Oncology Practice Questions

Q: A neonate presents with a palpable mass in the kidney. Ultrasound reveals a hypoechoic shadow. What is the most likely diagnosis?

Mesonephroblastic tumor. **Explanation:** The most likely diagnosis is **Mesoblastic Nephroma** (Congenital Mesoblastic Nephroma). The key clinical clue here is the **age of the patient (neonate)**. **1. Why Mesoblastic Nephroma is correct:** Mesoblastic nephroma is the **most common renal tumor found in the newborn period** (neonates <3 months). It is a benign spindle-cell tumor that typically presents as a large, firm, palpable flank mass. On ultrasound, it classically appears as a solid, hypoechoic mass, sometimes showing a "concentric ring" or "whorled" appearance. It is often considered the benign neonatal counterpart of Wilms' tumor. **2. Why other options are incorrect:** * **Wilms' Tumor (Nephroblastoma):** While it is the most common renal malignancy in children, it is **extremely rare in the neonatal period**. The peak incidence is between 2–5 years of age. * **Neuroblastoma:** This is the most common extracranial solid tumor of infancy. However, it typically arises from the **adrenal gland** (suprarenal) rather than the kidney itself. While it can displace the kidney, the primary mass is extra-renal. * **Adenoma:** Renal adenomas are rare in the pediatric population and are typically incidental findings in adults. **High-Yield Clinical Pearls for NEET-PG:** * **Most common neonatal renal mass:** Multicystic Dysplastic Kidney (MCDK) – Non-neoplastic. * **Most common neonatal renal *tumor*:** Mesoblastic Nephroma – Neoplastic. * **Treatment of choice for Mesoblastic Nephroma:** Radical nephrectomy (chemotherapy is usually not required). * **Association:** Mesoblastic nephroma is frequently associated with **polyhydramnios** during pregnancy. * **Wilms’ Tumor vs. Neuroblastoma:** Wilms' tumor usually does *not* cross the midline and has a smooth surface, whereas Neuroblastoma often crosses the midline and has an irregular surface.

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

Arthrogryposis multiplex congenita. **Explanation:** Wilm’s tumor (Nephroblastoma) is the most common primary renal tumor of childhood. Approximately 10% of cases are associated with specific congenital malformation syndromes, primarily linked to mutations or deletions on chromosome **11p13 (WT1 gene)** or **11p15 (WT2 gene)**. **Why Arthrogryposis multiplex congenita is the correct answer:** Arthrogryposis multiplex congenita (AMC) refers to the development of multiple joint contractures present at birth. It is typically caused by fetal akinesia (decreased fetal movement) due to muscular, neurological, or connective tissue disorders. It has **no established clinical or genetic association** with Wilm’s tumor. **Analysis of Incorrect Options:** * **Aniridia:** This is the absence of the iris. It is a key component of the **WAGR Syndrome** (Wilm’s tumor, Aniridia, Genitourinary anomalies, and intellectual disability/Range of developmental delays), caused by a microdeletion at 11p13. * **Male pseudohermaphroditism:** This is seen in **Denys-Drash Syndrome**, characterized by the triad of progressive renal disease (mesangial sclerosis), male pseudohermaphroditism (gonadal dysgenesis), and a high risk (90%) of Wilm’s tumor. * **Hemihypertrophy:** This refers to the overgrowth of one side of the body. It is a classic feature of **Beckwith-Wiedemann Syndrome (BWS)**, an overgrowth disorder (11p15) that carries an increased risk for Wilm’s tumor and hepatoblastoma. **High-Yield Clinical Pearls for NEET-PG:** 1. **WAGR Syndrome:** Deletion of *WT1* and *PAX6* genes. 2. **Beckwith-Wiedemann Syndrome:** Features include macroglossia, omphalocele, hemihypertrophy, and visceromegaly. 3. **Screening:** Children with these syndromes require abdominal ultrasounds every 3 months until age 7 for early detection of Wilm’s tumor. 4. **Most common presentation:** A painless, smooth, firm abdominal mass that rarely crosses the midline.

Q: Which of the following is NOT associated with an increased risk of acute leukemia in children?

Marfan's syndrome. **Explanation:** The association between chromosomal abnormalities and pediatric malignancies is a high-yield topic for NEET-PG. The correct answer is **Marfan’s syndrome**, as it is a connective tissue disorder caused by a mutation in the *FBN1* gene (fibrillin-1) and is **not** associated with a predisposition to hematological malignancies. **Why the other options are incorrect:** * **Down’s Syndrome (Trisomy 21):** This is the most significant risk factor. Children with Down’s syndrome have a 10–20 fold increased risk of leukemia. Specifically, they are prone to **Acute Megakaryoblastic Leukemia (AML-M7)** before age 3 and **ALL** after age 3. * **Klinefelter’s Syndrome (47, XXY):** This condition is associated with an increased risk of both **ALL** and extragonadal germ cell tumors (mediastinal). * **Turner’s Syndrome (45, XO):** While the risk is lower than in Down’s syndrome, studies have shown a statistically significant increased predisposition to **acute leukemias** and certain solid tumors (like neuroblastoma) in these patients. **Clinical Pearls for NEET-PG:** 1. **DNA Repair Defects:** Conditions like **Fanconi Anemia**, **Ataxia-Telangiectasia**, and **Bloom Syndrome** carry the highest risks for developing AML and ALL due to chromosomal instability. 2. **Transient Myeloproliferative Disorder (TMD):** Unique to neonates with Down’s syndrome; it often resolves spontaneously but increases the risk of future AML. 3. **Neurofibromatosis Type 1:** Strongly associated with **Juvenile Myelomonocytic Leukemia (JMML)**. 4. **Li-Fraumeni Syndrome:** Caused by *TP53* mutations; associated with "SBLA" syndrome (Sarcoma, Breast cancer, Leukemia, Adrenal cortical carcinoma).

Q: What is the most common presentation of Wilm's tumour?

Abdominal mass. **Explanation:** **Wilms’ Tumor (Nephroblastoma)** is the most common primary renal malignancy in children, typically occurring between the ages of 2 and 5 years. 1. **Why Abdominal Mass is Correct:** The hallmark presentation of Wilms’ tumor is a **painless, asymptomatic abdominal mass**. In approximately 80–90% of cases, the mass is discovered incidentally by a parent while bathing or dressing the child or during a routine physical examination. Characteristically, the mass is firm, smooth, and **does not cross the midline** (unlike Neuroblastoma, which often does). 2. **Why Other Options are Incorrect:** * **Fever:** While fever can occur due to tumor necrosis, it is present in only about 20% of cases and is rarely the primary complaint. * **Hematuria:** Microscopic hematuria is seen in about 25% of patients, but gross hematuria is uncommon (less than 10%). It usually indicates invasion of the renal pelvis. * **Urinary Tract Infection (UTI):** While a large tumor might cause stasis or compression leading to a UTI, it is a secondary complication rather than a classic presenting feature. **Clinical Pearls for NEET-PG:** * **Most common associated sign:** Hypertension (seen in ~25% of cases due to increased renin production). * **WAGR Syndrome:** Wilms’ tumor, Aniridia, Genitourinary anomalies, and intellectual disability (formerly Retardation). * **Beckwith-Wiedemann Syndrome:** Macroglossia, organomegaly, and hemihypertrophy; these children require screening ultrasounds for Wilms’ tumor. * **Management Tip:** Avoid vigorous palpation of the abdomen to prevent rupture of the tumor capsule and subsequent peritoneal seeding.

Q: A 6-year-old child presents with joint pain, respiratory distress, recurrent infections, pallor, conjunctival hemorrhage, and splenomegaly. A chest X-ray shows a mediastinal mass. What is the most likely diagnosis?

Acute lymphoblastic leukemia (ALL). ### Explanation **Correct Option: A. Acute Lymphoblastic Leukemia (ALL)** Acute Lymphoblastic Leukemia is the most common pediatric malignancy. This clinical presentation is classic for **T-cell ALL**, which typically affects older children and adolescents (unlike B-cell ALL). * **Bone Marrow Failure:** Pallor (anemia), recurrent infections (leukopenia/dysfunctional WBCs), and conjunctival hemorrhage (thrombocytopenia) reflect marrow infiltration. * **Extramedullary Involvement:** Splenomegaly and joint pain (due to leukemic infiltration of the periosteum) are common. * **Mediastinal Mass:** A hallmark of T-cell ALL is a **thymic mass**, which leads to respiratory distress or Superior Vena Cava (SVC) syndrome. **Why Other Options are Incorrect:** * **B. Chronic Myeloid Leukemia (CML):** Rare in children. It typically presents with massive splenomegaly but lacks the acute onset of bone marrow failure and mediastinal masses seen here. * **C. Pulmonary Metastasis:** While it could cause respiratory distress, it does not explain the systemic triad of anemia, thrombocytopenia, and splenomegaly. * **D. Sjogren Syndrome:** An autoimmune condition characterized by dry eyes and mouth; it does not present with acute bone marrow failure or a mediastinal mass in a 6-year-old. **NEET-PG High-Yield Pearls:** * **Most common subtype of ALL:** B-cell ALL (CD10/CALLA positive). * **T-cell ALL Presentation:** Often presents as a "Teenager with a Thymic mass" and high WBC count. * **Good Prognostic Factors:** Age 1–9 years, low initial WBC count ( 10 years, high WBC count, hypodiploidy, and t(9;22) (Philadelphia chromosome). * **Common sites of relapse:** Central Nervous System (CNS) and Testes (blood-organ barriers).

Q: A 4-year-old child presents with an abdominal mass and fever. CT scan reveals a crescent-shaped, discrete, and peripheral calcification. Which chemotherapy regimen is indicated for this condition?

VCD. **Explanation:** The clinical presentation of a 4-year-old child with an abdominal mass, fever, and specific CT findings of **crescent-shaped, peripheral calcifications** is classic for **Neuroblastoma**. Neuroblastoma is the most common extracranial solid tumor of childhood, typically arising from the adrenal medulla or sympathetic chain. **Why VCD is correct:** The standard chemotherapy backbone for Neuroblastoma involves a combination of **Vincristine, Cyclophosphamide, and Doxorubicin (VCD)**. In high-risk cases, this is often intensified with Etoposide and Cisplatin. These agents target the rapidly dividing neural crest cells characteristic of the tumor. **Why the other options are incorrect:** * **FOLFOX (Folinic acid, Fluorouracil, Oxaliplatin):** Primarily used for colorectal cancer and other gastrointestinal malignancies; not used in pediatric embryonal tumors. * **ECF (Epirubicin, Cisplatin, Fluorouracil):** A regimen typically indicated for gastric and esophageal carcinomas. * **CAF (Cyclophosphamide, Adriamycin/Doxorubicin, Fluorouracil):** Historically used in the treatment of breast cancer. **Clinical Pearls for NEET-PG:** * **Calcification Pattern:** While Neuroblastoma shows **stippled/peripheral** calcifications, Wilms' tumor (the main differential) rarely calcifies. * **Vessel Involvement:** Neuroblastoma **encases** major vessels (e.g., Aorta), whereas Wilms' tumor **displaces** them. * **Markers:** Elevated urinary catecholamines (VMA and HVA) are diagnostic in 90% of cases. * **Prognosis:** **N-myc amplification** is the most important poor prognostic genetic marker. * **Opsoclonus-Myoclonus Syndrome:** A high-yield paraneoplastic syndrome associated with Neuroblastoma ("dancing eyes, dancing feet").

Q: What is the most common liver tumor in children?

Hemangioma. **Explanation:** The most common liver tumor in children is the **Infantile Hemangioma**. It is important to distinguish between "benign tumors" and "malignant tumors" in pediatric hepatology. While **Hepatoblastoma** is the most common *malignant* liver tumor in children, **Hemangiomas** are the most common *overall* (benign) liver tumors. * **Correct Option (A): Hemangioma:** These are benign vascular tumors. They often present in the first six months of life and may be associated with cutaneous hemangiomas. While many are asymptomatic and undergo spontaneous involution, large lesions can lead to high-output congestive heart failure due to arteriovenous shunting or Kasabach-Merritt syndrome (consumptive coagulopathy). **Why other options are incorrect:** * **B. Non-parasitic cyst:** These are generally congenital (simple cysts) and are much less common than vascular tumors in the pediatric population. * **C. Adenoma:** Hepatic adenomas are rare in children and are typically associated with specific conditions like Glycogen Storage Disease (Type I) or long-term steroid use. * **D. Focal Nodular Hyperplasia (FNH):** This is the second most common benign liver tumor in children, characterized by a "central stellate scar" on imaging, but it occurs less frequently than hemangiomas. **High-Yield Clinical Pearls for NEET-PG:** * **Most common primary malignant liver tumor:** Hepatoblastoma (associated with Beckwith-Wiedemann syndrome and FAP). * **Most common liver malignancy overall:** Metastatic disease (commonly from Neuroblastoma). * **Tumor Marker:** Elevated **Alpha-fetoprotein (AFP)** is a hallmark of Hepatoblastoma. * **Imaging:** Infantile hemangiomas typically show peripheral puddling and centripetal enhancement on contrast CT/MRI.

Q: Which of the following is a good prognostic factor for neuroblastoma?

Triploidy. **Explanation:** Neuroblastoma is the most common extracranial solid tumor of childhood. Its prognosis is highly dependent on the biological and molecular characteristics of the tumor cells. **Why Triploidy is Correct:** The DNA index (ploidy) is a significant prognostic marker in infants. **Hyperdiploidy** (specifically **triploidy**) is associated with a **favorable prognosis**. These tumors tend to be more differentiated, respond better to chemotherapy, and are often seen in younger children (Stage 4S), where spontaneous regression can occur. **Analysis of Incorrect Options:** * **A. Diploidy:** A DNA index of 1 (diploid) is associated with a **poor prognosis**. It often correlates with more aggressive tumor behavior and genetic instability. * **B. N-myc amplification:** This is the **most important poor prognostic factor** in neuroblastoma. Found on chromosome 2p, amplification (>10 copies) signifies rapid tumor progression and resistance to standard therapy, regardless of the clinical stage. * **C. Loss of 11q:** Segmental chromosomal aberrations, such as the loss of 11q or 1p, are markers of **unfavorable prognosis** and are typically seen in high-risk, non-amplified N-myc cases. **High-Yield Clinical Pearls for NEET-PG:** * **Age:** The most important clinical prognostic factor; age **<18 months** has a better prognosis. * **Shimada Classification:** Histopathological grading where "Favorable Histology" (FH) includes well-differentiated tumors with a low Mitosis-Karyorrhexis Index (MKI). * **Stage 4S (Special):** Occurs in infants <1 year with localized primary tumor but dissemination limited to liver, skin, and bone marrow (excluding cortical bone). It has an excellent prognosis. * **Urinary Markers:** Elevated VMA (Vanillylmandelic acid) and HVA (Homovanillic acid) are diagnostic.

Question 1

A neonate presents with a palpable mass in the kidney. Ultrasound reveals a hypoechoic shadow. What is the most likely diagnosis?

Accepted Answer

Mesonephroblastic tumor

Answer

Wilm's tumor

Answer

Neuroblastoma

Answer

Adenoma

Question 2

All of the following are associated with Wilm's tumor except:

Accepted Answer

Arthrogryposis multiplex congenita

Answer

Aniridia

Answer

Male pseudohermaphroditism

Answer

Hemihypertrophy

Question 3

Which of the following is NOT associated with an increased risk of acute leukemia in children?

Accepted Answer

Marfan's syndrome

Answer

Down's syndrome

Answer

Klinefelter's syndrome

Answer

Turner's syndrome

Question 4

What are the long-term effects of CNS tumors in children, excluding which of the following?

Accepted Answer

Neuropsychological effects are independent of radiation dose

Answer

Reduced IQ and learning difficulties

Answer

Endocrine dysfunction

Answer

Musculoskeletal problems

Question 5

What is the most common presentation of Wilm's tumour?

Accepted Answer

Abdominal mass

Answer

Fever

Answer

Hematuria

Answer

Urinary tract infection

Question 6

A 6-year-old child presents with joint pain, respiratory distress, recurrent infections, pallor, conjunctival hemorrhage, and splenomegaly. A chest X-ray shows a mediastinal mass. What is the most likely diagnosis?

Accepted Answer

Acute lymphoblastic leukemia (ALL)

Answer

Chronic myeloid leukemia (CML)

Answer

Pulmonary metastasis

Answer

Sjogren syndrome

Question 7

A 4-year-old child presents with an abdominal mass and fever. CT scan reveals a crescent-shaped, discrete, and peripheral calcification. Which chemotherapy regimen is indicated for this condition?

Accepted Answer

VCD

Answer

FOLFOX

Answer

ECF

Answer

CAF

Question 8

What is the most common liver tumor in children?

Accepted Answer

Hemangioma

Answer

Non-parasitic cyst

Answer

Adenoma

Answer

Focal nodular hyperplasia

Question 9

Good prognosis in Wilm's tumour is seen in which of the following?

Accepted Answer

Patient aged less than 1 year

Answer

Patient aged 2-5 years

Answer

Male child

Answer

Female child

Question 10

Which of the following is a good prognostic factor for neuroblastoma?

Accepted Answer

Triploidy

Answer

Diploidy

Answer

N-myc amplification

Answer

Loss of 11q

Oncology — MCQs

Oncology — MCQs

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