Oncology Practice Questions

Q: A 2-year-old boy presents with an abdominal mass discovered by his foster mother. Medical evaluation reveals aniridia, hypospadias, horseshoe kidney, and hemihypertrophy. What is the most likely diagnosis?

Wilms tumor. ### Explanation **Correct Answer: B. Wilms Tumor (Nephroblastoma)** The clinical presentation is a classic description of **Wilms tumor** associated with specific congenital syndromes. Wilms tumor is the most common primary renal malignancy in children, typically presenting as an asymptomatic abdominal mass. The key to this diagnosis lies in the associated anomalies: 1. **WAGR Syndrome:** **W**ilms tumor, **A**niridia (absence of iris), **G**enitourinary anomalies (hypospadias, horseshoe kidney), and mental **R**etardation. This is caused by a microdeletion on chromosome **11p13** (WT1 gene). 2. **Beckwith-Wiedemann Syndrome:** Characterized by **hemihypertrophy**, macroglossia, and organomegaly (linked to chromosome **11p15**). **Why other options are incorrect:** * **Neuroblastoma:** While it also presents as an abdominal mass in toddlers, it typically crosses the midline, is irregular/calcified, and is associated with opsoclonus-myoclonus or periorbital ecchymosis (raccoon eyes), not aniridia or hemihypertrophy. * **Hepatoblastoma:** This is a liver tumor. While associated with Beckwith-Wiedemann, it would present with RUQ mass and elevated Alpha-fetoprotein (AFP), without renal or iris anomalies. * **Rhabdomyosarcoma:** The most common soft tissue sarcoma in children, usually presenting in the head/neck or genitourinary tract (sarcoma botryoides), but not linked to this specific constellation of congenital syndromes. **High-Yield Pearls for NEET-PG:** * **Most common site of metastasis:** Lungs (via hematogenous spread). * **Staging:** Unlike many tumors, Wilms tumor staging is based on **surgical evaluation** and pathological findings. * **Denys-Drash Syndrome:** Triad of Wilms tumor, male pseudohermaphroditism, and early-onset renal failure (mesangial sclerosis). * **Management:** Multimodal (Surgery + Chemotherapy ± Radiotherapy). In North America (NWTSG), nephrectomy is usually the first step.

Q: Neuroblastoma originates from which structure?

Adrenal medulla. **Explanation:** **Neuroblastoma** is the most common extracranial solid tumor of childhood. It originates from **primordial neural crest cells**, which are the embryological precursors to the sympathetic nervous system. 1. **Why Adrenal Medulla is Correct:** The adrenal medulla is derived from neural crest cells (specifically chromaffin cells). Because neuroblastoma arises from these sympathetic progenitor cells, the **adrenal medulla** is the most common primary site (approximately 40–50% of cases), followed by the paraspinal sympathetic ganglia. 2. **Why Other Options are Incorrect:** * **Mediastinum:** While the posterior mediastinum is the second most common site for neuroblastoma (arising from thoracic sympathetic chains), it is a *location*, not the *structure of origin*. * **Chest Wall:** Tumors of the chest wall in children are more likely to be Ewing sarcoma (Askin tumor) or rhabdomyosarcoma, not neuroblastoma. * **Peripheral Nerves:** Neuroblastoma specifically involves the *autonomic* (sympathetic) nervous system, not the general peripheral sensory or motor nerves. **High-Yield Clinical Pearls for NEET-PG:** * **Median Age:** 2 years (usually occurs in children <5 years). * **Clinical Feature:** A firm, irregular, **nontender abdominal mass that crosses the midline** (unlike Wilms tumor, which usually does not cross the midline). * **Biomarkers:** Elevated urinary catecholamines (**VMA and HVA**). * **Pathology:** Characterized by **Homer-Wright rosettes** and Small Round Blue Cells. * **Prognostic Marker:** **N-myc amplification** is the most important indicator of poor prognosis. * **Opsoclonus-Myoclonus Syndrome:** A classic paraneoplastic syndrome associated with neuroblastoma ("dancing eyes, dancing feet").

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

Radiosensitive. **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.

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

Neuroblastoma. **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").

Q: Which of the following is used in the treatment of ALL?

All of the above. **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.

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

Neuroblastoma. **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.

Q: 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?

Neuroblastoma. **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.

Q: What is the commonest sarcoma in children?

Rhabdomyosarcoma. **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 1

A 2-year-old boy presents with an abdominal mass discovered by his foster mother. Medical evaluation reveals aniridia, hypospadias, horseshoe kidney, and hemihypertrophy. What is the most likely diagnosis?

Accepted Answer

Wilms tumor

Answer

Neuroblastoma

Answer

Hepatoblastoma

Answer

Rhabdomyosarcoma

Question 2

Neuroblastoma originates from which structure?

Accepted Answer

Adrenal medulla

Answer

Mediastinum

Answer

Chest wall

Answer

Peripheral nerves

Question 3

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

Accepted Answer

Radiosensitive

Answer

Always unilateral

Answer

Associated with poor prognosis

Answer

Most common site of metastasis is bone

Question 4

Which of the following is the most common malignant tumor of infancy?

Accepted Answer

Neuroblastoma

Answer

Wilm's tumor

Answer

Polycystic kidney disease

Answer

Renal cell carcinoma

Question 5

Which of the following is used in the treatment of ALL?

Accepted Answer

All of the above

Answer

Vincristine

Answer

L-asparaginase

Answer

Prednisolone

Question 6

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

Accepted Answer

Neuroblastoma

Answer

Wilm's tumor

Answer

Rhabdomyosarcoma

Answer

Primitive neuroectodermal tumor (PNET)

Question 7

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

Accepted Answer

Wilm's tumor

Answer

Neuroblastoma

Answer

Nephronophthisis

Answer

Pheochromocytoma

Question 8

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?

Accepted Answer

Neuroblastoma

Answer

Lymphoma

Answer

Acute lymphoblastic leukemia (ALL)

Answer

Wilm's tumor

Question 9

What is the commonest sarcoma in children?

Accepted Answer

Rhabdomyosarcoma

Answer

Lipoma

Answer

Angiosarcoma

Answer

Fibrosarcoma

Question 10

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?

Accepted Answer

Dinutuximab

Answer

Ipilimumab

Answer

Atezolizumab

Answer

Olarutumab

Oncology — MCQs

Oncology — MCQs

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