Oncology Practice Questions

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, typically presenting with the classic triad of **raised intracranial pressure** (headache), **visual field defects**, and **endocrine dysfunction**. - Characteristic imaging shows a **cystic-solid suprasellar mass** with **calcifications** on CT/MRI, which is pathognomonic in pediatric patients. *Pituitary macroadenoma* - Extremely **rare in children** and adolescents, with peak incidence in adults aged 30-50 years. - When present in pediatrics, typically causes **prolactin excess** or **growth hormone excess** rather than mass effect symptoms. *Rathke's cleft cyst* - Usually **asymptomatic** and discovered incidentally, rarely causing visual disturbances in children. - When symptomatic, more commonly presents with **mild endocrine dysfunction** rather than significant mass effect. *Hypothalamic hamartoma* - Classically presents with **gelastic seizures** (laughing fits) and **precocious puberty**, not headache and visual disturbances. - Typically **non-calcified** on imaging and located within the **hypothalamus** rather than suprasellar region.

Q: Which of the following is the most common intracranial tumor in children?

Glioma. **Explanation:** In the pediatric population, central nervous system (CNS) tumors are the most common solid tumors. When categorizing these by histological type, **Gliomas** are the most common intracranial tumors in children. **1. Why Glioma is Correct:** The term "Glioma" is an umbrella category that includes various subtypes such as Astrocytomas, Ependymomas, and Oligodendrogliomas. Among these, **Astrocytomas** (specifically Juvenile Pilocytic Astrocytoma) are the most frequent. Collectively, gliomas account for approximately 50-60% of all pediatric brain tumors. **2. Why the other options are incorrect:** * **Medulloblastoma:** While this is the most common **malignant** (embryonal) brain tumor in children and the most common tumor of the posterior fossa, it is less frequent than the total group of gliomas. * **Meningioma:** These are common in adults but are extremely rare in children. Their presence in a pediatric patient should raise suspicion for Neurofibromatosis Type 2 (NF2). * **Lymphangioma:** This is a benign malformation of the lymphatic system, typically occurring in the neck (cystic hygroma) or axilla, and is not an intracranial tumor. **High-Yield Clinical Pearls for NEET-PG:** * **Most common solid tumor in children:** CNS Tumors. * **Most common benign CNS tumor:** Pilocytic Astrocytoma (Grade I). * **Most common malignant CNS tumor:** Medulloblastoma. * **Infratentorial vs. Supratentorial:** In children (aged 1–10 years), the majority of tumors are **infratentorial** (posterior fossa). In infants (<1 year) and adults, they are primarily supratentorial. * **Classic Triad:** Morning headache, vomiting, and papilledema (signs of increased ICP).

Q: Which of the following is true about leukemia in Down's syndrome?

Prognosis in AML is better than in the general population.. Children with Down syndrome (Trisomy 21) have a unique predisposition to leukemia, characterized by distinct clinical behaviors and treatment responses. **Explanation of the Correct Answer (C):** While children with Down syndrome have a 10–20 fold increased risk of developing leukemia, they exhibit an **exquisitely high sensitivity to chemotherapy**, particularly Cytarabine (Ara-C). In cases of Acute Myeloid Leukemia (AML)—specifically the **M7 subtype (Acute Megakaryoblastic Leukemia)**—patients have significantly better event-free survival and overall prognosis compared to non-Down syndrome children, provided they are treated with reduced-intensity protocols to minimize toxicity. **Analysis of Incorrect Options:** * **Option A:** Incorrect. The risk of both ALL and AML is significantly **higher** (about 10–20 times) than in the general population. * **Option B:** Incorrect. While the *relative* risk of AML is higher in Down syndrome than in the general population, **ALL remains the most common** type of leukemia in these children overall (similar to the general pediatric population). The exception is in children under the age of 3, where AML (M7) is more frequent. * **Option D:** Incorrect. Children with Down syndrome are highly susceptible to **methotrexate-induced gastrointestinal toxicity and myelosuppression**. Therefore, methotrexate doses must be **reduced**, not increased. **High-Yield Clinical Pearls for NEET-PG:** * **TAM (Transient Abnormal Myelopoiesis):** A "pseudo-leukemia" seen in neonates with Down syndrome; it usually resolves spontaneously but predisposes them to AML later. * **GATA1 Mutation:** Characteristically associated with both TAM and AML-M7 in Down syndrome. * **Age Factor:** AML is more common in Down syndrome children 3 years old.

Q: What is the commonest site of metastasis for Wilms' tumor?

Lungs. **Explanation:** Wilms' tumor (Nephroblastoma) is the most common primary renal malignancy in children. The correct answer is **Lungs** because Wilms' tumor primarily spreads via the **hematogenous route**. Once the tumor cells enter the renal vein and the inferior vena cava, the first major capillary bed they encounter is in the pulmonary circulation, making the lungs the most frequent site of distant metastasis (seen in approximately 10-15% of patients at diagnosis). **Analysis of Options:** * **Lungs (Correct):** The primary site of distant spread. Pulmonary metastases typically appear as "cannonball" lesions on imaging. * **Liver:** This is the second most common site of distant metastasis. It occurs via hematogenous spread or direct extension. * **Bones & Brain:** These are **extremely rare** sites for classic Wilms' tumor. If a pediatric renal tumor metastasizes to the bone, clinicians should strongly suspect **Clear Cell Sarcoma of the Kidney (CCSK)**, often nicknamed the "Bone-metastasizing renal tumor." If it spreads to the brain, **Rhabdoid Tumor of the Kidney** is a more likely diagnosis. **High-Yield NEET-PG Pearls:** 1. **Staging:** Unlike many other tumors, Wilms' staging is based on both surgical and pathological findings. 2. **WAGR Syndrome:** Associated with *WT1* gene deletion (Wilms tumor, Aniridia, Genitourinary anomalies, and intellectual disability/Retardation). 3. **Beckwith-Wiedemann Syndrome:** Associated with *WT2* gene; presents with macroglossia, omphalocele, and hemihypertrophy. 4. **Clinical Presentation:** Most commonly presents as a painless, smooth abdominal mass that **does not cross the midline** (unlike Neuroblastoma).

Q: What is the best prognostic factor for a 2-year-old child diagnosed with Acute Lymphoblastic Leukemia (ALL)?

Age between 1 and 10 years. In Pediatric Acute Lymphoblastic Leukemia (ALL), the two most important clinical predictors of prognosis are **Age** and **Initial White Blood Cell (WBC) count**. **Explanation of the Correct Option:** * **Age between 1 and 10 years:** This is the "favorable age group." Children diagnosed within this window have significantly higher cure rates (exceeding 90%) compared to infants ( 10 years), who often present with more aggressive biological subtypes. **Explanation of Incorrect Options:** * **TLC > 1 lac (100,000/mm³):** This is a **poor** prognostic factor. A high tumor burden at presentation (TLC > 50,000/mm³) indicates a higher risk of relapse and central nervous system involvement. * **Presence of petechiae:** This is a clinical sign of thrombocytopenia, which is a common presenting feature of ALL. It does not serve as an independent prognostic marker for long-term survival. * **Translocation t(9:22):** Also known as the **Philadelphia chromosome**, this is a **very poor** prognostic factor. It is associated with resistance to standard chemotherapy and requires targeted therapy (e.g., Imatinib). **High-Yield Clinical Pearls for NEET-PG:** * **Best Prognostic Factors:** Age 1–10 years, TLC 50 chromosomes), and the presence of **t(12;21)** (TEL-AML1). * **Worst Prognostic Factors:** Age 10 years, TLC > 50,000/mm³, Hypodiploidy, and **t(9;22)** or **t(4;11)**. * **Most Important Response Marker:** Minimal Residual Disease (MRD) at the end of induction therapy is currently the strongest predictor of overall outcome.

Q: Which of the following malignancies in children does not typically require immediate treatment?

Neuroblastoma. ### **Explanation** The correct answer is **Neuroblastoma (Option A)**. #### **Why Neuroblastoma is the Correct Answer** In pediatric oncology, certain cases of Neuroblastoma—specifically **Stage 4S (Special)**—are unique because they can undergo **spontaneous regression** without any aggressive intervention. Stage 4S occurs in infants (<12 months) where the tumor is localized but has spread limited to the liver, skin, and/or bone marrow (less than 10% involvement). Because these tumors often differentiate into benign forms or disappear on their own, a "watch and wait" approach is frequently adopted unless life-threatening symptoms (like respiratory distress from hepatomegaly) occur. #### **Why Other Options are Incorrect** * **Burkitt’s Lymphoma (Option B):** This is one of the fastest-growing human tumors with a doubling time of ~24 hours. It carries a massive risk of **Tumor Lysis Syndrome (TLS)** and requires immediate stabilization and chemotherapy. * **T-cell Lymphoblastic Leukemia (Option C):** Often presents with a large mediastinal mass, leading to **Superior Vena Cava (SVC) syndrome** or airway obstruction. It is a medical emergency requiring prompt treatment. * **Acute Myeloid Leukemia (AML) (Option D):** AML is associated with severe cytopenias, life-threatening infections, and coagulopathies (especially DIC in the M3 subtype). Immediate induction therapy is mandatory. #### **High-Yield Clinical Pearls for NEET-PG** * **Stage 4S Neuroblastoma:** Defined by age <1 year, localized primary, and metastasis limited to **S**kin, **S**panned liver, and **S**canty bone marrow. * **Tumor Marker:** Elevated urinary catecholamines (VMA/HVA) are seen in 90% of neuroblastomas. * **Opsoclonus-Myoclonus Syndrome:** A paraneoplastic syndrome ("dancing eyes, dancing feet") associated with favorable-prognosis neuroblastoma. * **N-myc Amplification:** The most important poor prognostic genetic marker in neuroblastoma.

Q: An 8-year-old child is diagnosed with Langerhans cell histiocytosis with bone involvement. All of the following are true about Langerhans cell histiocytosis, EXCEPT:

Enophthalmos is present.. **Explanation:** Langerhans Cell Histiocytosis (LCH) is a rare proliferative disorder of myeloid dendritic cells. The correct answer is **D** because LCH is classically associated with **Exophthalmos (Proptosis)**, not enophthalmos. This occurs due to retro-orbital infiltration or bony destruction of the orbit. **Analysis of Options:** * **Option A (Peak incidence < 3 years):** This is **true**. While LCH can occur at any age, the peak incidence is between 1–3 years. Multi-system involvement is more common in infants, whereas single-system bone disease is more common in older children. * **Option B (Radiosensitive):** This is **true**. LCH lesions are highly sensitive to low-dose radiotherapy. While surgery or observation is preferred for solitary bone lesions, radiotherapy is an effective salvage treatment for lesions in critical areas (e.g., optic nerve compression). * **Option C (Letterer-Siwe disease):** This is **true**. LCH was historically categorized into three clinical syndromes: 1. **Letterer-Siwe disease:** Acute disseminated/diffuse form (infants; poor prognosis). 2. **Hand-Schüller-Christian disease:** Chronic disseminated triad of diabetes insipidus, exophthalmos, and bone lesions. 3. **Eosinophilic Granuloma:** Solitary or few bone lesions. **High-Yield Clinical Pearls for NEET-PG:** * **Pathognomonic Marker:** **Birbeck granules** (tennis-racket shaped) seen on Electron Microscopy. * **Immunohistochemistry (IHC):** Positive for **CD1a, CD207 (Langerin)**, and S100. * **Radiology:** "Punched-out" lytic lesions in the skull without a sclerotic rim. * **Commonest Site:** Bone (Skull is the most frequent site). * **Endocrine Association:** Central Diabetes Insipidus is the most common permanent endocrine complication.

Q: A 7-year-old boy presents with a left renal mass, bone pain, and radiographically confirmed bone metastatic deposits. What is the most likely renal tumor?

Clear cell sarcoma of the kidney. ***Clear cell sarcoma of the kidney*** - Known as the **"bone-staining tumor"**, it is the pediatric renal malignancy most frequently associated with **skeletal metastases**. - It typically presents in children aged **2 to 5 years** and carries a significantly **worse prognosis** than favorable histology Wilms tumor. *Favorable histology Wilms tumor* - This is the most common pediatric renal tumor, but it primarily metastasizes to the **lungs** and **liver**. - Bone involvement is extremely rare, occurring in less than **1% of cases** of Wilms tumor. *Renal cell carcinoma* - While it can spread to bone, it is much **rarer in children** and typically occurs in older children or **adolescents**. - It is usually associated with the classic triad of **hematuria**, flank pain, and a palpable mass, rather than early isolated bone pain. *Rhabdoid tumor of the kidney* - This is a highly aggressive tumor primarily seen in **infants** and very young children, typically under **2 years of age**. - It is characteristically associated with synchronous **brain tumors** (specifically medulloblastomas/ATRT) rather than isolated bone metastases.

Q: Which of the following statements regarding Wilms tumor is false?

Peak incidence is below the age of 1 year. **Explanation:** Wilms tumor (Nephroblastoma) is the most common primary renal malignancy in children. The correct answer is **D** because the peak incidence of Wilms tumor occurs between **2 and 5 years of age**. It is relatively uncommon in infants under the age of 1 year (where Mesoblastic Nephroma is more frequent). **Analysis of Options:** * **Option A (Aniridia):** This is a classic association. Wilms tumor is a component of the **WAGR syndrome** (Wilms tumor, Aniridia, Genitourinary anomalies, and intellectual disability/Range of developmental delays). * **Option B (Beckwith-Wiedemann Syndrome):** This is an overgrowth disorder characterized by macroglossia, omphalocele, and hemihypertrophy. These patients have a significantly increased risk of embryonal tumors, specifically Wilms tumor and hepatoblastoma. * **Option C (WT1/WT2 deletion):** The molecular pathogenesis involves the **WT1 gene** (located on chromosome 11p13) and the **WT2 gene** (located on chromosome 11p15). Deletions or mutations in these regions are central to the development of the tumor. **High-Yield Clinical Pearls for NEET-PG:** * **Presentation:** Most commonly presents as an asymptomatic, smooth, firm **abdominal mass that does not cross the midline** (unlike Neuroblastoma). * **Histology:** Characterized by a "triphasic" pattern consisting of blastemal, stromal, and epithelial cells. * **Denys-Drash Syndrome:** Associated with WT1 mutations, characterized by Wilms tumor, early-onset nephropathy, and male pseudohermaphroditism. * **Metastasis:** The most common site of distant spread is the **lungs**. * **Prognosis:** Generally excellent, with survival rates exceeding 90% for localized disease. Anaplasia is the most important unfavorable prognostic factor.

Question 1

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

Rathke's cleft cyst

Answer

Hypothalamic hamartoma

Question 2

Which of the following is the most common intracranial tumor in children?

Accepted Answer

Glioma

Answer

Meningioma

Answer

Medulloblastoma

Answer

Lymphangioma

Question 3

Which of the following is true?

Accepted Answer

All of the above

Answer

Anemia

Answer

Leukopenia

Answer

Hepatomegaly

Question 4

Which of the following is true about leukemia in Down's syndrome?

Accepted Answer

Prognosis in AML is better than in the general population.

Answer

Acute leukemia is less common than in the general population.

Answer

Acute myeloid leukemia (AML) is more common than acute lymphoblastic leukemia (ALL).

Answer

If diagnosed with ALL, higher doses of methotrexate should be given.

Question 5

What is the commonest site of metastasis for Wilms' tumor?

Accepted Answer

Lungs

Answer

Bones

Answer

Liver

Answer

Brain

Question 6

What is the best prognostic factor for a 2-year-old child diagnosed with Acute Lymphoblastic Leukemia (ALL)?

Accepted Answer

Age between 1 and 10 years

Answer

Total Leukocyte Count (TLC) greater than 1 lac

Answer

Presence of petechiae

Answer

Translocation t(9:22)

Question 7

Which of the following malignancies in children does not typically require immediate treatment?

Accepted Answer

Neuroblastoma

Answer

Burkitt's lymphoma

Answer

T cell lymphoblastic leukemia

Answer

Acute myeloid leukemia (AML)

Question 8

An 8-year-old child is diagnosed with Langerhans cell histiocytosis with bone involvement. All of the following are true about Langerhans cell histiocytosis, EXCEPT:

Accepted Answer

Enophthalmos is present.

Answer

Peak incidence is less than 3 years of age.

Answer

It is radiosensitive.

Answer

The diffuse form is known as Letterer-Siwe disease.

Question 9

A 7-year-old boy presents with a left renal mass, bone pain, and radiographically confirmed bone metastatic deposits. What is the most likely renal tumor?

Accepted Answer

Clear cell sarcoma of the kidney

Answer

Favorable histology Wilms tumor

Answer

Renal cell carcinoma

Answer

Rhabdoid tumor of the kidney

Question 10

Which of the following statements regarding Wilms tumor is false?

Accepted Answer

Peak incidence is below the age of 1 year

Answer

May be associated with aniridia

Answer

Associated with Beckwith-Wiedemann syndrome

Answer

May be associated with deletion of WT1 or WT2 gene

Oncology — MCQs

Oncology — MCQs

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