Oncology Practice Questions

Q: All of the statements regarding Wilm's tumor are true EXCEPT:

It typically presents after 5 years of age.. **Explanation:** Wilms tumor (Nephroblastoma) is the most common primary renal malignancy in children. Understanding its clinical profile is crucial for NEET-PG. **1. Why Option A is the correct answer (The False Statement):** Wilms tumor is primarily a disease of early childhood. The median age at diagnosis is **3 to 4 years**. Approximately 80% of cases present before the age of 5. Presenting *after* 5 years of age is uncommon, making this statement false. **2. Analysis of Incorrect Options (True Statements):** * **Option B (Hematuria):** While an asymptomatic abdominal mass is the most common sign, **microscopic hematuria** occurs in about 25–33% of patients due to tumor invasion of the renal pelvis. * **Option C (Abdominal Mass):** The classic presentation is a **smooth, firm, and unilateral abdominal mass** that rarely crosses the midline (unlike Neuroblastoma). It is often discovered incidentally by parents during bathing. * **Option D (Metastasis):** The **lungs** are the most common site of hematogenous metastasis, followed by the liver. **Clinical Pearls for NEET-PG:** * **WAGR Syndrome:** Wilms tumor, Aniridia, Genitourinary anomalies, and intellectual disability (formerly Retardation). Associated with **WT1 gene** deletion on Chromosome 11p13. * **Beckwith-Wiedemann Syndrome:** Characterized by macroglossia, omphalocele, and hemihypertrophy; associated with **WT2 gene** on Chromosome 11p15. * **Management:** Treatment usually involves a combination of nephrectomy and chemotherapy. It has an excellent prognosis with survival rates exceeding 90% for localized disease. * **Rule of Thumb:** If a renal mass crosses the midline and shows calcification on X-ray, think **Neuroblastoma**; if it is smooth and confined to one side, think **Wilms tumor**.

Q: What is the most common leukemia in children with Down syndrome less than 3 years old?

Acute myeloid leukemia M7 (AML-M7). Children with Down syndrome (Trisomy 21) have a significantly increased risk (10–20 fold) of developing leukemia. The specific type of leukemia is highly dependent on the child's age. **Explanation of the Correct Answer:** In children with Down syndrome **under the age of 3 years**, the most common leukemia is **Acute Myeloid Leukemia (AML)**, specifically the **M7 subtype (Acute Megakaryoblastic Leukemia)**. This is often preceded by a unique condition called Transient Myeloproliferative Disorder (TMD). The pathogenesis is linked to a somatic mutation in the **GATA1 gene**, which is almost pathognomonic for Down syndrome-related megakaryocytic proliferation. Interestingly, children with Down syndrome and AML-M7 have a much better prognosis and higher sensitivity to chemotherapy (cytarabine) compared to non-Down syndrome children. **Analysis of Incorrect Options:** * **Option A (ALL):** While ALL is the most common leukemia in the general pediatric population and is also more common in Down syndrome patients **overall**, it typically presents in children **older than 3 years**. * **Option B (AML-M5):** Acute Monocytic Leukemia is a subtype of AML but is not specifically associated with the unique genetic predisposition of Down syndrome. * **Option C (Hodgkin Lymphoma):** This is a solid tumor of the lymphoid system. While Down syndrome increases the risk of leukemia, it does not significantly increase the risk of Hodgkin lymphoma. **High-Yield Clinical Pearls for NEET-PG:** * **Age 3 years:** ALL is most common. * **Genetic Marker:** GATA1 mutation (specific to DS-AML and TMD). * **Transient Myeloproliferative Disorder (TMD):** Occurs in newborns with Down syndrome; usually resolves spontaneously but predisposes to AML-M7 later. * **Prognosis:** DS-AML has a superior cure rate compared to sporadic AML.

Q: A child presents with proptosis, multiple skeletal limb secondaries, and sutural separation. What is the most likely diagnosis?

Neuroblastoma. **Explanation:** The correct diagnosis is **Neuroblastoma**. In the pediatric population, Neuroblastoma is the most common extracranial solid tumor and a frequent cause of **metastatic orbital disease**. **1. Why Neuroblastoma is correct:** Neuroblastoma (specifically Stage IV) characteristically metastasizes to the bones and the orbit. The classic presentation includes: * **Proptosis:** Rapidly progressing, often bilateral, due to orbital wall metastases. * **Ecchymosis:** Often referred to as "Raccoon eyes" (periorbital ecchymosis). * **Skeletal Involvement:** Metastases to long bones and the skull. * **Sutural Separation:** This occurs due to increased intracranial pressure or direct infiltration of the cranial sutures by the tumor cells, a hallmark finding in metastatic neuroblastoma. **2. Why other options are incorrect:** * **Medulloblastoma:** This is a primary CNS tumor of the posterior fossa. While it can cause papilledema and signs of increased intracranial pressure, it typically does not present with proptosis or systemic skeletal limb secondaries. * **Retinoblastoma:** While it is the most common primary intraocular tumor in children, it usually presents with **leukocoria** (white pupillary reflex) or strabismus. Metastasis to the orbit can cause proptosis, but it does not typically cause widespread limb secondaries and sutural separation as its primary presentation. **Clinical Pearls for NEET-PG:** * **Most common primary orbital malignancy in children:** Rhabdomyosarcoma. * **Most common metastatic orbital tumor in children:** Neuroblastoma. * **Diagnostic Marker:** Elevated urinary catecholamines (VMA and HVA). * **Opsoclonus-Myoclonus Syndrome:** A paraneoplastic syndrome associated with Neuroblastoma ("dancing eyes, dancing feet").

Q: All of the following are conditions associated with Wilm's tumor, except?

Polycystic kidney. **Explanation:** Wilms tumor (Nephroblastoma) is the most common primary renal malignancy in children. It is frequently associated with specific congenital malformations and overgrowth syndromes due to mutations in the **WT1** (Chromosome 11p13) or **WT2** (11p15) genes. **Why Polycystic Kidney is the Correct Answer:** Polycystic kidney disease (PKD) is a genetic disorder characterized by the growth of numerous cysts in the kidneys. While it can lead to renal failure, it is **not** a recognized syndromic association or a precursor to Wilms tumor. **Analysis of Incorrect Options:** * **Aniridia:** This is the absence of the iris. It is a hallmark component of the **WAGR Syndrome** (Wilms tumor, Aniridia, Genitourinary anomalies, and intellectual disability/Range of developmental delays), caused by a microdeletion at 11p13. * **Gonadal Dysgenesis:** This refers to the atypical development of the gonads. It is a key feature of **Denys-Drash Syndrome**, which consists of a triad of Wilms tumor, pseudohermaphroditism (gonadal dysgenesis), and early-onset nephropathy. * **Hemihypertrophy:** This is the asymmetric overgrowth of one side of the body. It is strongly associated with **Beckwith-Wiedemann Syndrome (BWS)**, an overgrowth disorder (WT2 mutation) that carries a significantly increased risk for Wilms tumor and hepatoblastoma. **High-Yield Clinical Pearls for NEET-PG:** * **Most common presentation:** Asymptomatic abdominal mass (does not cross the midline). * **WAGR Syndrome:** Associated with *WT1* deletion. * **Beckwith-Wiedemann Syndrome:** Features include macroglossia, omphalocele, and hemihypertrophy. * **Screening:** Children with these associated syndromes require serial abdominal ultrasounds every 3 months until age 7 for early detection. * **Staging:** Unlike Neuroblastoma, Wilms tumor staging is based on surgical findings.

Q: All of the following are good prognostic factors for childhood ALL except?

Female sex. In childhood Acute Lymphoblastic Leukemia (ALL), prognosis is determined by age, initial white blood cell (WBC) count, cytogenetics, and response to therapy. **Explanation of the Correct Answer:** **Option A (Female sex)** is the correct answer because, while females historically had a slightly better prognosis than males (due to the absence of testicular relapses), modern risk stratification protocols consider **female sex a neutral factor** rather than a "good prognostic factor." In the context of this question, the other three options are well-established, classic indicators of a favorable outcome, making "Female sex" the least accurate description of a "good prognostic factor" in contemporary oncology. **Explanation of Incorrect Options:** * **B. Hyperdiploidy (>50 chromosomes):** This is one of the strongest indicators of a favorable prognosis. These cells are highly sensitive to methotrexate and apoptosis. * **C. t(12;21) translocation:** Also known as the *TEL-AML1* (ETV6-RUNX1) fusion, this is the most common translocation in childhood ALL and is associated with an excellent long-term cure rate. * **D. Pre-B cell ALL:** This subtype generally carries a better prognosis compared to T-cell ALL or mature B-cell (Burkitt) leukemia. **High-Yield Clinical Pearls for NEET-PG:** * **Age:** 1–9 years is favorable; 10 years is poor. * **WBC Count:** 50,000/µL is poor. * **Cytogenetics (Good):** Hyperdiploidy, t(12;21), Trisomy 4, 10, and 17. * **Cytogenetics (Poor):** Hypodiploidy, t(9;22) [Philadelphia chromosome], t(4;11) [MLL gene rearrangement]. * **CNS Involvement:** Presence of blasts in CSF at diagnosis is a poor prognostic sign.

Q: What is the most important determinant of prognosis in Wilms tumor?

Histology. **Explanation:** In Wilms tumor (Nephroblastoma), **Histology** is the single most important prognostic factor. It is broadly categorized into two groups: **Favorable Histology (FH)** and **Unfavorable Histology (UH)**. The presence of **anaplasia** (diffuse or focal) defines unfavorable histology and is strongly associated with resistance to chemotherapy and a higher risk of relapse, regardless of the stage. **Analysis of Options:** * **Option A (Stage of disease):** While staging (NWTS/SIOP criteria) is crucial for determining the intensity of treatment and predicting outcomes, it is secondary to histology. A Stage I tumor with diffuse anaplasia often has a worse prognosis than a Stage III tumor with favorable histology. * **Option B (Loss of heterozygosity of 1p and 16q):** These are specific molecular markers used for risk stratification. While LOH of 1p and 16q is associated with a poorer prognosis in favorable histology tumors, it is not as globally significant as the microscopic appearance (histology) itself. * **Option D (Age):** While younger age (especially <2 years) generally correlates with better outcomes and lower stages, it is not a primary determinant of the biological behavior of the tumor. **High-Yield Clinical Pearls for NEET-PG:** * **Most common presentation:** Asymptomatic abdominal mass (does not cross the midline). * **Associated Syndromes:** WAGR (WT1), Denys-Drash (WT1), and Beckwith-Wiedemann (WT2). * **Most common site of metastasis:** Lungs (Cannon-ball metastasis). * **Triphasic Histology:** Classic Wilms tumor consists of three elements: Blastemal, Stromal, and Epithelial cells. * **Treatment:** Multimodal (Surgery + Chemotherapy +/- Radiotherapy). In the USA (NWTS), surgery is performed first; in Europe (SIOP), pre-operative chemotherapy is preferred.

Q: Which factor is associated with a good prognosis in Wilms' tumor?

Presence of aniridia. ### Explanation **Correct Answer: B. Presence of aniridia** In Wilms' tumor (Nephroblastoma), the association with **Aniridia** (absence of the iris) is a classic component of the **WAGR syndrome** (Wilms tumor, Aniridia, Genitourinary anomalies, and Range of developmental delays). The medical reasoning behind this being a "good prognosis" factor is **surveillance**. Children born with sporadic aniridia are known to be at a high risk (approx. 33%) of developing Wilms' tumor. Consequently, these patients undergo rigorous **screening with serial renal ultrasounds** every 3 months. This leads to the detection of the tumor at a much earlier, localized stage compared to the general population, resulting in better clinical outcomes. **Analysis of Incorrect Options:** * **A. Age 2-5 years:** This is the peak age of incidence for Wilms' tumor, but it is not a prognostic marker. In fact, age **<2 years** is generally associated with a better prognosis. * **C. Unilateral disease:** While bilateral disease (Stage V) is more complex, "unilateral disease" is the standard presentation and does not inherently signify a "good" prognosis compared to specific screening-detected cases. * **D. Stage I disease:** While Stage I has a better prognosis than Stage IV, the question asks for a specific *associated factor*. In the context of NEET-PG, the "Aniridia" association is a high-yield concept specifically linked to early detection benefits. **High-Yield Clinical Pearls for NEET-PG:** * **Most Important Prognostic Factor:** The **Histology** (Favorable vs. Unfavorable/Anaplastic) is the single most important predictor of survival. * **WAGR Syndrome:** Associated with a microdeletion on chromosome **11p13** (WT1 gene). * **Beckwith-Wiedemann Syndrome:** Associated with **11p15** (WT2 gene), macroglossia, and hemihypertrophy. * **Common Presentation:** Asymptomatic abdominal mass that **does not cross the midline** (unlike Neuroblastoma).

Q: Which of the following is associated with a good prognosis in Acute Lymphoblastic Leukemia (ALL)?

Hyperdiploidy. **Explanation:** Acute Lymphoblastic Leukemia (ALL) is the most common pediatric malignancy, and its prognosis is heavily influenced by cytogenetic and clinical factors. **Why Hyperdiploidy is Correct:** Hyperdiploidy (defined as >50 chromosomes per cell) is one of the most significant **favorable prognostic markers** in pediatric ALL. It is associated with an excellent response to chemotherapy, likely due to the increased sensitivity of hyperdiploid blasts to methotrexate and apoptosis. Specifically, trisomies of chromosomes 4, 10, and 17 are linked to superior outcomes. **Analysis of Incorrect Options:** * **Hypodiploidy:** Defined as <44 chromosomes, this is a **poor prognostic factor** associated with high rates of treatment failure and early relapse. * **T-cell ALL:** Traditionally carries a **worse prognosis** compared to B-cell ALL. It often presents with a high white blood cell (WBC) count and a mediastinal mass, requiring more intensive therapy. * **Philadelphia Chromosome [t(9;22)]:** This translocation (BCR-ABL1) is a **very poor prognostic marker**. While prognosis has improved with the addition of Tyrosine Kinase Inhibitors (e.g., Imatinib), it remains a high-risk feature. **High-Yield Clinical Pearls for NEET-PG:** * **Favorable Prognosis:** Age 1–9 years, WBC count 10 years, WBC count >50,000/µL, Hypodiploidy, t(9;22), and t(4;11) [KMT2A rearrangement]. * **Most common subtype:** Pre-B cell ALL (CD10/CALLA positive) generally has a better prognosis than T-cell or mature B-cell (Burkitt-type) ALL.

Q: Which of the following conditions is associated with the highest risk of Wilm's tumor?

Denys Drash syndrome. **Explanation:** The risk of developing Wilms tumor (nephroblastoma) varies significantly among associated genetic syndromes. The correct answer is **Denys-Drash Syndrome (DDS)** because it carries the highest cumulative risk, exceeding **90%**. **1. Why Denys-Drash Syndrome is Correct:** DDS is characterized by the triad of progressive renal failure (mesangial sclerosis), male pseudohermaphroditism, and Wilms tumor. It is caused by a point mutation in the **WT1 gene** (11p13). Due to the extremely high risk (>90%), prophylactic nephrectomy is often considered once renal failure progresses. **2. Analysis of Incorrect Options:** * **WAGR Syndrome:** (Wilms tumor, Aniridia, Genitourinary anomalies, and intellectual disability/Retardation). This is caused by a microdeletion at 11p13 involving both *WT1* and *PAX6* genes. The risk of Wilms tumor is approximately **30-50%**. * **Beckwith-Wiedemann Syndrome (BWS):** An overgrowth disorder (macroglossia, hemihypertrophy, omphalocele) linked to the **WT2 gene** (11p15.5). While it is the most common overgrowth syndrome associated with Wilms, the actual risk of developing the tumor is relatively lower, at approximately **5-10%**. **3. High-Yield Clinical Pearls for NEET-PG:** * **Most common presentation:** Asymptomatic abdominal mass that does **not** cross the midline (unlike Neuroblastoma). * **Staging:** Wilms tumor is staged surgically (Post-nephrectomy). * **Metastasis:** The most common site for distant metastasis is the **Lungs**. * **Screening Protocol:** For children with these syndromes, abdominal ultrasound is recommended every 3 months until age 7-8 to ensure early detection.

Q: Hutchinson's secondaries in the skull are due to tumors in which organ?

Adrenals. **Explanation:** **Hutchinson’s secondaries** refer to metastatic deposits in the skull and orbit, specifically originating from a **Neuroblastoma**. In children, the most common site for a primary neuroblastoma is the **Adrenal Medulla** (derived from neural crest cells). 1. **Why Adrenals are Correct:** Neuroblastoma is the most common extracranial solid tumor of childhood. It has a unique propensity for hematogenous spread to the bones. When it involves the skull and orbit, it leads to characteristic clinical features like **proptosis and periorbital ecchymosis ("Raccoon Eyes")**. Radiologically, these secondaries often cause "sunray" spicule formation and sutural widening due to increased intracranial pressure. 2. **Why Other Options are Incorrect:** * **Lungs & Breast:** These are common primary sites for bone metastasis in **adults**. In the pediatric age group, primary lung or breast carcinomas are extremely rare. * **Liver:** While neuroblastoma can metastasize to the liver (known as **Pepper’s syndrome**), the term "Hutchinson’s secondaries" is specifically reserved for bone/skull involvement. **Clinical Pearls for NEET-PG:** * **Hutchinson’s Type:** Metastasis to the skull, orbit, and long bones (common in older children). * **Pepper’s Type:** Massive involvement of the liver (common in infants). * **Smith’s Type:** Metastasis to the cervical lymph nodes. * **Radiology Sign:** On X-ray, look for the **"Sunray appearance"** of the skull and widening of cranial sutures. * **Biochemical Marker:** Elevated urinary VMA (Vanillylmandelic acid) and HVA (Homovanillic acid).

Question 1

All of the statements regarding Wilm's tumor are true EXCEPT:

Accepted Answer

It typically presents after 5 years of age.

Answer

Hematuria is a common presenting symptom.

Answer

It often presents as an abdominal mass.

Answer

It most commonly metastasizes to the lungs.

Question 2

What is the most common leukemia in children with Down syndrome less than 3 years old?

Accepted Answer

Acute myeloid leukemia M7 (AML-M7)

Answer

Acute lymphoblastic leukemia (ALL)

Answer

Acute myeloid leukemia M5 (AML-M5)

Answer

Hodgkin lymphoma

Question 3

A child presents with proptosis, multiple skeletal limb secondaries, and sutural separation. What is the most likely diagnosis?

Accepted Answer

Neuroblastoma

Answer

Medulloblastoma

Answer

Retinoblastoma

Answer

None of the above

Question 4

All of the following are conditions associated with Wilm's tumor, except?

Accepted Answer

Polycystic kidney

Answer

Aniridia

Answer

Gonadal dysgenesis

Answer

Hemihypertrophy

Question 5

All of the following are good prognostic factors for childhood ALL except?

Accepted Answer

Female sex

Answer

Hyperdiploidy

Answer

t(12:21) translocation

Answer

Pre B cell ALL

Question 6

What is the most important determinant of prognosis in Wilms tumor?

Accepted Answer

Histology

Answer

Stage of disease

Answer

Loss of heterozygosity of chromosome 1p

Answer

Age less than one year at presentation

Question 7

Which factor is associated with a good prognosis in Wilms' tumor?

Accepted Answer

Presence of aniridia

Answer

Age 2-5 years

Answer

Unilateral disease

Answer

Stage I disease

Question 8

Which of the following is associated with a good prognosis in Acute Lymphoblastic Leukemia (ALL)?

Accepted Answer

Hyperdiploidy

Answer

Hypodiploidy

Answer

T cell line

Answer

Philadelphia chromosome

Question 9

Which of the following conditions is associated with the highest risk of Wilm's tumor?

Accepted Answer

Denys Drash syndrome

Answer

WAGR syndrome

Answer

Beckwith-Weidemann syndrome

Answer

None of the above

Question 10

Hutchinson's secondaries in the skull are due to tumors in which organ?

Accepted Answer

Adrenals

Answer

Lungs

Answer

Breast

Answer

Liver

Oncology — MCQs

Oncology — MCQs

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