Oncology — MCQs

Oncology Indian Medical PG Practice Questions and MCQs

Question 61: What is the commonest intra-abdominal tumor in children between 2 and 5 years of age?

A. Neuroblastoma (Correct Answer)
B. Wilm's tumor
C. Hepatoblastoma
D. Lymphoma

Explanation: **Explanation:** **Neuroblastoma** is the most common extracranial solid tumor of childhood and the **most common intra-abdominal tumor** overall in children. It originates from primordial neural crest cells of the sympathetic nervous system, most frequently occurring in the adrenal medulla (approx. 40%). While it can occur at any age, the median age of diagnosis is 19 months, making it the predominant abdominal malignancy in the 2–5 year age group. **Analysis of Options:** * **Wilms’ Tumor (Nephroblastoma):** This is the most common *renal* tumor in children. While it also peaks between 2 and 5 years, it is statistically less frequent than neuroblastoma when considering all intra-abdominal sites. A key clinical differentiator is that Wilms’ tumor usually presents as a smooth mass that **does not cross the midline**, whereas neuroblastoma is often irregular and **crosses the midline**. * **Hepatoblastoma:** This is the most common primary liver tumor in children, but it is significantly rarer than neuroblastoma and typically presents earlier (usually before age 3). * **Lymphoma:** While abdominal (Burkitt) lymphoma occurs in children, it is less common than the primary solid organ tumors mentioned above and often presents with acute bowel symptoms (intussusception). **High-Yield Clinical Pearls for NEET-PG:** * **Biomarker:** Elevated urinary catecholamines (VMA and HVA) are found in 90% of neuroblastoma cases. * **Opsoclonus-Myoclonus Syndrome:** A classic paraneoplastic syndrome associated with neuroblastoma ("dancing eyes, dancing feet"). * **Homer-Wright Rosettes:** The characteristic histopathological finding. * **N-myc Amplification:** The most important prognostic factor (indicates poor prognosis). * **Staging:** Look for "Pepper Syndrome" (massive liver involvement in infants) and "Blueberry Muffin" skin nodules.

Question 62: What is the most common presentation of neuroblastoma?

A. Lytic lesion in the skull with suture diastasis (Correct Answer)
B. Lung metastasis
C. Renal invasion
D. Secondaries in the brain

Explanation: **Explanation:** Neuroblastoma is the most common extracranial solid tumor of childhood, arising from primordial neural crest cells. While the primary tumor is most commonly found in the adrenal medulla, neuroblastoma is notorious for being **highly metastatic** at the time of diagnosis (seen in approximately 60-70% of cases). **Why Option A is Correct:** Neuroblastoma has a unique predilection for metastasizing to the **bones and bone marrow**. Specifically, it frequently involves the **skull and orbits**. When the tumor involves the skull, it causes characteristic **punched-out lytic lesions**. As these lesions grow, they increase intracranial pressure and infiltrate the cranial sutures, leading to **suture diastasis** (widening of the sutures). This is a classic radiological and clinical hallmark of metastatic neuroblastoma. **Why Other Options are Incorrect:** * **B. Lung metastasis:** Unlike Wilms tumor (where the lung is the most common site of spread), neuroblastoma rarely involves the lungs. * **C. Renal invasion:** Neuroblastoma is an extra-renal tumor. While it may displace the kidney (causing the "drooping lily" sign), it rarely invades the renal parenchyma. * **D. Secondaries in the brain:** Metastasis to the brain parenchyma is extremely rare in neuroblastoma; it typically involves the skull bones and the meningeal surfaces instead. **High-Yield Clinical Pearls for NEET-PG:** * **Opsoclonus-Myoclonus Syndrome:** A paraneoplastic syndrome ("dancing eyes, dancing feet") associated with favorable prognosis. * **Hutchinson’s Syndrome:** Orbital metastasis leading to proptosis and periorbital ecchymosis (**"Raccoon eyes"**). * **Pepper Syndrome:** Massive hepatomegaly due to liver metastasis (common in Stage 4S). * **Diagnosis:** Elevated urinary catecholamines (VMA and HVA) and **N-myc amplification** (the most important poor prognostic factor).

Question 63: Which of the following is the most common solid tumor of childhood?

A. Ganglioneuroma
B. Neuroblastoma (Correct Answer)
C. Retinoblastoma
D. Wilms tumor

Explanation: **Explanation:** The correct answer is **Neuroblastoma**. In pediatric oncology, it is crucial to distinguish between hematological and solid malignancies. While **Leukemia** is the most common childhood cancer overall, **Neuroblastoma** is the most common **extracranial solid tumor** of childhood. It originates from primordial neural crest cells of the sympathetic nervous system, most frequently occurring in the adrenal medulla (approx. 40%) or the paraspinal sympathetic ganglia. **Analysis of Options:** * **Neuroblastoma (Correct):** It accounts for roughly 7-10% of all pediatric cancers and is the most common solid tumor in infants. * **Ganglioneuroma:** This is the benign, fully differentiated end of the neuroblastic tumor spectrum. While related to neuroblastoma, it is far less common. * **Retinoblastoma:** This is the most common primary intraocular tumor in children, but its overall incidence is lower than neuroblastoma and Wilms tumor. * **Wilms Tumor (Nephroblastoma):** This is the most common primary **renal** tumor in children. While highly common, it ranks second to neuroblastoma among extracranial solid tumors. **High-Yield Clinical Pearls for NEET-PG:** 1. **Age Factor:** Neuroblastoma is the most common malignancy in the **neonatal period**. 2. **Clinical Sign:** It typically presents as a firm, irregular abdominal mass that **crosses the midline** (unlike Wilms tumor, which usually does not cross the midline). 3. **Biomarkers:** Elevated urinary catecholamines (**VMA and HVA**) are found in 90% of cases. 4. **Opsoclonus-Myoclonus Syndrome:** A classic paraneoplastic syndrome associated with neuroblastoma ("dancing eyes, dancing feet"). 5. **N-myc Amplification:** The most important poor prognostic genetic marker.

Question 64: A 5-year-old child presents with a renal mass. Considering the most common cause in this age group, which of the following statements is not true?

A. Triphasic histology
B. Risk of WAGR syndrome
C. Bilateral involvement
D. Increased urine vanillylmandelic acid (VMA) levels (Correct Answer)

Explanation: **Explanation:** The clinical presentation of a renal mass in a 5-year-old child most likely points to **Wilms Tumor (Nephroblastoma)**, which is the most common primary renal malignancy in children. **Why Option D is the Correct Answer (False Statement):** Increased urine **Vanillylmandelic Acid (VMA)** and Homovanillic Acid (HVA) levels are characteristic markers for **Neuroblastoma**, not Wilms tumor. Neuroblastoma arises from neural crest cells (sympathetic chain or adrenal medulla) and produces catecholamines. Wilms tumor is a renal parenchymal tumor and does not typically produce these metabolites. **Analysis of Incorrect Options (True Statements for Wilms Tumor):** * **A. Triphasic histology:** This is the classic pathological hallmark of Wilms tumor, consisting of three components: **Blastemal, Stromal, and Epithelial** cells. * **B. Risk of WAGR syndrome:** Wilms tumor is associated with several genetic syndromes. **WAGR** stands for **W**ilms tumor, **A**niridia, **G**enitourinary anomalies, and mental **R**etardation (associated with WT1 gene deletion on chromosome 11p13). * **C. Bilateral involvement:** While most cases are unilateral, approximately **5-7%** of children present with synchronous or metachronous bilateral Wilms tumor (Stage V). **High-Yield Clinical Pearls for NEET-PG:** * **Most common presentation:** Asymptomatic, firm, smooth abdominal mass that **does not cross the midline** (unlike Neuroblastoma). * **Beckwith-Wiedemann Syndrome:** Another major association (WT2 gene) featuring macroglossia, hemihypertrophy, and omphalocele. * **Investigation of choice:** Contrast-Enhanced CT (CECT) abdomen. * **Most important prognostic factor:** Histology (Favorable vs. Anaplastic). * **Common site of metastasis:** Lungs (presents as "cannonball" appearance on X-ray).

Question 65: What is the most common tumor of infancy?

A. Lymphangioma
B. Rhabdomyoma
C. Hemangioma (Correct Answer)
D. Lipoma

Explanation: **Explanation:** **Infantile Hemangioma** is the correct answer as it is the most common benign tumor of infancy, affecting approximately 4–5% of all infants. These are vascular tumors characterized by a rapid proliferative phase (usually in the first few months of life) followed by a slow spontaneous involution phase (the "50% by age 5, 90% by age 9" rule). **Analysis of Options:** * **A. Lymphangioma:** These are congenital malformations of the lymphatic system (e.g., cystic hygroma). While common in the neck region of infants, their overall incidence is significantly lower than hemangiomas. * **B. Rhabdomyoma:** This is the most common primary **cardiac** tumor in infants and children, often associated with Tuberous Sclerosis. It is not the most common tumor overall. * **C. Hemangioma (Correct):** As noted, these are the most frequent neoplasms of childhood. * **D. Lipoma:** These are common benign soft tissue tumors in adults but are relatively rare in the neonatal and infantile periods. **High-Yield Clinical Pearls for NEET-PG:** * **Most common benign tumor of infancy:** Hemangioma. * **Most common malignant extracranial solid tumor of childhood:** Neuroblastoma. * **Most common childhood malignancy overall:** Acute Lymphoblastic Leukemia (ALL). * **Management:** Most hemangiomas require observation only. However, if they obstruct vision (amblyopia) or the airway, the first-line medical treatment is **Oral Propranolol**. * **Kasabach-Merritt Syndrome:** This is associated with *Tufted Angioma* or *Kaposiform Hemangioendothelioma*, NOT typical infantile hemangiomas. It involves profound thrombocytopenia due to platelet trapping.

Question 66: What is the most common primary lung cancer in children?

A. Metastasis
B. Bronchial adenoma (Correct Answer)
C. Adenocarcinoma
D. Pulmonary blastoma

Explanation: **Explanation:** Primary lung malignancies are exceedingly rare in the pediatric population, accounting for less than 1% of all childhood tumors. **1. Why Bronchial Adenoma is Correct:** Among primary lung tumors in children, **Bronchial Adenoma** (specifically **Carcinoid tumors**) is the most common. These are low-grade malignant epithelial tumors arising from the bronchial mucosa. They typically present with symptoms of bronchial obstruction, such as persistent cough, localized wheezing, or recurrent pneumonia in the same lobe. **2. Analysis of Incorrect Options:** * **Metastasis (Option A):** While metastatic lesions (from Osteosarcoma, Wilms tumor, or Ewing sarcoma) are the **most common cause of a lung mass** in children, the question specifically asks for the most common **primary** lung cancer. * **Adenocarcinoma (Option C):** This is the most common primary lung cancer in adults but is extremely rare in children. * **Pulmonary Blastoma (Option D):** This is a rare, highly aggressive primary lung tumor of childhood containing mesenchymal and epithelial elements. While it is a classic pediatric lung tumor, its incidence is much lower than that of bronchial adenomas. **3. NEET-PG High-Yield Pearls:** * **Most common lung tumor in children:** Metastasis (Secondary). * **Most common primary lung tumor in children:** Bronchial Adenoma (Carcinoid is the most frequent subtype). * **Most common malignant primary lung tumor:** Bronchial Carcinoid. * **Pleuropulmonary Blastoma (PPB):** Associated with the **DICER1** gene mutation; it is a mesenchymal tumor of childhood, often presenting as a cystic or solid lung mass. * **Clinical Presentation:** Any child with "unresolved pneumonia" or "monophonic wheeze" that does not respond to standard therapy should be evaluated for an endobronchial lesion like a carcinoid tumor.

Question 67: Which of the following is NOT associated with Wilm's tumor?

A. Aniridia
B. Genitourinary abnormalities
C. Cataract (Correct Answer)
D. Hemihypertrophy

Explanation: **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 Cataract is the correct answer:** Cataract is **not** a recognized component of Wilms tumor-associated syndromes. While ocular abnormalities like aniridia are classic, cataracts are typically associated with other pediatric conditions (e.g., congenital rubella, galactosemia, or Lowe syndrome) rather than nephroblastoma. **Analysis of Incorrect Options:** * **Aniridia (Option A):** This is the absence of the iris. It is a hallmark of **WAGR syndrome** (Wilms tumor, Aniridia, Genitourinary anomalies, and mental Retardation). * **Genitourinary abnormalities (Option B):** These include cryptorchidism, hypospadias, or gonadal dysgenesis, commonly seen in both **WAGR** and **Denys-Drash syndrome**. * **Hemihypertrophy (Option D):** This refers to the overgrowth of one side of the body. It is a key feature of **Beckwith-Wiedemann Syndrome (BWS)**, a macrosomic overgrowth disorder with a high predisposition to Wilms tumor. **Clinical Pearls for NEET-PG:** * **WAGR Syndrome:** Caused by a microdeletion at **11p13** (WT1 gene). * **Denys-Drash Syndrome:** Characterized by the triad of Wilms tumor, pseudohermaphroditism, and early-onset renal failure (mesangial sclerosis). * **Beckwith-Wiedemann Syndrome:** Associated with **11p15** (WT2 gene), macroglossia, omphalocele, and hemihypertrophy. * **Screening:** Children with these syndromes require serial abdominal ultrasounds every 3 months until age 7-8 for early detection of Wilms tumor.

Question 68: Which of the following findings is not compatible with a diagnosis of juvenile myelomonocytic leukemia?

A. Peripheral blood monocytosis, more than 1 x 10^9/L
B. Increased hemoglobin F levels for age
C. Presence of bcr/abl fusion gene (Correct Answer)
D. GM-CSF hypersensitivity of myeloid progenitors in vitro

Explanation: **Explanation:** Juvenile Myelomonocytic Leukemia (JMML) is a unique, aggressive clonal hematopoietic stem cell disorder of childhood that overlaps features of both myelodysplastic and myeloproliferative syndromes. **1. Why Option C is the Correct Answer:** The **bcr/abl fusion gene** (Philadelphia chromosome) is the hallmark of **Chronic Myeloid Leukemia (CML)**. By definition, JMML is a **bcr/abl negative** disease. The presence of this translocation excludes a diagnosis of JMML and confirms a diagnosis of CML (which is rare in children). JMML is instead characterized by mutations in the RAS pathway (PTPN11, NF1, NRAS, KRAS, or CBL). **2. Analysis of Incorrect Options:** * **Option A (Monocytosis):** Absolute monocytosis (>1 x 10⁹/L) is a mandatory diagnostic criterion for JMML. * **Option B (Increased HbF):** Elevated Hemoglobin F for age is a classic "major" diagnostic criterion. It reflects the "juvenile" or fetal-like nature of the malignant clone. * **Option D (GM-CSF Hypersensitivity):** This is a pathognomonic laboratory finding. Myeloid progenitors in JMML show spontaneous proliferation and exquisite sensitivity to Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF) in vitro. **Clinical Pearls for NEET-PG:** * **Age Group:** Usually occurs in children <2 years of age. * **Clinical Features:** Hepatosplenomegaly, lymphadenopathy, and skin rashes (xanthomas or café-au-lait spots, especially if associated with Neurofibromatosis type 1). * **Diagnostic Clue:** Absence of the Philadelphia chromosome is essential to differentiate it from pediatric CML. * **Treatment:** Hematopoietic stem cell transplant (HSCT) is the only curative treatment.

Question 69: Chronic myeloid leukemia (CML) in children is associated with which of the following genetic syndromes?

A. Down syndrome (Correct Answer)
B. Klinefelter syndrome
C. Marranos syndrome
D. Turner syndrome

Explanation: **Explanation:** **1. Why Down Syndrome is Correct:** Children with Down syndrome (Trisomy 21) have a significantly increased risk (approximately 10–20 fold) of developing leukemia compared to the general population. While Down syndrome is most classically associated with **Acute Megakaryoblastic Leukemia (AMKL/M7)** and **Transient Myeloproliferative Disorder (TMD)** in neonates, it also predisposes children to both Acute Lymphoblastic Leukemia (ALL) and **Chronic Myeloid Leukemia (CML)**. The genetic instability and overexpression of genes on chromosome 21 (such as *GATA1* mutations in myeloid lineages) create a pro-leukemogenic environment. **2. Why Other Options are Incorrect:** * **Klinefelter Syndrome (47, XXY):** While associated with an increased risk of germ cell tumors (specifically mediastinal) and breast cancer, it is not a primary risk factor for CML. * **Turner Syndrome (45, XO):** This syndrome is associated with an increased risk of gonadoblastoma (if Y-chromosome material is present) and certain solid tumors, but not specifically CML. * **Marranos Syndrome:** This is not a recognized genetic syndrome associated with hematological malignancies. (Note: Marfan syndrome is a connective tissue disorder with no established link to CML). **3. NEET-PG High-Yield Clinical Pearls:** * **Most common leukemia in Down Syndrome:** ALL (overall), but the *relative* risk increase is highest for AML (specifically M7). * **TMD (Transient Myeloproliferative Disorder):** Occurs in 10% of newborns with Down syndrome; it often resolves spontaneously but carries a risk of future AML. * **CML Hallmark:** The Philadelphia chromosome **t(9;22)** resulting in the *BCR-ABL1* fusion gene. In pediatrics, CML accounts for only 2-3% of all childhood leukemias. * **Other Syndromes linked to Leukemia:** Fanconi Anemia, Bloom Syndrome, and Ataxia-Telangiectasia.

Question 70: A child with Acute myeloid Leukemia presents with Hyperleukocytosis. Management includes all of the following, except:

A. IV fluids
B. Allopurinol
C. Alkalinization
D. Immediate induction chemotherapy (Correct Answer)

Explanation: **Explanation:** Hyperleukocytosis (defined as a WBC count >100,000/mm³) is a medical emergency in AML due to the high risk of **leukostasis** and **Tumor Lysis Syndrome (TLS)**. **Why "Immediate induction chemotherapy" is the correct (except) answer:** While treating the underlying leukemia is the ultimate goal, starting full-dose induction chemotherapy immediately in a patient with hyperleukocytosis is contraindicated. Rapid cell lysis can trigger catastrophic **Tumor Lysis Syndrome**, worsening metabolic derangements and causing acute kidney injury. The priority is to stabilize the patient, reduce blood viscosity, and manage metabolic risks *before* starting intensive chemotherapy. **Analysis of other options:** * **IV Fluids (Hyperhydration):** Essential to improve rheology (reduce viscosity) and promote the excretion of uric acid and phosphates. * **Allopurinol:** Crucial for preventing hyperuricemia by inhibiting xanthine oxidase, thereby protecting renal function from the effects of cell turnover. * **Alkalinization:** Historically used to increase the solubility of uric acid in urine (though its use is now debated and often replaced by Rasburicase, it remains a standard textbook management step for preventing urate nephropathy). **Clinical Pearls for NEET-PG:** 1. **Leukostasis** is more common in **AML** than ALL because myeloblasts are larger and less deformable than lymphoblasts. 2. **Symptoms of Leukostasis:** Primarily involve the CNS (altered sensorium, stroke) and Lungs (dyspnea, hypoxia). 3. **Exchange Transfusion/Leukapheresis:** May be considered if symptomatic leukostasis is present to rapidly reduce the blast count. 4. **Avoid Diuretics:** Unless the patient is fluid overloaded, as dehydration worsens blood viscosity. 5. **Avoid Platelet Transfusions:** Unless there is active bleeding, as it can increase "sludging" and worsen leukostasis.

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Leukemias

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Lymphomas

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CNS Tumors

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Neuroblastoma

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Wilms Tumor

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Rhabdomyosarcoma

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Bone Tumors

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Retinoblastoma

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Histiocytosis Syndromes

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Principles of Pediatric Chemotherapy

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Hematopoietic Stem Cell Transplantation

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Late Effects of Cancer Treatment

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Oncology — MCQs

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