Oncology — MCQs

A 2-year-old boy presents with abnormal pigmentation around the eyes, abnormal eye movements characterized by spontaneous bursts of non-rhythmic conjugate eye movements in various directions, hypotonia, myoclonus, and an abdominal mass. Which of the following drugs is approved for this clinical scenario?

Oncology Indian Medical PG Practice Questions and MCQs

Question 91: Which syndrome is associated with Acute Myeloid Leukemia (AML) and Acute Lymphoblastic Leukemia (ALL)?

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

Explanation: **Explanation:** The correct answer is **Turner Syndrome (45, XO)**. While Turner syndrome is primarily known for physical findings like short stature and webbed neck, it is associated with an increased risk of both **Acute Lymphoblastic Leukemia (ALL)** and **Acute Myeloid Leukemia (AML)**. The underlying mechanism is thought to involve chromosomal instability and the loss of tumor suppressor genes on the X chromosome. **Analysis of Options:** * **Down Syndrome (Trisomy 21):** This is the most common chromosomal disorder associated with leukemia. However, it has a very specific pattern: a 10-20 fold increased risk of ALL and a unique association with **AML-M7 (Acute Megakaryoblastic Leukemia)**, often preceded by Transient Myeloproliferative Disorder (TMD). * **Noonan Syndrome:** This "male version of Turner" is caused by mutations in the RAS-MAPK pathway. It is specifically associated with **Juvenile Myelomonocytic Leukemia (JMML)**, not typically a balanced risk of ALL/AML. * **Klinefelter Syndrome (47, XXY):** This syndrome is primarily associated with an increased risk of **extragonadal germ cell tumors** (specifically mediastinal) and breast cancer, rather than a significant predisposition to childhood leukemias. **High-Yield Clinical Pearls for NEET-PG:** * **Down Syndrome:** Under age 3, AML (M7) is more common; over age 3, ALL is more common. * **Fanconi Anemia:** Highest risk for **AML** and squamous cell carcinomas. * **Bloom Syndrome & Ataxia-Telangiectasia:** High risk for both ALL and AML due to DNA repair defects. * **Li-Fraumeni Syndrome (p53 mutation):** Associated with ALL, soft tissue sarcomas, and adrenocortical carcinoma.

Question 92: Which of the following statements about Wilm's tumor is true?

A. Always unilateral
B. Associated with poor prognosis
C. Most common site of metastasis is bone
D. Radiosensitive (Correct Answer)

Explanation: **Explanation:** **Wilms' Tumor (Nephroblastoma)** is the most common primary renal malignancy in children, typically presenting as an asymptomatic abdominal mass. **Why Option D is correct:** Wilms' tumor is highly **radiosensitive** and **chemosensitive**. While surgery (radical nephrectomy) is the primary treatment, radiotherapy is a crucial adjuvant modality, especially for advanced stages (Stage III and IV) or unfavorable histology, to achieve local control and treat pulmonary metastases. **Why other options are incorrect:** * **Option A:** While 90-95% of cases are unilateral, **5-10% are bilateral** (synchronous or metachronous), often associated with genetic syndromes like WAGR or Denys-Drash. * **Option B:** It has an **excellent prognosis**, with overall survival rates exceeding 90% for favorable histology due to its high sensitivity to multimodal therapy. * **Option C:** The most common site of distant metastasis is the **Lungs**, followed by the liver. Bone and brain metastases are rare (unlike Neuroblastoma, which frequently involves bone). **High-Yield Clinical Pearls for NEET-PG:** * **Genetics:** Associated with mutations in the **WT1 gene** (Chromosome 11p13). * **Syndromes:** * **WAGR:** Wilms, Aniridia, Genitourinary anomalies, Retardation. * **Beckwith-Wiedemann:** Macrosomia, Macroglossia, Omphalocele, and Wilms. * **Pathology:** Characterized by a **triphasic morphology** (Blastemal, Stromal, and Epithelial cells). * **Clinical Sign:** Hypertension may occur due to increased renin production. * **Important Contraindication:** Avoid vigorous palpation of the abdomen to prevent tumor rupture and peritoneal seeding.

Question 93: Which of the following is the most common malignant tumor of infancy?

A. Neuroblastoma (Correct Answer)
B. Wilm's tumor
C. Polycystic kidney disease
D. Renal cell carcinoma

Explanation: **Explanation:** **Neuroblastoma** is the most common extracranial solid malignant tumor of childhood and the **most common malignancy of infancy** (children under 1 year of age). It originates from primordial neural crest cells of the sympathetic nervous system, most frequently occurring in the adrenal medulla (40%) or the paraspinal sympathetic ganglia. Its high incidence in infancy is a classic NEET-PG fact, often associated with the characteristic "blueberry muffin" skin nodules in neonates. **Analysis of Incorrect Options:** * **Wilms’ Tumor (Nephroblastoma):** While it is the most common primary renal tumor in children, its peak incidence is between **2 to 5 years** of age. It is rare in the neonatal period and infancy compared to neuroblastoma. * **Polycystic Kidney Disease (PKD):** This is a genetic disorder characterized by the growth of numerous cysts in the kidneys. It is a **benign (non-malignant)** structural condition, not a tumor. * **Renal Cell Carcinoma (RCC):** This is primarily a tumor of **adulthood**. It is extremely rare in the pediatric population and almost never seen in infancy. **High-Yield Clinical Pearls for NEET-PG:** * **Most common overall childhood cancer:** Acute Lymphoblastic Leukemia (ALL). * **Most common solid tumor in children:** Brain tumors (e.g., Medulloblastoma). * **Most common extracranial solid tumor:** Neuroblastoma. * **Diagnostic Marker:** Elevated urinary catecholamines (VMA and HVA). * **Homer-Wright Rosettes:** Characteristic histopathological finding in Neuroblastoma. * **Opsoclonus-Myoclonus Syndrome:** A classic paraneoplastic syndrome associated with Neuroblastoma ("Dancing eyes, dancing feet").

Question 94: Which of the following is used in the treatment of ALL?

A. Vincristine
B. L-asparaginase
C. Prednisolone
D. All of the above (Correct Answer)

Explanation: **Explanation:** Acute Lymphoblastic Leukemia (ALL) is the most common pediatric malignancy. Its management involves a multi-drug chemotherapy regimen divided into phases: Induction, Consolidation, and Maintenance. The goal of the **Induction phase** is to achieve complete remission, and it typically utilizes a combination of the drugs listed in the options. * **Vincristine (Option A):** A vinca alkaloid that inhibits microtubule formation (mitotic inhibitor). It is a backbone of ALL therapy. A high-yield side effect to remember is **peripheral neuropathy** (foot drop/constipation). * **L-asparaginase (Option B):** An enzyme that depletes asparagine. Since leukemic cells cannot synthesize their own asparagine, they undergo apoptosis. Key side effects include **pancreatitis** and **hypofibrinogenemia**. * **Prednisolone (Option C):** Glucocorticoids are lymphocytotoxic and are essential in the initial induction phase to rapidly reduce the blast burden. Since all three agents are standard components of the induction protocol (often referred to as the **VPL protocol**), the correct answer is **All of the above**. **Clinical Pearls for NEET-PG:** * **Maintenance Therapy:** Usually consists of **6-Mercaptopurine (6-MP)** and **Methotrexate**. * **CNS Prophylaxis:** Essential in ALL to prevent relapse; typically involves **Intrathecal Methotrexate**. * **Tumor Lysis Syndrome (TLS):** A common complication during the initiation of treatment; managed with aggressive hydration, Allopurinol, or Rasburicase. * **Prognosis:** The presence of the **Philadelphia chromosome t(9;22)** carries a poor prognosis in ALL, whereas **t(12;21)** carries a favorable prognosis.

Question 95: A 4-year-old girl presents with an abdominal lump. Bone scan is indicated in which of the following conditions?

A. Wilm's tumor
B. Neuroblastoma (Correct Answer)
C. Rhabdomyosarcoma
D. Primitive neuroectodermal tumor (PNET)

Explanation: **Explanation:** In pediatric oncology, the choice of imaging depends on the tumor’s characteristic metastatic pattern. **Neuroblastoma** is the correct answer because it is a "bone-seeking" tumor. Approximately 60–70% of patients present with metastatic disease at diagnosis, frequently involving the cortical bone and bone marrow. A **Technetium-99m (Tc-99m) bone scan** or an **MIBG scan** (more specific) is mandatory to evaluate the extent of skeletal involvement and for staging (International Neuroblastoma Staging System). **Analysis of Options:** * **Wilms’ Tumor (Option A):** This is the most common primary renal tumor in children. It primarily metastasizes to the **lungs** (via hematogenous spread) and liver. Bone metastasis is extremely rare in classic Wilms’ tumor; if bone lesions are present, one should suspect *Clear Cell Sarcoma of the Kidney*. * **Rhabdomyosarcoma (Option C):** While it can spread to bones, the initial staging focuses on local invasion, regional lymph nodes, and lungs. Bone scans are not routinely the primary diagnostic indicator for an abdominal lump in this context. * **PNET (Option D):** Now considered part of the Ewing Sarcoma family, PNET involves bone primarily or as a metastatic site, but in the context of a 4-year-old with an **abdominal lump**, Neuroblastoma is the classic association for mandatory bone surveys. **High-Yield Clinical Pearls for NEET-PG:** * **Neuroblastoma:** Originates from neural crest cells; most common site is the adrenal medulla. Look for **elevated urinary VMA/HVA** and "dancing eyes-dancing feet" (opsoclonus-myoclonus syndrome). * **Wilms’ Tumor:** Associated with WAGR syndrome, Beckwith-Wiedemann syndrome, and Denys-Drash syndrome. It **rarely crosses the midline**, unlike Neuroblastoma. * **Imaging Choice:** MIBG scan is the gold standard for Neuroblastoma, but Tc-99m bone scan is used if MIBG is unavailable or the tumor is non-avid.

Question 96: A 1-year-old child presents with an abdominal mass with calcification. What are the most likely possibilities?

A. Neuroblastoma
B. Wilm's tumor (Correct Answer)
C. Nephronophthisis
D. Pheochromocytoma

Explanation: ### Explanation The presence of an **abdominal mass with calcification** in a 1-year-old child is a classic presentation for pediatric solid tumors. While both Neuroblastoma and Wilms' tumor are common, the key differentiator in this question is the age and the radiological finding. **Why Wilms' Tumor is the Correct Answer:** Wilms' tumor (Nephroblastoma) is the most common renal tumor in children. While calcification is more frequent in Neuroblastoma (85-90%), it occurs in approximately **10-15% of Wilms' tumors**. In the context of NEET-PG questions, if an abdominal mass is specified as the primary finding in a young child, Wilms' tumor is a high-probability diagnosis. *Note: Historically, many examiners use this specific presentation to test the differentiation between renal and extra-renal masses.* **Analysis of Incorrect Options:** * **Neuroblastoma (Option A):** This is the most common extracranial solid tumor in children. It typically presents with **stippled calcification** (90% on CT) and crosses the midline. While a very strong contender, in many standardized formats, Wilms' is favored if the mass is specifically intra-renal. * **Nephronophthisis (Option C):** This is a genetic cystic kidney disease leading to medullary cysts and chronic renal failure. It presents with polyuria and anemia, not a calcified abdominal mass. * **Pheochromocytoma (Option D):** Extremely rare in a 1-year-old. It presents with the classic triad of episodic headache, sweating, and tachycardia due to catecholamine excess. **High-Yield Clinical Pearls for NEET-PG:** * **Wilms' Tumor:** Does **not** usually cross the midline; associated with WAGR syndrome, Denys-Drash, and Beckwith-Wiedemann syndrome. * **Neuroblastoma:** Often crosses the midline; associated with increased urinary VMA/HVA levels and "Dancing eyes-dancing feet" (Opsoclonus-myoclonus) syndrome. * **Calcification Rule:** If calcification is **fine/stippled**, think Neuroblastoma. If it is **chunky/peripheral**, think Wilms' tumor.

Question 97: What is the ideal timing for radiotherapy for Wilms' tumor after surgery?

A. Within 10 days (Correct Answer)
B. Within 2 weeks
C. Within 2 months
D. Any time after surgery

Explanation: **Explanation:** The management of Wilms’ tumor (Nephroblastoma) involves a multimodal approach including surgery, chemotherapy, and sometimes radiotherapy. The timing of postoperative radiotherapy is critical for preventing local recurrence. **1. Why "Within 10 days" is correct:** According to the **NWTS (National Wilms Tumor Study)** protocols, if radiotherapy is indicated (typically for Stage III and IV disease), it should ideally be initiated **within 10 days** of surgery. Delays beyond 10 days are associated with a significantly increased risk of local abdominal recurrence and poorer survival outcomes. The goal is to eliminate residual microscopic disease before tumor cell repopulation occurs. **2. Why the other options are incorrect:** * **Within 2 weeks (Option B):** While close to the 10-day mark, clinical studies show that the risk of recurrence begins to rise sharply after the 10th postoperative day. Therefore, 10 days is the strict oncological benchmark. * **Within 2 months (Option C):** This is far too late. By two months, any residual tumor cells would have likely proliferated, rendering the radiotherapy less effective and increasing the risk of metastasis. * **Any time after surgery (Option D):** Radiotherapy is time-sensitive. Indiscriminate timing ignores the biological behavior of the tumor and the necessity of early local control. **3. NEET-PG High-Yield Pearls:** * **Most common presentation:** Asymptomatic abdominal mass (does not cross the midline). * **Staging:** Stage III is defined by residual non-hematogenous tumor confined to the abdomen (e.g., lymph node involvement or peritoneal seeding). * **Radiotherapy Indications:** Generally reserved for Stage III and Stage IV disease with favorable histology, or Stage II-IV with unfavorable (anaplastic) histology. * **Common Site of Metastasis:** Lungs (Canonical "Cannonball" appearance on X-ray, though more common in RCC). * **Associated Syndromes:** WAGR, Denys-Drash, and Beckwith-Wiedemann syndromes.

Question 98: A 1-year-old child presents with fever, fatigue, irritability, flushing, eating difficulties, and bone pain. On examination, an abdominal mass is palpable. What is the most likely diagnosis?

A. Lymphoma
B. Acute lymphoblastic leukemia (ALL)
C. Neuroblastoma (Correct Answer)
D. Wilm's tumor

Explanation: **Explanation:** The clinical presentation of a 1-year-old child with an abdominal mass, bone pain, and systemic symptoms like irritability and flushing strongly points toward **Neuroblastoma**. **Why Neuroblastoma is the correct answer:** Neuroblastoma is the most common extracranial solid tumor of childhood, typically arising from the adrenal medulla or sympathetic chain. It is derived from **neural crest cells**. * **Age:** Most cases occur in children under 2 years. * **Systemic Symptoms:** "Flushing" and "irritability" are classic signs of **catecholamine excess** (VMA/HVA in urine). * **Bone Pain:** This indicates metastasis to the bone or bone marrow (Hutchinson-type), a common feature at presentation. * **Abdominal Mass:** Unlike Wilms' tumor, the mass in neuroblastoma is usually irregular, firm, and **crosses the midline**. **Why other options are incorrect:** * **Wilms' Tumor (Nephroblastoma):** Typically presents in older children (3–4 years). The mass is smooth, firm, and **rarely crosses the midline**. Bone pain and catecholamine-related flushing are not characteristic. * **Acute Lymphoblastic Leukemia (ALL):** While it presents with fever, fatigue, and bone pain, the primary finding is usually lymphadenopathy and hepatosplenomegaly rather than a discrete, large abdominal mass. * **Lymphoma:** Burkitt lymphoma can present as an abdominal mass, but it is more common in older children and does not typically cause catecholamine-mediated flushing. **NEET-PG High-Yield Pearls:** * **Opsoclonus-Myoclonus Syndrome:** A paraneoplastic "dancing eyes, dancing feet" syndrome associated with neuroblastoma. * **Homer-Wright Rosettes:** The characteristic histological finding. * **N-myc Amplification:** The most important prognostic factor (indicates poor prognosis). * **Periorbital Ecchymosis:** Known as "Raccoon eyes," seen due to orbital metastasis.

Question 99: What is the commonest sarcoma in children?

A. Rhabdomyosarcoma (Correct Answer)
B. Lipoma
C. Angiosarcoma
D. Fibrosarcoma

Explanation: **Explanation:** **Rhabdomyosarcoma (RMS)** is the correct answer as it is the most common soft tissue sarcoma in the pediatric population, accounting for approximately 50% of all soft tissue sarcomas in children and adolescents. It originates from primitive mesenchymal cells that are committed to skeletal muscle lineage. * **Why Rhabdomyosarcoma is correct:** It shows a bimodal age distribution, peaking between ages 2–6 years and again in adolescence. The two main histological subtypes are **Embryonal** (most common, better prognosis, often found in the head/neck or genitourinary tract) and **Alveolar** (worse prognosis, associated with t(2;13) or t(1;13) translocations). * **Why other options are incorrect:** * **Lipoma:** This is a benign fatty tumor. While common in adults, it is not a malignancy (sarcoma). * **Angiosarcoma:** A rare malignant tumor of vascular origin, typically seen in elderly adults, often following radiation or chronic lymphedema. * **Fibrosarcoma:** Though it can occur in infants (Congenital Fibrosarcoma), it is significantly less common than RMS. **High-Yield Clinical Pearls for NEET-PG:** * **Commonest site:** Head and Neck (specifically the orbit). * **Sarcoma Botryoides:** A variant of embryonal RMS found in hollow organs (vagina/bladder) presenting as a "grape-like" mass. * **Diagnostic Marker:** Desmin, Myogenin, and MyoD1 (Immunohistochemistry). * **Staging:** Unlike many tumors, RMS staging utilizes both the TNM system and a "Clinical Grouping" based on the extent of initial surgical resection.

Question 100: A 2-year-old boy presents with abnormal pigmentation around the eyes, abnormal eye movements characterized by spontaneous bursts of non-rhythmic conjugate eye movements in various directions, hypotonia, myoclonus, and an abdominal mass. Which of the following drugs is approved for this clinical scenario?

A. Ipilimumab
B. Atezolizumab
C. Dinutuximab (Correct Answer)
D. Olarutumab

Explanation: ### **Explanation** **Clinical Diagnosis: Neuroblastoma with Opsoclonus-Myoclonus-Ataxia Syndrome (OMS)** The clinical presentation of a 2-year-old with an **abdominal mass** and **periorbital ecchymosis** ("raccoon eyes" due to orbital metastases) is classic for **Neuroblastoma**. The "spontaneous bursts of non-rhythmic conjugate eye movements" (opsoclonus) combined with myoclonus and hypotonia describes **Opsoclonus-Myoclonus-Ataxia Syndrome**, a well-known paraneoplastic manifestation of neuroblastoma. **Why Dinutuximab is Correct:** **Dinutuximab** is a monoclonal antibody that targets **GD2**, a glycolipid highly expressed on the surface of neuroblastoma cells. It is FDA-approved for the treatment of high-risk neuroblastoma in pediatric patients who have achieved at least a partial response to prior first-line multi-agent, multimodality therapy. It works via antibody-dependent cell-mediated cytotoxicity (ADCC). **Analysis of Incorrect Options:** * **A. Ipilimumab:** A CTLA-4 inhibitor used primarily in adult malignancies like melanoma and renal cell carcinoma. * **B. Atezolizumab:** A PD-L1 inhibitor used for various adult cancers (e.g., urothelial carcinoma, NSCLC) but not the standard of care for neuroblastoma. * **D. Olarutumab:** A PDGF receptor-alpha antagonist previously used for soft tissue sarcomas (withdrawn from the market in many regions due to lack of efficacy in Phase 3 trials). **High-Yield Clinical Pearls for NEET-PG:** * **Neuroblastoma:** Most common extracranial solid tumor of childhood; arises from neural crest cells (sympathetic chain or adrenal medulla). * **Biomarkers:** Elevated urinary catecholamines (VMA and HVA). * **Genetic Marker:** **N-myc (MYCN) amplification** is the most important poor prognostic factor. * **Opsoclonus-Myoclonus Syndrome:** Often associated with favorable-prognosis neuroblastomas, but carries significant long-term neurologic morbidity. * **Homer-Wright Rosettes:** Characteristic histopathology finding.

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Bone Tumors

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Oncology — MCQs

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