Rhabdomyosarcoma** is a malignant tumor of mesenchymal origin with skeletal muscle differentiation, and its prognosis depends heavily on site, histology (e.g., alveolar vs. embryonal), and resectability, with **overall survival rates lower than Wilms tumor**, especially for unfavorable subtypes or metastatic disease. - Aggressive treatment including chemotherapy, radiation, and surgery is often necessary, but recurrence rates can be high.

Question 8: A 2 year old child is seen on a routine visit in the pediatric clinic. Abdominal examination demonstrates a palpable, non-tender mass on the left side of the abdomen. The mother had no idea the mass was present and the pediatrician did not note the presence of the mass at the child's 18-month's visit for immunisation. Physical examination is otherwise unremarkable. If a CT guided biopsy of the mass were performed, which of the following histological patterns would be most suggestive of the likely diagnosis?

A. Triphasic pattern with tubule formation, spindle cells, and blastemal elements (Correct Answer)
B. Invasive papillary lesions with delicate connective tissue stalk covered with epithelium resembling that lining the bladder.
C. Cords of clear cells with rounded or polygonal shape and abundant clear cytoplasm
D. Small dark cells embedded in a finely fibrillar matrix with formation of numerous rosettes

Explanation: ***Triphasic pattern with tubule formation, spindle cells, and blastemal elements*** - The presence of a **palpable, non-tender abdominal mass** in a 2-year-old, previously undetected, is highly suggestive of **Wilms tumor (nephroblastoma)**. - Wilms tumor characteristically exhibits a **triphasic histological pattern** consisting of **blastemal**, **stromal (spindle cells)**, and **epithelial (tubule formation)** elements. *Invasive papillary lesions with delicate connective tissue stalk covered with epithelium resembling that lining the bladder.* - This description typically refers to **papillary urothelial carcinoma**, a tumor of the **bladder or urinary tract**, which is rarely seen in young children and presents with hematuria, not primarily an abdominal mass. - The absence of urinary symptoms and the age of the patient make this an unlikely diagnosis for an abdominal mass. *Cords of clear cells with rounded or polygonal shape and abundant clear cytoplasm* - This histological pattern is characteristic of **clear cell renal carcinoma**, an adult kidney malignancy, not a common pediatric abdominal tumor. - While clear cell renal cell carcinoma can present as an abdominal mass, it is exceptionally rare in a 2-year-old child. *Small dark cells embedded in a finely fibrillar matrix with formation of numerous rosettes* - This description is highly suggestive of **neuroblastoma**, a small round blue cell tumor that arises from neural crest cells (e.g., adrenal gland or sympathetic chain). - While **neuroblastoma** is the second most common abdominal mass in children after Wilms tumor, it typically presents with different histological features and may be associated with systemic symptoms like weight loss, fever, or bone pain. - The **triphasic pattern** described in option A is more specific for Wilms tumor.

Question 9: A 1-year-old child presented with a swelling in the left flank with episodes of flushing, diarrhea, sweating and bone pain. The diagnosis is -

A. Wilms tumor
B. Pheochromocytoma
C. Neuroblastoma (Correct Answer)
D. Medulloblastoma

Explanation: ***Neuroblastoma*** - The combination of a **flank mass** in a 1-year-old child, along with paraneoplastic symptoms like **flushing, diarrhea, and sweating** (due to catecholamine secretion), and **bone pain** (indicating metastatic disease), is highly characteristic of neuroblastoma. - Neuroblastoma is a common extracranial solid tumor of childhood arising from **neural crest cells**, often presenting with abdominal mass and systemic symptoms. *Wilms tumor* - While Wilms tumor also presents as a **flank mass** in young children, it typically does not cause the systemic symptoms of flushing, diarrhea, or sweating, nor significant bone pain from metastasis. - Patients with Wilms tumor often present with **asymptomatic abdominal mass** detected incidentally. *Pheochromocytoma* - **Pheochromocytoma** can cause symptoms like flushing, sweating, and episodic hypertension due to catecholamine release, but it is **rare in children** and typically presents as an adrenal mass, not a flank mass with bone pain. - It usually presents with **hypertension crises**, which are not mentioned here. *Medulloblastoma* - **Medulloblastoma** is a **brain tumor** and would present with neurological symptoms such as headache, vomiting, and ataxia, not a flank mass or systemic symptoms like flushing, diarrhea, and sweating. - It is a **malignant tumor** of the cerebellum and does not cause peripheral masses or bone metastases in this manner.

Question 10: Which of the following statements regarding Wilms tumor is false?

A. Most commonly seen < 5 years of age
B. Most patients recover well with chemotherapy and radiotherapy.
C. Presents as a renal mass.
D. Lung metastasis is rare and occurs late (Correct Answer)

Explanation: ***Lung metastasis is rare and occurs late*** - This statement is **false** primarily because when lung metastases occur, they often present **early (at initial diagnosis)** rather than late. - Approximately **10-15% of patients** have pulmonary metastases at presentation, making the "occurs late" portion incorrect. - While lungs are the **most common site** for distant metastases when they do occur, the overall incidence is relatively low, so calling it "rare" is debatable but the "late" timing is definitively wrong. - The statement's claim about late occurrence contradicts clinical reality where metastatic disease, when present, is typically identified at diagnosis or within the first 2 years. *Most commonly seen < 5 years of age* - This statement is **true** as Wilms tumor has **peak incidence at ages 2-3 years**, with the majority of cases diagnosed before age **5 years**. - It is the most common primary **renal malignancy in children**, predominantly affecting young children. *Most patients recover well with chemotherapy and radiotherapy.* - This statement is **true** as Wilms tumor has an **excellent prognosis** with **overall survival rates exceeding 90%** using multimodal therapy. - Treatment includes **surgery (nephrectomy), chemotherapy**, and **radiotherapy** (in selected cases), resulting in high cure rates even in advanced stages. *Presents as a renal mass.* - This statement is **true** as the typical presentation is an **asymptomatic abdominal mass** discovered incidentally by parents or during routine examination. - The mass is usually **firm, smooth, and non-tender**, representing an **intrarenal tumor** that may cause abdominal distension.

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