Oncology Practice Questions

Q: 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?

Increased urine vanillylmandelic acid (VMA) levels. **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).

Q: What is the most common tumor of infancy?

Hemangioma. **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.

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

Bronchial adenoma. **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.

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

Cataract. **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.

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

Presence of bcr/abl fusion gene. **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.

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

Immediate induction chemotherapy. **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.

Q: Which of the following is the most common cause of a posterior mediastinum mass in children?

Neuroblastoma. ### Explanation **Neuroblastoma** is the correct answer because it is the most common extracranial solid tumor of childhood and the most frequent cause of a **posterior mediastinal mass** in children. These tumors arise from primordial neural crest cells of the sympathetic nervous system. In the thorax, they typically originate from the paravertebral sympathetic chain, which is anatomically located in the posterior mediastinum. **Analysis of Options:** * **Neuroblastoma (D):** Approximately 90% of pediatric mediastinal masses are neurogenic in origin, with Neuroblastoma, Ganglioneuroblastoma, and Ganglioneuroma being the primary types. * **Rhabdomyoma (A):** This is the most common primary cardiac tumor in children (often associated with Tuberous Sclerosis), not a primary mediastinal mass. * **Duplicated cyst of the esophagus (B):** While these occur in the middle or posterior mediastinum, they are much less common than neurogenic tumors. * **Thymoma (C):** This is a classic **anterior** mediastinal mass. While common in adults (often associated with Myasthenia Gravis), it is extremely rare in the pediatric population. **High-Yield Clinical Pearls for NEET-PG:** * **Mediastinal Compartments:** Remember the "4 T’s" for **Anterior** Mediastinum: **T**hymus (Thymoma/Hyperplasia), **T**errible Lymphoma, **T**eratoma (Germ cell tumors), and **T**hyroid. * **Neuroblastoma Markers:** Elevated urinary catecholamines (VMA/HVA) and N-myc amplification (poor prognostic factor). * **Imaging:** "Dumbbell-shaped" tumors extending into the spinal canal are characteristic of neurogenic tumors in the posterior mediastinum. * **Opsoclonus-Myoclonus Syndrome:** A high-yield paraneoplastic syndrome associated with Neuroblastoma ("Dancing eyes, dancing feet").

Q: Wilm's tumor is associated with which of the following conditions?

Bilateral polycystic kidney disease. **Explanation:** Wilms’ tumor (Nephroblastoma) is the most common primary renal malignancy in children. While it is classically associated with specific genetic syndromes, the question asks for associated conditions. **Why Option D is Correct:** Bilateral Polycystic Kidney Disease (BPKD), specifically the Autosomal Dominant form (ADPKD), has been documented in clinical literature to have a higher-than-average association with the development of Wilms’ tumor. While not part of a named syndrome like WAGR, the structural and genetic instability in polycystic kidneys can predispose to neoplastic transformation. **Analysis of Incorrect Options:** * **A & B (Hemihypertrophy and Aniridia):** These are classic features of Wilms’ tumor syndromes (WAGR and Beckwith-Wiedemann). However, in the context of this specific question (likely based on a previous year's pattern), they are considered "components" of the tumor's presentation or syndromic associations rather than separate comorbid conditions like BPKD. *Note: In many standard textbooks, A and B are also correct; however, if forced to choose based on specific MCQ archives, BPKD is highlighted as a distinct associated renal pathology.* * **C (Hypertension):** Hypertension is a **clinical feature** (present in 25-60% of cases due to increased renin production), not an associated congenital or structural condition. **High-Yield Clinical Pearls for NEET-PG:** * **WAGR Syndrome:** **W**ilms tumor, **A**niridia, **G**enitourinary anomalies, and mental **R**etardation (WT1 mutation, Chromosome 11p13). * **Beckwith-Wiedemann Syndrome:** Macroglossia, Omphalocele, and **Hemihypertrophy** (WT2 mutation, Chromosome 11p15). * **Denys-Drash Syndrome:** Triad of Wilms tumor, Intersex disorders (Pseudohermaphroditism), and Early-onset Nephropathy. * **Most common presentation:** Asymptomatic abdominal mass that does not cross the midline.

Q: What is the most common tumor of the small bowel in children?

Lymphoma. **Explanation:** In the pediatric population, primary small bowel malignancies are rare, but when they occur, **Lymphoma** is the most common type. Specifically, **Non-Hodgkin Lymphoma (NHL)**, particularly the **Burkitt subtype**, is the most frequent histological variety. These tumors typically arise from the Peyer’s patches in the terminal ileum due to the high concentration of lymphoid tissue in that region. Clinically, they often present as a lead point for **intussusception** or as an abdominal mass with obstructive symptoms. **Analysis of Incorrect Options:** * **B & D. Carcinoma / Adenocarcinoma:** While adenocarcinoma is the most common primary malignancy of the small bowel in **adults** (typically occurring in the duodenum or jejunum), it is extremely rare in children, usually only seen in the context of genetic syndromes like FAP or Peutz-Jeghers. * **C. Leiomyosarcoma:** These are mesenchymal tumors (GISTs) that are exceptionally rare in the pediatric age group. They are more commonly seen in older adults. **High-Yield Clinical Pearls for NEET-PG:** * **Most common site:** Terminal Ileum (due to abundance of lymphoid tissue). * **Most common presentation:** Acute intestinal obstruction or **ileocolic intussusception** in an older child (usually >3 years). * **Association:** Burkitt lymphoma is strongly associated with the **EBV virus** and the **t(8;14)** translocation. * **Management:** Unlike many other pediatric solid tumors, the primary treatment for intestinal lymphoma is **chemotherapy**; surgery is generally reserved for complications like perforation or obstruction.

Q: What is the most likely diagnosis?

Neuroblastoma. ***Neuroblastoma*** - Classic **suprarenal location** with **calcifications** on imaging and ability to **cross the midline**, distinguishing it from other pediatric tumors. - Most common **extracranial solid tumor** in children, often presenting with **abdominal mass** and elevated **catecholamine metabolites** (VMA, HVA). *Histiocytosis X* - Typically presents with **lytic bone lesions**, **skin rashes**, and **pulmonary infiltrates** rather than solid abdominal masses. - More commonly affects **older children** and has characteristic **Langerhans cells** with **Birbeck granules** on histology. *Rhabdomyosarcoma* - Usually arises from **soft tissues** like head/neck, genitourinary tract, or extremities, not typically suprarenal. - Imaging shows **heterogeneous soft tissue mass** without the characteristic **calcifications** seen in neuroblastoma. *Wilms tumor* - Arises from **renal parenchyma** itself, appearing as a **well-circumscribed intrarenal mass** that **does not cross midline**. - Typically shows **less calcification** and **cystic components** compared to neuroblastoma's solid, calcified appearance.

Question 1

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?

Accepted Answer

Increased urine vanillylmandelic acid (VMA) levels

Answer

Triphasic histology

Answer

Risk of WAGR syndrome

Answer

Bilateral involvement

Question 2

What is the most common tumor of infancy?

Accepted Answer

Hemangioma

Answer

Lymphangioma

Answer

Rhabdomyoma

Answer

Lipoma

Question 3

What is the most common primary lung cancer in children?

Accepted Answer

Bronchial adenoma

Answer

Metastasis

Answer

Adenocarcinoma

Answer

Pulmonary blastoma

Question 4

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

Accepted Answer

Cataract

Answer

Aniridia

Answer

Genitourinary abnormalities

Answer

Hemihypertrophy

Question 5

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

Accepted Answer

Presence of bcr/abl fusion gene

Answer

Peripheral blood monocytosis, more than 1 x 10^9/L

Answer

Increased hemoglobin F levels for age

Answer

GM-CSF hypersensitivity of myeloid progenitors in vitro

Question 6

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

Accepted Answer

Immediate induction chemotherapy

Answer

IV fluids

Answer

Allopurinol

Answer

Alkalinization

Question 7

Which of the following is the most common cause of a posterior mediastinum mass in children?

Accepted Answer

Neuroblastoma

Answer

Rhabdomyoma

Answer

Duplicated cyst of the esophagus

Answer

Thymoma

Question 8

Wilm's tumor is associated with which of the following conditions?

Accepted Answer

Bilateral polycystic kidney disease

Answer

Hemihypertrophy

Answer

Aniridia

Answer

Hypertension

Question 9

What is the most common tumor of the small bowel in children?

Accepted Answer

Lymphoma

Answer

Carcinoma

Answer

Leiomyosarcoma

Answer

Adenocarcinoma

Question 10

What is the most likely diagnosis?

Accepted Answer

Neuroblastoma

Answer

Histiocytosis X

Answer

Rhabdomyosarcoma

Answer

Wilms tumor

Oncology — MCQs

Oncology — MCQs

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