Oncology Practice Questions

Q: A 5-year-old child presents with a history of fever off and on for the past 2 weeks, petechial spots all over the body, and increasing pallor for the past 1 month. Examination reveals splenomegaly of 2 cm below the costal margin. What is the most likely diagnosis?

Acute leukemia. **Explanation:** The clinical triad of **fever** (due to neutropenia/infection), **petechiae** (due to thrombocytopenia), and **pallor** (due to anemia) in a child strongly suggests **bone marrow failure** or infiltration [1]. 1. **Why Acute Leukemia is correct:** In children, Acute Lymphoblastic Leukemia (ALL) is the most common malignancy. The presence of **splenomegaly** is the key differentiating factor here [1]. It indicates an infiltrative process where leukemic cells have spread to the reticuloendothelial system [2]. The combination of pancytopenia (fever, spots, pallor) plus organomegaly is a classic presentation of Acute Leukemia. 2. **Why other options are incorrect:** * **Idiopathic Thrombocytopenic Purpura (ITP):** While it causes petechiae [1], it typically presents as "isolated thrombocytopenia." The child is usually otherwise healthy (no pallor or fever) and splenomegaly is characteristically absent. * **Aplastic Anemia:** This presents with pancytopenia (fever, pallor, bleeding), but **splenomegaly is never a feature** of primary aplastic anemia. Its presence points toward an alternative diagnosis [1]. * **Hypersplenism:** While it can cause cytopenias, it is usually secondary to portal hypertension or chronic malaria. It does not typically present with the acute, febrile, and rapidly progressive symptoms seen in this 5-year-old. **Clinical Pearls for NEET-PG:** * **Most common childhood cancer:** ALL (Peak age: 2–5 years). * **Pancytopenia + Splenomegaly:** Think Leukemia, Lymphoma, or Gaucher’s disease [2]. * **Pancytopenia WITHOUT Splenomegaly:** Think Aplastic Anemia or Vitamin B12 deficiency. * **Diagnostic Gold Standard:** Bone marrow aspiration showing >20% blasts.

Q: What is the most common tumor of the face in children?

Rhabdomyosarcoma. **Explanation:** **Rhabdomyosarcoma (RMS)** is the correct answer because it is the most common soft tissue sarcoma in children, accounting for approximately 50% of all pediatric soft tissue sarcomas. The **head and neck** are the most frequent primary sites (approx. 35-40%), with the orbit, nasopharynx, and facial soft tissues being common locations. Unlike adults, where epithelial tumors (carcinomas) are common, pediatric malignancies are predominantly mesenchymal or embryonal in origin. **Analysis of Incorrect Options:** * **B & C (Squamous Cell and Basal Cell Carcinoma):** These are the most common skin cancers in **adults**, typically resulting from cumulative UV damage over decades. They are extremely rare in children unless there is a predisposing genetic condition like Xeroderma Pigmentosum. * **D (Mixed Parotid Tumor/Pleomorphic Adenoma):** While this is the most common benign salivary gland tumor, it is significantly less frequent in the pediatric population compared to RMS and typically presents as a localized parotid swelling rather than a general facial tumor. **Clinical Pearls for NEET-PG:** * **Subtypes:** The **Embryonal** variant is the most common overall (better prognosis), while the **Alveolar** variant is more aggressive and often found in the extremities. * **Common Sites:** Orbit is the most common head and neck site; it presents with rapid-onset proptosis. * **Staging:** RMS is unique as it uses both a "Stage" (TNM) and a "Group" (based on the extent of surgical resection). * **Histology:** Look for **"Rhabdomyoblasts"** or "Strap cells" and immunohistochemistry markers like **Desmin, Myogenin, and Myo-D1**.

Q: Which of the following is NOT a characteristic feature of neuroblastoma in children?

Cafe-au-lait spots. **Explanation:** Neuroblastoma is a common extracranial solid tumor in children, arising from primordial neural crest cells of the sympathetic nervous system. **Why Cafe-au-lait spots is the correct answer:** Cafe-au-lait spots are hyperpigmented macules typically associated with **Neurofibromatosis Type 1 (NF-1)**, McCune-Albright syndrome, or Tuberous Sclerosis. While NF-1 increases the risk of certain tumors (like optic gliomas or pheochromocytomas), it is not a characteristic clinical feature of neuroblastoma. **Analysis of other options:** * **Proptosis:** This is a classic sign of metastatic neuroblastoma. Retro-orbital metastasis leads to periorbital ecchymosis and proptosis, famously known as **"Raccoon Eyes."** * **Hypertension:** Neuroblastomas are embryonal tumors of the sympathetic chain/adrenal medulla. They often produce catecholamines (VMA/HVA). While not as common as in pheochromocytoma, hypertension can occur due to catecholamine excess or renal artery compression (Goldblatt kidney). * **Hepatomegaly:** Massive liver involvement is a hallmark of **Stage 4S neuroblastoma** (Pepper Syndrome), seen in infants, where the liver is a primary site of metastatic spread. **High-Yield Clinical Pearls for NEET-PG:** * **Opsoclonus-Myoclonus Syndrome:** A paraneoplastic syndrome ("Dancing eyes, dancing feet") highly specific to neuroblastoma. * **Homer-Wright Rosettes:** The characteristic histopathological finding (pseudorosettes). * **N-myc Amplification:** The most important prognostic factor; its presence indicates a poor prognosis. * **Differentiating from Wilms Tumor:** Neuroblastoma often **crosses the midline**, is irregular/nodular, and may show **calcifications** on X-ray/CT, unlike Wilms tumor which is usually smooth and confined to one side.

Q: A child presents with hemihypertrophy (left arm and leg appear larger than the right) and aniridia. There is no family history of aniridia or hemihypertrophy. Which of the following is the most likely associated major abnormality in this patient?

Wilms tumor. **Explanation:** The clinical presentation of **hemihypertrophy** (asymmetric overgrowth) and **aniridia** (absence of the iris) in a child strongly suggests **WAGR Syndrome**. This is a microdeletion syndrome involving chromosome **11p13**, affecting the *WT1* (Wilms tumor suppressor) and *PAX6* (ocular development) genes. 1. **Why Wilms Tumor is correct:** The acronym **WAGR** stands for **W**ilms tumor, **A**niridia, **G**enitourinary anomalies, and **R**ange of developmental delays (Intellectual disability). Hemihypertrophy is a classic phenotypic association with Wilms tumor, often seen in WAGR or Beckwith-Wiedemann Syndrome. Due to the deletion of the *WT1* gene, these children have a 40–50% lifetime risk of developing Wilms tumor. 2. **Why other options are incorrect:** * **Deafness:** Not a component of WAGR or common Wilms-associated syndromes. (Associated with syndromes like Alport or Waardenburg). * **Seizures:** While intellectual disability is part of WAGR, epilepsy is not a primary or defining feature. * **Congestive Heart Failure:** Not typically associated with Wilms tumor unless as a rare complication of chemotherapy (e.g., Doxorubicin) or severe hypertension. **High-Yield Clinical Pearls for NEET-PG:** * **WAGR Syndrome:** Deletion of **11p13** (*WT1* and *PAX6*). * **Beckwith-Wiedemann Syndrome (BWS):** Characterized by macroglossia, omphalocele, and hemihypertrophy; involves **11p15** (*IGF-2*). * **Denys-Drash Syndrome:** Triad of Wilms tumor, pseudohermaphroditism, and early-onset renal failure (nephropathy). * **Screening:** Children with hemihypertrophy or WAGR require abdominal ultrasounds every 3 months until age 7–8 to screen for Wilms tumor.

Q: A 1.5-year-old female presents with excessive head enlargement, feeding intolerance, and severe malnutrition. MRI suggests a medulloblastoma causing obstructive hydrocephalus. Which of the following represents an irrational management approach for this patient?

Radiotherapy of 35-40 Gy to the whole craniospinal axis.. **Explanation:** The core concept in pediatric neuro-oncology is the **avoidance of craniospinal irradiation (CSI) in children under 3 years of age.** **Why Option D is the Correct (Irrational) Choice:** In a 1.5-year-old child, the developing brain is highly sensitive to radiation. Radiotherapy to the craniospinal axis at this age leads to devastating long-term sequelae, including severe neurocognitive deficits, endocrine dysfunction (growth hormone deficiency), and secondary malignancies. Therefore, the standard of care for medulloblastoma in infants is maximal safe resection followed by intensive chemotherapy to delay or eliminate the need for radiotherapy. **Analysis of Incorrect Options:** * **Option A:** Subtotal excision is often preferred over gross total resection if the tumor is adherent to vital structures like the brainstem (colliculus) to prevent permanent neurological deficits. * **Option B:** Obstructive hydrocephalus is a common complication of posterior fossa tumors. A VP shunt or an External Ventricular Drain (EVD) is a standard palliative or preoperative measure to stabilize intracranial pressure. * **Option C:** Chemotherapy (using agents like Vincristine, Cisplatin, or CCNU) is the primary adjuvant treatment modality for medulloblastoma in children <3 years to avoid the toxic effects of radiation. **High-Yield Clinical Pearls for NEET-PG:** * **Medulloblastoma:** Most common malignant brain tumor in children; arises from the roof of the 4th ventricle (vermis). * **Homer-Wright Rosettes:** Classic histopathological finding. * **Drop Metastasis:** Medulloblastoma has a high propensity for CSF seeding; hence, the entire neuraxis must be imaged (MRI Spine). * **Age Cut-off:** Always look for the age in the stem. If the patient is **<3 years old**, radiotherapy is generally contraindicated.

Q: All are true about the presentation of Wilms tumor, except?

Bilateral involvement is common. **Explanation:** Wilms tumor (Nephroblastoma) is the most common primary renal malignancy in children. The correct answer is **D** because bilateral involvement is **uncommon**, occurring in only **5–7%** of cases. Most Wilms tumors are sporadic and unilateral. **Breakdown of Options:** * **Option A (Painless mass):** This is the most common presentation. It typically manifests as a smooth, firm, abdominal mass that rarely crosses the midline. It is usually discovered incidentally by parents during bathing or dressing. * **Option B (Fever):** Fever is a documented constitutional symptom in about 20% of cases, often due to tumor necrosis or hemorrhage within the tumor. * **Option C (Can present in infancy):** While the peak incidence is between **2 to 5 years** of age, Wilms tumor can occur in infancy. However, if a renal mass is found in a neonate (under 3 months), Congenital Mesoblastic Nephroma is more likely. **High-Yield 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:** Macroglossia, organomegaly, and hemihypertrophy. Associated with **WT2** gene on Chromosome 11p15. * **Hypertension:** Seen in 25% of cases due to increased renin production or renal artery compression. * **Management Tip:** Avoid vigorous palpation of the abdomen to prevent rupture of the tumor capsule and subsequent peritoneal seeding.

Q: Which of the following is NOT a favorable prognostic marker in pediatric acute lymphoblastic leukemia (ALL)?

Hypodiploidy. **Explanation:** In pediatric Acute Lymphoblastic Leukemia (ALL), prognosis is determined by age, initial white blood cell (WBC) count, and cytogenetic abnormalities. **1. Why Hypodiploidy is the Correct Answer:** **Hypodiploidy** (defined as 50 chromosomes) is a favorable prognostic factor. **2. Analysis of Incorrect Options (Favorable Markers):** * **Age (2–10 years):** This is the "golden age" for ALL prognosis. Children diagnosed between ages 1 and 10 have significantly better outcomes than infants ( 10 years). * **Low WBC count:** An initial WBC count of ** 50,000) suggests a more aggressive disease. * **t(12;21):** Also known as the *TEL-AML1* (ETV6-RUNX1) fusion, this is the most common translocation in childhood B-ALL and is associated with an **excellent prognosis** and high cure rates. **Clinical Pearls for NEET-PG:** * **Most common childhood cancer:** ALL. * **Best Prognosis Cytogenetics:** t(12;21) and Hyperdiploidy. * **Worst Prognosis Cytogenetics:** t(9;22) [Philadelphia chromosome/BCR-ABL1], t(4;11) [MLL rearrangement], and Hypodiploidy. * **Early Response:** The most important independent prognostic factor today is the **Minimal Residual Disease (MRD)** status at the end of induction therapy.

Q: A 9-year-old boy presents with fever, feeling unwell, and easy bruising on his legs. On examination, he is pale, with a blood pressure of 100/60 mm Hg, pulse of 100/min, and a temperature of 37.8°C. His lungs are clear, his abdomen is soft with a palpable spleen, and there are petechiae and bruises on his legs. His CBC reveals a hemoglobin of 8.5 g/dL, WBC of 17,000/mL, and platelets of 30,000/mL. A blood film reveals neutropenia and leukemic lymphoblasts. What is the most appropriate initial diagnostic test to confirm the diagnosis?

Bone marrow aspirate and biopsy. **Explanation:** The clinical presentation of fever, pallor (anemia), petechiae/bruising (thrombocytopenia), and splenomegaly in a child, combined with the presence of **lymphoblasts** on a peripheral smear, is highly suggestive of **Acute Lymphoblastic Leukemia (ALL)**—the most common pediatric malignancy. 1. **Why Bone Marrow Aspirate and Biopsy is correct:** While a peripheral smear provides a clue, the definitive diagnosis of leukemia requires a bone marrow examination. According to standard diagnostic criteria, the presence of **≥20% blasts** in the bone marrow is required to confirm the diagnosis. The aspirate also allows for essential ancillary testing, including **immunophenotyping (flow cytometry)** to differentiate B-cell from T-cell ALL, and **cytogenetics** (e.g., t(12;21)), which are critical for risk stratification and treatment planning. 2. **Why other options are incorrect:** * **Monospot test:** Used for Infectious Mononucleosis (EBV). While it presents with fever and splenomegaly, it would show atypical lymphocytes, not lymphoblasts, and would not explain the severe cytopenias (anemia/thrombocytopenia). * **CT scan of the abdomen:** May confirm splenomegaly but cannot diagnose the underlying hematological malignancy. * **Chest X-ray:** Useful to check for a mediastinal mass (common in T-cell ALL), but it is a staging/supportive tool, not a primary diagnostic test for leukemia. **High-Yield Clinical Pearls for NEET-PG:** * **Most common childhood leukemia:** ALL (Peak age: 2–5 years). * **Best prognostic factor:** Age (1–9 years) and low initial WBC count (<50,000/µL). * **Commonest cytogenetic abnormality (Good prognosis):** t(12;21) / *ETV6-RUNX1*. * **Poor prognosis:** t(9;22) / Philadelphia chromosome (more common in adults). * **Sanctuary sites:** Testes and CNS (require specific prophylactic therapy).

Q: Which of the following is a definite marker for hepatoblastoma?

Alpha-fetoprotein (AFP). **Explanation:** **Hepatoblastoma** is the most common primary liver tumor in children, typically occurring before the age of 3. The definitive biochemical marker for this malignancy is **Alpha-fetoprotein (AFP)**. 1. **Why AFP is correct:** AFP is a glycoprotein normally produced by the fetal liver and yolk sac. In hepatoblastoma, serum AFP levels are significantly elevated (often >100,000 ng/mL) in over 90% of cases. It serves three critical roles: **diagnosis**, monitoring **response to therapy**, and detecting **recurrence**. A very low AFP at diagnosis (<100 ng/mL) is actually a poor prognostic sign, as it indicates a more aggressive, undifferentiated tumor subtype (small cell undifferentiated). 2. **Why other options are incorrect:** * **LDH:** A non-specific marker of cell turnover. While elevated in many pediatric malignancies (like neuroblastoma or lymphoma), it lacks the specificity required for a diagnosis of hepatoblastoma. * **HCG:** While some hepatoblastomas can secrete HCG (leading to precocious puberty), it is not a universal or definitive marker. It is more classically associated with germ cell tumors. * **Alkaline Phosphatase:** This is a marker of biliary obstruction or bone turnover; it is not specific to the pathogenesis of hepatoblastoma. **High-Yield Clinical Pearls for NEET-PG:** * **Associated Conditions:** Hepatoblastoma is strongly linked with **Beckwith-Wiedemann Syndrome** and **Familial Adenomatous Polyposis (FAP)**. * **Radiology:** Look for a large, solitary mass in the right lobe of the liver with "sunburst" calcifications on CT. * **Treatment:** The mainstay is neoadjuvant chemotherapy followed by surgical resection. * **Differential:** In an older child (>5 years) with a liver mass and normal AFP, consider **Hepatocellular Carcinoma (HCC)** or **Mesenchymal Hamartoma**.

Q: A 9-year-old female child presents with a history of headache and visual disturbances. What is the most likely diagnosis?

Craniopharyngioma. ***Craniopharyngioma*** - Most common **suprasellar tumor** in children, presenting with the classic triad of **raised intracranial pressure** (headache), **visual field defects** (bitemporal hemianopia), and **endocrine dysfunction**. - Characteristic imaging shows a **suprasellar cystic and solid mass** with **calcifications** in 90% of childhood cases, distinguishing it from other sellar region tumors. *Pituitary macroadenoma* - Extremely rare in **pediatric population**, typically occurs in adults over 40 years of age. - Usually presents with **hormonal hypersecretion syndromes** (prolactinoma, growth hormone excess) rather than mass effect symptoms in children. *Hypothalamic hamartoma* - Primarily causes **gelastic seizures** (laughing spells) and **precocious puberty**, not typically headache and visual disturbances. - Appears as a **non-enhancing isodense mass** attached to the hypothalamus without calcifications or cystic components. *Optic glioma* - More commonly presents with **unilateral visual loss** and **proptosis** rather than bitemporal visual field defects. - Associated with **neurofibromatosis type 1** and appears as **fusiform enlargement** of the optic nerve without suprasellar calcifications.

Question 1

A 5-year-old child presents with a history of fever off and on for the past 2 weeks, petechial spots all over the body, and increasing pallor for the past 1 month. Examination reveals splenomegaly of 2 cm below the costal margin. What is the most likely diagnosis?

Accepted Answer

Acute leukemia

Answer

Idiopathic thrombocytopenic purpura

Answer

Hypersplenism

Answer

Aplastic anemia

Question 2

What is the most common tumor of the face in children?

Accepted Answer

Rhabdomyosarcoma

Answer

Squamous cell carcinoma

Answer

Basal cell carcinoma

Answer

Mixed parotid tumor

Question 3

Which of the following is NOT a characteristic feature of neuroblastoma in children?

Accepted Answer

Cafe-au-lait spots

Answer

Proptosis

Answer

Hypertension

Answer

Hepatomegaly

Question 4

A child presents with hemihypertrophy (left arm and leg appear larger than the right) and aniridia. There is no family history of aniridia or hemihypertrophy. Which of the following is the most likely associated major abnormality in this patient?

Accepted Answer

Wilms tumor

Answer

Deafness

Answer

Seizures

Answer

Congestive heart failure

Question 5

A 1.5-year-old female presents with excessive head enlargement, feeding intolerance, and severe malnutrition. MRI suggests a medulloblastoma causing obstructive hydrocephalus. Which of the following represents an irrational management approach for this patient?

Accepted Answer

Radiotherapy of 35-40 Gy to the whole craniospinal axis.

Answer

Craniotomy with subtotal tumor excision, leaving the layer of tumor adherent to the colliculus.

Answer

Ventriculoperitoneal shunt placement.

Answer

Administration of CCNU and vincristine as chemotherapy.

Question 6

All are true about the presentation of Wilms tumor, except?

Accepted Answer

Bilateral involvement is common

Answer

Painless mass

Answer

Fever

Answer

Can present in infancy

Question 7

Which of the following is NOT a favorable prognostic marker in pediatric acute lymphoblastic leukemia (ALL)?

Accepted Answer

Hypodiploidy

Answer

2-10 years of age

Answer

Low WBC count

Answer

t(12;21)

Question 8

A 9-year-old boy presents with fever, feeling unwell, and easy bruising on his legs. On examination, he is pale, with a blood pressure of 100/60 mm Hg, pulse of 100/min, and a temperature of 37.8°C. His lungs are clear, his abdomen is soft with a palpable spleen, and there are petechiae and bruises on his legs. His CBC reveals a hemoglobin of 8.5 g/dL, WBC of 17,000/mL, and platelets of 30,000/mL. A blood film reveals neutropenia and leukemic lymphoblasts. What is the most appropriate initial diagnostic test to confirm the diagnosis?

Accepted Answer

Bone marrow aspirate and biopsy

Answer

Monospot test

Answer

CT scan of the abdomen

Answer

Chest x-ray

Question 9

Which of the following is a definite marker for hepatoblastoma?

Accepted Answer

Alpha-fetoprotein (AFP)

Answer

Lactate dehydrogenase (LDH)

Answer

Human chorionic gonadotropin (HCG)

Answer

Alkaline phosphatase

Question 10

A 9-year-old female child presents with a history of headache and visual disturbances. What is the most likely diagnosis?

Accepted Answer

Craniopharyngioma

Answer

Pituitary macroadenoma

Answer

Hypothalamic hamartoma

Answer

Optic glioma

Oncology — MCQs

Oncology — MCQs

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