Oncology Practice Questions

Q: A 4-year-old child presented with a palpable, painless, and slowly increasing abdominal mass in the right flank region, accompanied by episodes of fever and hematuria. Examination revealed hypertension. Following a CT scan of the abdomen, the patient underwent surgical resection of the mass. The gross specimen and histopathological examination are provided. Which of the following drugs is NOT approved for the management of this condition?

Bleomycin. ***Bleomycin*** - **Bleomycin** is NOT part of standard **Wilms' tumor** chemotherapy protocols (NWTS/COG regimens). - It is primarily used for **germ cell tumors** and **lymphomas**, not for **nephroblastoma**. *Doxorubicin* - **Doxorubicin** is a key component of **intermediate** and **high-risk** Wilms' tumor treatment protocols. - It is used in combination with other agents for **stage III-V** disease or **unfavorable histology**. *Dactinomycin* - **Dactinomycin** is a cornerstone drug in **all risk groups** of Wilms' tumor treatment. - It is part of the **standard two-drug regimen** along with vincristine for **favorable histology** cases. *Vincristine* - **Vincristine** is universally used in **all Wilms' tumor protocols** regardless of stage or histology. - It forms the **backbone** of treatment, often combined with dactinomycin in **low-risk** cases.

Q: WAGR syndrome includes all except?

Anorectal malformation. **Explanation:** WAGR syndrome is a rare genetic contiguous gene deletion syndrome caused by the microdeletion of chromosome **11p13**. This specific region contains the **WT1** (Wilms Tumor 1) gene and the **PAX6** gene, which are responsible for the clinical manifestations of the syndrome. The acronym **WAGR** stands for: * **W – Wilms tumor:** Approximately 45-50% of affected children develop this nephroblastoma. * **A – Aniridia:** The absence of the iris (due to *PAX6* deletion), often the first sign noted at birth. * **G – Genitourinary anomalies:** Including cryptorchidism, hypospadias, or ambiguous genitalia. * **R – Range of developmental delays:** Formerly referred to as Mental Retardation. **Why Option C is correct:** **Anorectal malformations** are not a component of WAGR syndrome. They are more commonly associated with the **VACTERL** association (Vertebral, Anal atresia, Cardiac, Tracheo-Esophageal, Renal, Limb defects) or Currarino syndrome. **Why other options are incorrect:** * **Wilms tumor (A):** A core component; these patients require frequent renal ultrasounds for early screening. * **Mental Retardation (B):** Now termed intellectual disability, it is a classic feature of the syndrome. * **Genital anomalies (D):** These occur due to the involvement of the *WT1* gene, which is essential for normal urogenital development. **High-Yield Clinical Pearls for NEET-PG:** * **Genetics:** Microdeletion of **11p13**. * **WAGRO Syndrome:** If the deletion extends to include the *BDNF* gene, patients also present with **O**besity. * **Denys-Drash Syndrome:** Another *WT1* related disorder characterized by Wilms tumor, pseudohermaphroditism, and early-onset nephrotic syndrome (diffuse mesangial sclerosis). * **Beckwith-Wiedemann Syndrome:** Associated with Wilms tumor but involves chromosome **11p15** (WT2).

Q: What is the most common malignancy of the liver in children?

Hepatoblastoma. **Explanation:** **Hepatoblastoma** is the most common primary liver malignancy in children, accounting for approximately 50% of all pediatric liver tumors. It typically presents in infants and toddlers, with the majority of cases diagnosed before the age of 3 years. The tumor originates from primitive epithelial cells (blasts) and is strongly associated with prematurity, low birth weight, and genetic syndromes like **Beckwith-Wiedemann Syndrome** and **Familial Adenomatous Polyposis (FAP)**. **Analysis of Options:** * **Hepatocellular Carcinoma (HCC):** While it is the most common primary liver cancer in adults, it is the second most common in children. It usually occurs in older children (10–15 years) and is often associated with underlying chronic liver diseases like Hepatitis B or Tyrosinemia. * **Lymphoma:** While the liver can be involved in systemic lymphoma (secondary involvement), primary hepatic lymphoma is extremely rare in the pediatric population. * **None of the above:** Incorrect, as Hepatoblastoma is the established leading primary malignancy. **High-Yield Clinical Pearls for NEET-PG:** * **Tumor Marker:** Serum **Alpha-fetoprotein (AFP)** is elevated in >90% of cases and is used for both diagnosis and monitoring treatment response. * **Imaging:** Ultrasound is the initial screening tool, but CT/MRI is required for Pre-treatment Extent of Disease (PRETEXT) staging. * **Pathology:** Look for "fetal" or "embryonal" epithelial cells on histology. * **Treatment:** A combination of neoadjuvant chemotherapy and surgical resection. It is a highly vascular tumor; hence, complete resection is the goal.

Q: Wilms' tumour is associated with all of the following except?

Polycystic kidney disease. **Explanation:** Wilms’ tumor (Nephroblastoma) is the most common primary renal malignancy in children. It is frequently associated with specific congenital malformation syndromes due to genetic mutations on chromosome 11 (WT1 and WT2 genes). **Why Polycystic Kidney Disease (PKD) is the correct answer:** PKD is a genetic disorder characterized by the growth of numerous cysts in the kidneys, but it is **not** a recognized syndromic association or a precursor to Wilms’ tumor. While both involve the kidneys, their underlying pathophysiology and genetic origins (PKD1/PKD2 vs. WT1/WT2) are distinct. **Analysis of Incorrect Options:** * **Aniridia:** This is the absence of the iris. It is a key component of the **WAGR syndrome** (Wilms’ tumor, Aniridia, Genitourinary anomalies, and intellectual disability/Range of developmental delays), caused by a microdeletion on chromosome 11p13. * **Beckwith-Wiedemann Syndrome (BWS):** A classic overgrowth syndrome (macroglossia, omphalocele, organomegaly) associated with Wilms’ tumor due to mutations at the 11p15.5 locus (WT2). * **Hemihypertrophy:** This refers to the asymmetric overgrowth of one side of the body. It is a common clinical finding in children with BWS and is a known high-risk marker for the development of Wilms’ tumor. **NEET-PG High-Yield Pearls:** 1. **Denys-Drash Syndrome:** Characterized by the triad of Wilms’ tumor, male pseudohermaphroditism, and early-onset renal failure (mesangial sclerosis). 2. **Screening:** Children with associated syndromes (BWS, WAGR, or isolated hemihypertrophy) require screening with abdominal ultrasound every 3 months until age 7. 3. **Most common presentation:** A painless, palpable abdominal mass that typically does not cross the midline (unlike Neuroblastoma). 4. **Metastasis:** The most common site of distant spread is the **Lungs**.

Q: Which of the following is the most important prognostic factor in ALL?

Response to steroids. **Explanation:** In Acute Lymphoblastic Leukemia (ALL), the **response to initial therapy (specifically steroids)** is considered the single most important independent prognostic factor. This is because the "in-vivo" sensitivity of leukemic blasts to corticosteroids (prednisolone) within the first 7–14 days of treatment reflects the underlying biological aggressiveness and chemo-sensitivity of the disease. A poor response (high blast count after a steroid prophase) indicates a significantly higher risk of relapse and treatment failure. **Analysis of Options:** * **Response to Steroids (Correct):** Early clearance of peripheral blasts (prednisolone response) and achieving Minimal Residual Disease (MRD) negativity are the strongest predictors of long-term survival. * **Age (Incorrect):** While age is a major prognostic factor (favorable between 1–9 years; unfavorable 10 years), it is secondary to the dynamic response to treatment. * **Total Leukocyte Count (TLC) (Incorrect):** A TLC >50,000/µl is a poor prognostic marker (High-Risk category), but many patients with high TLC still achieve remission if they respond well to induction therapy. * **Hyperploidy (Incorrect):** Cytogenetics are vital for risk stratification (e.g., Hyperdiploidy >50 is favorable; Philadelphia chromosome t(9;22) is unfavorable), but clinical response remains the ultimate guide for prognosis. **High-Yield Clinical Pearls for NEET-PG:** * **Best Prognosis:** Age 1–9 years, TLC 50 chromosomes), and t(12;21) (TEL-AML1). * **Worst Prognosis:** Age 10 years, TLC >50,000, Hypodiploidy, and t(9;22) (BCR-ABL). * **L3 Subtype (Burkitt’s):** Associated with t(8;14) and has the worst prognosis among FAB classifications.

Question 1

A 4-year-old child presented with a palpable, painless, and slowly increasing abdominal mass in the right flank region, accompanied by episodes of fever and hematuria. Examination revealed hypertension. Following a CT scan of the abdomen, the patient underwent surgical resection of the mass. The gross specimen and histopathological examination are provided. Which of the following drugs is NOT approved for the management of this condition?

Accepted Answer

Bleomycin

Answer

Doxorubicin

Answer

Dactinomycin

Answer

Vincristine

Question 2

WAGR syndrome includes all except?

Accepted Answer

Anorectal malformation

Answer

Wilms tumour

Answer

Mental Retardation

Answer

Genital anomalies

Question 3

What is the most common malignancy of the liver in children?

Accepted Answer

Hepatoblastoma

Answer

Hepatocellular carcinoma

Answer

Lymphoma

Answer

None of the above

Question 4

Wilms' tumour is associated with all of the following except?

Accepted Answer

Polycystic kidney disease

Answer

Hemihypertrophy

Answer

Beckwith syndrome

Answer

Aniridia

Question 5

Which of the following is the most important prognostic factor in ALL?

Accepted Answer

Response to steroids

Answer

Hyperploidy

Answer

Total leukocyte count greater than 50,000

Answer

Age

Oncology — MCQs

Oncology — MCQs

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