Oncology Practice Questions

Q: A 6-year-old child presents with joint pain, respiratory distress, recurrent infections, pallor, conjunctival hemorrhage, and splenomegaly. A chest X-ray shows a mediastinal mass. What is the most likely diagnosis?

Acute lymphoblastic leukemia (ALL). ### Explanation **Correct Option: A. Acute Lymphoblastic Leukemia (ALL)** Acute Lymphoblastic Leukemia is the most common pediatric malignancy. This clinical presentation is classic for **T-cell ALL**, which typically affects older children and adolescents (unlike B-cell ALL). * **Bone Marrow Failure:** Pallor (anemia), recurrent infections (leukopenia/dysfunctional WBCs), and conjunctival hemorrhage (thrombocytopenia) reflect marrow infiltration. * **Extramedullary Involvement:** Splenomegaly and joint pain (due to leukemic infiltration of the periosteum) are common. * **Mediastinal Mass:** A hallmark of T-cell ALL is a **thymic mass**, which leads to respiratory distress or Superior Vena Cava (SVC) syndrome. **Why Other Options are Incorrect:** * **B. Chronic Myeloid Leukemia (CML):** Rare in children. It typically presents with massive splenomegaly but lacks the acute onset of bone marrow failure and mediastinal masses seen here. * **C. Pulmonary Metastasis:** While it could cause respiratory distress, it does not explain the systemic triad of anemia, thrombocytopenia, and splenomegaly. * **D. Sjogren Syndrome:** An autoimmune condition characterized by dry eyes and mouth; it does not present with acute bone marrow failure or a mediastinal mass in a 6-year-old. **NEET-PG High-Yield Pearls:** * **Most common subtype of ALL:** B-cell ALL (CD10/CALLA positive). * **T-cell ALL Presentation:** Often presents as a "Teenager with a Thymic mass" and high WBC count. * **Good Prognostic Factors:** Age 1–9 years, low initial WBC count ( 10 years, high WBC count, hypodiploidy, and t(9;22) (Philadelphia chromosome). * **Common sites of relapse:** Central Nervous System (CNS) and Testes (blood-organ barriers).

Q: A 4-year-old child presents with an abdominal mass and fever. CT scan reveals a crescent-shaped, discrete, and peripheral calcification. Which chemotherapy regimen is indicated for this condition?

VCD. **Explanation:** The clinical presentation of a 4-year-old child with an abdominal mass, fever, and specific CT findings of **crescent-shaped, peripheral calcifications** is classic for **Neuroblastoma**. Neuroblastoma is the most common extracranial solid tumor of childhood, typically arising from the adrenal medulla or sympathetic chain. **Why VCD is correct:** The standard chemotherapy backbone for Neuroblastoma involves a combination of **Vincristine, Cyclophosphamide, and Doxorubicin (VCD)**. In high-risk cases, this is often intensified with Etoposide and Cisplatin. These agents target the rapidly dividing neural crest cells characteristic of the tumor. **Why the other options are incorrect:** * **FOLFOX (Folinic acid, Fluorouracil, Oxaliplatin):** Primarily used for colorectal cancer and other gastrointestinal malignancies; not used in pediatric embryonal tumors. * **ECF (Epirubicin, Cisplatin, Fluorouracil):** A regimen typically indicated for gastric and esophageal carcinomas. * **CAF (Cyclophosphamide, Adriamycin/Doxorubicin, Fluorouracil):** Historically used in the treatment of breast cancer. **Clinical Pearls for NEET-PG:** * **Calcification Pattern:** While Neuroblastoma shows **stippled/peripheral** calcifications, Wilms' tumor (the main differential) rarely calcifies. * **Vessel Involvement:** Neuroblastoma **encases** major vessels (e.g., Aorta), whereas Wilms' tumor **displaces** them. * **Markers:** Elevated urinary catecholamines (VMA and HVA) are diagnostic in 90% of cases. * **Prognosis:** **N-myc amplification** is the most important poor prognostic genetic marker. * **Opsoclonus-Myoclonus Syndrome:** A high-yield paraneoplastic syndrome associated with Neuroblastoma ("dancing eyes, dancing feet").

Q: Which of the following is a good prognostic factor for neuroblastoma?

Triploidy. **Explanation:** Neuroblastoma is the most common extracranial solid tumor of childhood. Its prognosis is highly dependent on the biological and molecular characteristics of the tumor cells. **Why Triploidy is Correct:** The DNA index (ploidy) is a significant prognostic marker in infants. **Hyperdiploidy** (specifically **triploidy**) is associated with a **favorable prognosis**. These tumors tend to be more differentiated, respond better to chemotherapy, and are often seen in younger children (Stage 4S), where spontaneous regression can occur. **Analysis of Incorrect Options:** * **A. Diploidy:** A DNA index of 1 (diploid) is associated with a **poor prognosis**. It often correlates with more aggressive tumor behavior and genetic instability. * **B. N-myc amplification:** This is the **most important poor prognostic factor** in neuroblastoma. Found on chromosome 2p, amplification (>10 copies) signifies rapid tumor progression and resistance to standard therapy, regardless of the clinical stage. * **C. Loss of 11q:** Segmental chromosomal aberrations, such as the loss of 11q or 1p, are markers of **unfavorable prognosis** and are typically seen in high-risk, non-amplified N-myc cases. **High-Yield Clinical Pearls for NEET-PG:** * **Age:** The most important clinical prognostic factor; age **<18 months** has a better prognosis. * **Shimada Classification:** Histopathological grading where "Favorable Histology" (FH) includes well-differentiated tumors with a low Mitosis-Karyorrhexis Index (MKI). * **Stage 4S (Special):** Occurs in infants <1 year with localized primary tumor but dissemination limited to liver, skin, and bone marrow (excluding cortical bone). It has an excellent prognosis. * **Urinary Markers:** Elevated VMA (Vanillylmandelic acid) and HVA (Homovanillic acid) are diagnostic.

Q: Which of the following is a poor prognostic factor associated with acute lymphoblastic leukemia (ALL) in children?

Testicular involvement. **Explanation:** Prognostic factors in Acute Lymphoblastic Leukemia (ALL) are critical for risk stratification and determining treatment intensity. **Why Testicular Involvement is Correct:** Testicular involvement at the time of diagnosis is considered a **poor prognostic factor**. The testes (along with the CNS) act as "pharmacological sanctuaries" where the blood-testis barrier prevents many standard chemotherapeutic agents from reaching effective concentrations. This increases the risk of relapse and necessitates more intensive systemic therapy or local radiation. **Analysis of Incorrect Options:** * **A. Total Leukocyte Count (TLC) of 4,000-10,000/µL:** This is a **favorable** prognostic factor. A low initial WBC count ( 50,000/µL in B-ALL or >100,000/µL in T-ALL) indicate high tumor burden and poor prognosis [1]. * **B. Age at diagnosis >2 years:** The "age peak" for a good prognosis is between **1 and 10 years**. Infants ( 10 years) have a significantly poorer prognosis. Therefore, being >2 years old (within the 1-10 range) is generally favorable. * **D. Female Gender:** Statistically, **females** have a slightly better prognosis than males. Males have a higher risk of late relapse, partly due to the potential for occult testicular involvement. **High-Yield Clinical Pearls for NEET-PG:** * **Best Prognostic Age:** 1–9 years. * **Cytogenetics:** **t(12;21)** (ETV6-RUNX1) has the **best** prognosis; **t(9;22)** (Philadelphia chromosome) and **t(4;11)** have the **worst** prognosis [1]. * **Ploidy:** Hyperdiploidy (>50 chromosomes) is a favorable factor. * **Early Response:** The most important independent prognostic factor is the **minimal residual disease (MRD)** status after the induction phase of chemotherapy [1].

Q: All of the following genetic syndromes are associated with Wilm's tumor except?

Opsoclonus-myoclonus-ataxia syndrome. **Explanation:** Wilms tumor (Nephroblastoma) is the most common primary renal tumor of childhood. Its pathogenesis is strongly linked to mutations in the **WT1 gene** (Chromosome 11p13) and the **WT2 gene** (Chromosome 11p15). **Why Option B is correct:** **Opsoclonus-myoclonus-ataxia syndrome (OMS)**, also known as "Dancing Eye-Dancing Feet" syndrome, is a paraneoplastic manifestation most classically associated with **Neuroblastoma**, not Wilms tumor. It is characterized by rapid, involuntary eye movements, muscle jerks, and cerebellar ataxia. **Why the other options are incorrect:** * **WAGR Syndrome (Option A):** A microdeletion at 11p13 involving the *WT1* and *PAX6* genes. It consists of **W**ilms tumor, **A**niridia, **G**enitourinary anomalies, and mental **R**etardation. * **Denys-Drash Syndrome (Option B):** Caused by point mutations in the *WT1* gene. It presents with a triad of early-onset nephropathy (diffuse mesangial sclerosis), male pseudohermaphroditism, and a high risk (>90%) of Wilms tumor. * **Beckwith-Wiedemann Syndrome (Option D):** An imprinting disorder at the *WT2* locus (11p15). Clinical features include macrosomia, macroglossia, omphalocele, and hemihyperplasia. These patients have an increased risk of Wilms tumor and hepatoblastoma. **High-Yield Clinical Pearls for NEET-PG:** 1. **Most common presentation:** Asymptomatic abdominal mass that does not cross the midline (unlike Neuroblastoma). 2. **Staging:** Wilms tumor is staged surgically (NWTS Staging), whereas Neuroblastoma uses the INSS system. 3. **WAGR vs. Denys-Drash:** Both involve *WT1*, but WAGR is a deletion (includes *PAX6*—hence aniridia), while Denys-Drash is a missense mutation. 4. **Perilobar Nephrogenic Rests:** These are precursor lesions found in the kidneys of children with Wilms tumor, especially in bilateral cases.

Q: Which of the following is the most common hematological malignancy in children with Down syndrome?

Acute megakaryoblastic leukemia. Children with Down syndrome (Trisomy 21) have a significantly increased risk (10–20 fold) of developing leukemia. The relationship between Down syndrome and leukemia is unique and age-dependent. **Explanation of the Correct Answer:** **Acute Megakaryoblastic Leukemia (AMKL/AML-M7)** is the most common subtype of Acute Myeloid Leukemia (AML) in children with Down syndrome, especially those under the age of 3. These patients often harbor a mutation in the **GATA1 gene**. Interestingly, children with Down syndrome and AMKL have a much better prognosis and higher sensitivity to chemotherapy (specifically Cytarabine) compared to non-Down syndrome children with the same subtype. **Analysis of Incorrect Options:** * **A. T-cell Acute Lymphoblastic Leukemia:** While ALL is common in children generally, Down syndrome patients are more predisposed to B-cell lineage ALL, not T-cell. * **B. Burkitt Leukemia (L3 ALL):** This is a mature B-cell neoplasm associated with c-myc translocation; it does not have a specific increased prevalence in Down syndrome. * **C. Acute Promyelocytic Leukemia (APL/AML-M3):** Characterized by the t(15;17) translocation, this subtype is not specifically associated with Trisomy 21. **High-Yield Clinical Pearls for NEET-PG:** * **Age Factor:** In Down syndrome, **AML (specifically M7)** is more common in the first 3 years of life. After age 3, **ALL** becomes more common than AML, but AMKL remains the most characteristic "high-yield" association. * **TMD:** Down syndrome neonates may present with **Transient Myeloproliferative Disorder (TMD)**, a self-limiting "leukemoid" condition that resolves spontaneously but increases the risk of developing AMKL later. * **GATA1 Mutation:** This is the hallmark molecular finding in both TMD and AMKL in Down syndrome.

Q: In a 6-year-old child with ALL relapse, what is the most common site of relapse?

Bone marrow. **Explanation:** In Acute Lymphoblastic Leukemia (ALL), the **bone marrow** is the most common site of relapse, occurring in approximately 70-80% of cases. Relapse is defined as the reappearance of lymphoblasts at any site after achieving complete remission. Bone marrow involvement can occur in isolation or concurrently with extramedullary sites. **Analysis of Options:** * **A. Bone marrow (Correct):** It remains the primary site of leukemic cell proliferation. Early relapses (within 36 months of diagnosis) carry a poorer prognosis compared to late relapses. * **B. Liver:** While hepatomegaly is a common finding at initial diagnosis due to leukemic infiltration, the liver is not a primary site for isolated relapse. * **C. Testes:** The testes and the Central Nervous System (CNS) are considered **"sanctuary sites"** because the blood-testis and blood-brain barriers limit the penetration of standard systemic chemotherapy. While the testes are the most common site of *extramedullary* relapse in males, they are less common than bone marrow relapse overall. * **D. Lung:** Pulmonary involvement is rare in ALL and is almost never the primary site of relapse. **High-Yield Clinical Pearls for NEET-PG:** * **CNS Relapse:** Presents with signs of increased intracranial pressure (headache, vomiting, papilledema). It is the second most common extramedullary site after the testes. * **Testicular Relapse:** Typically presents as painless, firm, unilateral or bilateral testicular enlargement. * **Prognostic Factor:** The **time to relapse** is the most significant prognostic factor; the longer the initial remission, the better the outcome after relapse. * **Sanctuary Sites:** Always remember that CNS and Testes require specific directed therapy (like intrathecal chemo or radiation) because systemic drugs don't reach them effectively.

Q: Which of the following conditions is characterized by the clinical feature described?

Neuroblastoma. ***Neuroblastoma*** - **Raccoon eyes** (bilateral periorbital ecchymosis) is a pathognomonic sign of neuroblastoma with **orbital metastases**, commonly seen in pediatric patients. - Elevated **urinary VMA/HVA** (vanillylmandelic acid/homovanillic acid) levels and **catecholamine secretion** are characteristic biochemical findings. *Pheochromocytoma* - Primarily presents with **hypertensive crises**, **sweating**, and **headaches** due to excessive catecholamine release. - Does not typically cause **orbital metastases** or raccoon eyes, even though it also secretes catecholamines. *Adrenal incidentaloma* - Usually **asymptomatic** and discovered incidentally on imaging studies. - Lacks the characteristic **periorbital ecchymosis** and catecholamine-related symptoms seen in this case. *Medullary thyroid cancer* - Commonly presents with **diarrhea**, **flushing**, and elevated **calcitonin** levels due to excessive calcitonin secretion. - Does not cause **orbital metastases** or raccoon eyes, and primarily affects the thyroid gland rather than adrenal tissue.

Q: Which of the following is the most common solid tumor in a child?

Neuroblastoma. **Explanation:** **Neuroblastoma** is the most common extracranial solid tumor of childhood. It originates from primordial neural crest cells of the sympathetic nervous system. While brain tumors are the most common solid tumors overall, among non-CNS (extracranial) solid tumors, Neuroblastoma holds the highest incidence, accounting for approximately 7-10% of all childhood cancers. **Analysis of Options:** * **Neuroblastoma (Correct):** It most commonly arises from the adrenal medulla (40%) or the paraspinal sympathetic ganglia. It is the most common solid tumor in infants (<1 year). * **Nephroblastoma (Wilms Tumor):** This is the most common primary **renal** tumor in children, but it is less frequent than Neuroblastoma overall. It typically presents as an asymptomatic abdominal mass that does not cross the midline. * **Mesoblastic Nephroma:** This is the most common renal tumor found in the **neonatal period** (first month of life). It is generally benign and treated with surgical excision. * **Rhabdomyosarcoma:** This is the most common **soft tissue sarcoma** in children, but its overall incidence is lower than that of Neuroblastoma and Wilms tumor. **High-Yield Clinical Pearls for NEET-PG:** * **Most common childhood cancer:** Leukemia (ALL). * **Most common solid tumor overall:** Brain tumors (e.g., Medulloblastoma/Astrocytoma). * **Most common extracranial solid tumor:** Neuroblastoma. * **Key Diagnostic Marker:** Elevated urinary catecholamines (VMA and HVA) are seen in 90% of Neuroblastoma cases. * **Prognostic Marker:** *N-myc* amplification is the most important poor prognostic factor in Neuroblastoma. * **Clinical Sign:** "Dancing eyes, dancing feet" (Opsoclonus-myoclonus syndrome) is a classic paraneoplastic association with Neuroblastoma.

Q: Cancer management in which of the following malignancies has dramatically increased survival?

Acute lymphoblastic leukemia (ALL) in children. **Explanation:** The correct answer is **Acute lymphoblastic leukemia (ALL) in children**. **Why it is correct:** Pediatric ALL is one of the greatest success stories in modern oncology. In the 1960s, the survival rate was less than 10%. Today, with the advent of risk-stratified multi-agent chemotherapy (including induction, consolidation, and long-term maintenance), the 5-year survival rate exceeds **90%**. This dramatic improvement is attributed to optimized protocols, CNS prophylaxis, and better supportive care. **Why the other options are incorrect:** * **Glioblastoma multiforme (GBM):** This remains one of the most aggressive and lethal primary brain tumors. Despite surgery, radiation, and chemotherapy (Temozolomide), the median survival is still only 12–15 months. * **Esophageal carcinoma:** Often diagnosed at an advanced stage, it carries a poor prognosis. Even with multimodal therapy (chemoradiation and surgery), the 5-year survival rate remains relatively low (approx. 20%). * **Cholangiocarcinoma:** This biliary tract cancer is notoriously difficult to treat and often resistant to chemotherapy. It has a very high mortality rate, with 5-year survival rates typically under 10% for unresectable cases. **High-Yield Clinical Pearls for NEET-PG:** * **Most common childhood malignancy:** ALL (Peak age: 2–5 years). * **Best Prognostic Factors in ALL:** Age 1–9 years, low initial WBC count ( 50 chromosomes), and the presence of *TEL-AML1* (t(12;21)) translocation. * **Poor Prognostic Factors:** Age 10 years, hypodiploidy, and the Philadelphia chromosome (t(9;22)). * **L-asparaginase:** A key drug in ALL protocols; watch for side effects like pancreatitis and thrombosis.

Question 1

A 6-year-old child presents with joint pain, respiratory distress, recurrent infections, pallor, conjunctival hemorrhage, and splenomegaly. A chest X-ray shows a mediastinal mass. What is the most likely diagnosis?

Accepted Answer

Acute lymphoblastic leukemia (ALL)

Answer

Chronic myeloid leukemia (CML)

Answer

Pulmonary metastasis

Answer

Sjogren syndrome

Question 2

A 4-year-old child presents with an abdominal mass and fever. CT scan reveals a crescent-shaped, discrete, and peripheral calcification. Which chemotherapy regimen is indicated for this condition?

Accepted Answer

VCD

Answer

FOLFOX

Answer

ECF

Answer

CAF

Question 3

Which of the following is a good prognostic factor for neuroblastoma?

Accepted Answer

Triploidy

Answer

Diploidy

Answer

N-myc amplification

Answer

Loss of 11q

Question 4

Which of the following is a poor prognostic factor associated with acute lymphoblastic leukemia (ALL) in children?

Accepted Answer

Testicular involvement

Answer

Total leukocyte count of 4,000-10,000/µL

Answer

Age at diagnosis more than 2 years

Answer

Female gender

Question 5

All of the following genetic syndromes are associated with Wilm's tumor except?

Accepted Answer

Opsoclonus-myoclonus-ataxia syndrome

Answer

WAGR syndrome

Answer

Denys Drash syndrome

Answer

Beckwith-Wiedemann syndrome

Question 6

Which of the following is the most common hematological malignancy in children with Down syndrome?

Accepted Answer

Acute megakaryoblastic leukemia

Answer

T-cell acute lymphoblastic leukemia

Answer

Burkitt leukemia

Answer

Acute promyelocytic leukemia

Question 7

In a 6-year-old child with ALL relapse, what is the most common site of relapse?

Accepted Answer

Bone marrow

Answer

Liver

Answer

Testes

Answer

Lung

Question 8

Which of the following conditions is characterized by the clinical feature described?

Accepted Answer

Neuroblastoma

Answer

Pheochromocytoma

Answer

Adrenal incidentaloma

Answer

Medullary thyroid cancer

Question 9

Which of the following is the most common solid tumor in a child?

Accepted Answer

Neuroblastoma

Answer

Nephroblastoma

Answer

Mesoblastic nephroma

Answer

Rhabdomyosarcoma

Question 10

Cancer management in which of the following malignancies has dramatically increased survival?

Accepted Answer

Acute lymphoblastic leukemia (ALL) in children

Answer

Glioblastoma multiforme

Answer

Esophageal carcinoma

Answer

Cholangiocarcinoma

Oncology — MCQs

Oncology — MCQs

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