Oncology — MCQs

Oncology Indian Medical PG Practice Questions and MCQs

Question 191: True about neuroblastoma:

A. Metastasis rarely present
B. Tumor of autonomic nervous system (Correct Answer)
C. Earliest detected in late childhood
D. All of the options

Explanation: ***Tumor of autonomic nervous system*** - **Neuroblastoma** is a malignancy that arises from **neuroblasts**, which are immature nerve cells of the **sympathetic nervous system** (part of the autonomic nervous system). - It most commonly originates in the **adrenal medulla** (50% of cases) or other areas along the sympathetic chain in the abdomen, chest, or neck. - This statement is **correct** and represents the fundamental pathophysiology of neuroblastoma. *Metastasis rarely present* - This statement is **incorrect** because neuroblastoma is known for its **high rate of metastasis** at diagnosis. - Approximately **50-60%** of patients present with **metastatic disease** at the time of diagnosis. - Common sites of metastasis include **bone marrow** (most common), bone, liver, and lymph nodes. - Stage 4 disease is more common than localized disease in children over 18 months. *Earliest detected in late childhood* - This statement is **incorrect** as neuroblastoma typically presents in **early childhood**, with a median age of diagnosis around **17-22 months**. - It is one of the most common **solid tumors in infants** and young children, with **90% of cases diagnosed before age 5 years**. - Rarely seen in late childhood or adolescence, making this the opposite of the truth. *All of the options* - This option is **incorrect** because only **one statement** (tumor of autonomic nervous system) is true, rendering "All of the options" false.

Question 192: A 4-year-old boy was diagnosed with a malignancy. On physical examination, he was found to have aniridia. Which of the following tumors is associated with aniridia?

A. Retinoblastoma
B. Hepatoblastoma
C. Nephroblastoma (Correct Answer)
D. Medulloblastoma

Explanation: ***Correct: Nephroblastoma*** - **Wilms' tumor**, or nephroblastoma, is a common pediatric renal malignancy that can be associated with **aniridia** as part of the **WAGR syndrome**. - **WAGR syndrome** is caused by a deletion on chromosome 11p13, which includes the *WT1* gene (responsible for Wilms' tumor) and the *PAX6* gene (responsible for aniridia). - Other features include **Genitourinary anomalies** and intellectual disability (formerly called mental **Retardation**). *Incorrect: Retinoblastoma* - This is a malignancy of the **retina** and is primarily associated with mutations in the **RB1 gene**. - While it affects the eye, it does not typically present with or cause aniridia. *Incorrect: Hepatoblastoma* - This is a rare malignant tumor of the **liver** and is not directly associated with aniridia. - It is sometimes linked to genetic syndromes like **Beckwith-Wiedemann syndrome** or familial adenomatous polyposis. *Incorrect: Medulloblastoma* - This is a malignant **brain tumor** originating in the cerebellum and is not associated with aniridia. - It often presents with symptoms related to increased intracranial pressure like **hydrocephalus** or ataxia.

Question 193: Most common manifestation of Wilms tumor is ________

A. Loss of weight
B. Hypertension
C. Haematuria
D. Abdominal mass (Correct Answer)

Explanation: ***Abdominal mass*** - An **abdominal mass** is the most common presenting symptom of Wilms' tumor, often noticed by parents during bathing or dressing. - This mass is typically **smooth, firm**, and usually does not cross the midline. *Loss of weight* - While **weight loss** can occur in malignancy, it is not the most common initial manifestation of Wilms' tumor. - The dominant sign is usually the discovery of an expanding abdominal mass. *Hypertension* - **Hypertension** is observed in a significant percentage of patients with Wilms' tumor (around 25-60%), often due to renin production by the tumor or compression of renal vessels. - However, it is less common than the presence of an abdominal mass and may not be noticed initially. *Haematuria* - **Haematuria (blood in urine)** can occur in Wilms' tumor if the tumor invades the renal collecting system. - It is present in about 15-25% of cases, making it a less frequent initial symptom compared to an abdominal mass.

Question 194: Not seen in Neuroblastoma is -

A. Bone involvement
B. Splenomegaly (Correct Answer)
C. Diarrhoea
D. Proptosis

Explanation: ***Splenomegaly*** - **Splenomegaly is NOT a typical feature of neuroblastoma**, making this the correct answer to this negation question. - Neuroblastoma commonly metastasizes to **bone marrow, liver, lymph nodes, and bones**, but **rarely involves the spleen**. - Unlike lymphomas and leukemias where splenomegaly is common, neuroblastoma's metastatic pattern typically spares the spleen. *Bone involvement* - **Bone metastases are common in neuroblastoma** (60-70% of advanced cases), particularly in stage 4 disease. - Presents with bone pain, pathologic fractures, and lytic lesions on imaging. - Hematogenous dissemination from the neural crest origin allows frequent skeletal involvement. *Diarrhoea* - **Secretory diarrhea occurs in 7-9% of neuroblastoma cases** due to vasoactive intestinal peptide (VIP) secretion. - Presents as severe watery diarrhea with hypokalemia and metabolic acidosis. - Part of the opsoclonus-myoclonus-ataxia syndrome or as an isolated paraneoplastic phenomenon. *Proptosis* - **Orbital metastases cause proptosis and periorbital ecchymoses** ("raccoon eyes"), a classic presentation. - Indicates advanced disease with skull and facial bone involvement. - Seen in approximately 10-15% of cases at presentation, particularly in younger children.

Question 195: Which of the following statements regarding Wilms tumor is false?

A. Most commonly seen < 5 years of age
B. Most patients recover well with chemotherapy and radiotherapy.
C. Presents as a renal mass.
D. Lung metastasis is rare and occurs late (Correct Answer)

Explanation: ***Lung metastasis is rare and occurs late*** - This statement is **false** primarily because when lung metastases occur, they often present **early (at initial diagnosis)** rather than late. - Approximately **10-15% of patients** have pulmonary metastases at presentation, making the "occurs late" portion incorrect. - While lungs are the **most common site** for distant metastases when they do occur, the overall incidence is relatively low, so calling it "rare" is debatable but the "late" timing is definitively wrong. - The statement's claim about late occurrence contradicts clinical reality where metastatic disease, when present, is typically identified at diagnosis or within the first 2 years. *Most commonly seen < 5 years of age* - This statement is **true** as Wilms tumor has **peak incidence at ages 2-3 years**, with the majority of cases diagnosed before age **5 years**. - It is the most common primary **renal malignancy in children**, predominantly affecting young children. *Most patients recover well with chemotherapy and radiotherapy.* - This statement is **true** as Wilms tumor has an **excellent prognosis** with **overall survival rates exceeding 90%** using multimodal therapy. - Treatment includes **surgery (nephrectomy), chemotherapy**, and **radiotherapy** (in selected cases), resulting in high cure rates even in advanced stages. *Presents as a renal mass.* - This statement is **true** as the typical presentation is an **asymptomatic abdominal mass** discovered incidentally by parents or during routine examination. - The mass is usually **firm, smooth, and non-tender**, representing an **intrarenal tumor** that may cause abdominal distension.

Question 196: A child with acute lymphoblastic leukemia on chemotherapy develops a fever. Which of the following is the most appropriate initial step in diagnostic assessment before starting empirical antibiotic therapy?

A. Start broad-spectrum antibiotics empirically
B. Perform a complete blood count and blood cultures (Correct Answer)
C. Administer antipyretics and observe
D. Order a PET scan to rule out metastasis

Explanation: ***Perform a complete blood count and blood cultures*** - In febrile neutropenia, **blood cultures** must be drawn immediately to identify the causative organism, and a **complete blood count (CBC)** assesses the degree of **neutropenia** (neutrophil count <500/mm³ or <1000/mm³ with predicted decline). - These investigations should be performed **urgently before initiating antibiotics** to avoid masking the pathogen, though antibiotic administration must follow within 60 minutes (door-to-needle time) as per febrile neutropenia protocols. - This diagnostic workup is the **critical first step** that guides subsequent targeted therapy and risk stratification. *Start broad-spectrum antibiotics empirically* - Empirical **broad-spectrum antibiotics** are indeed essential and should be started within **1 hour** of presentation in febrile neutropenia, but ideally *after* blood cultures are drawn. - The question specifically asks for the **diagnostic assessment step** before antibiotic therapy; starting antibiotics without cultures may compromise pathogen identification. - Standard protocols include anti-pseudomonal beta-lactams (piperacillin-tazobactam, cefepime, or meropenem) as first-line empirical therapy. *Administer antipyretics and observe* - **Antipyretics** may mask fever, which is often the only clinical sign of infection in neutropenic patients, and observation alone is dangerous in this medical emergency. - Febrile neutropenia requires **urgent intervention** within 60 minutes; a "wait and watch" approach can lead to rapid progression to septic shock and death due to profound immunosuppression. *Order a PET scan to rule out metastasis* - **PET scan** is used for staging malignancy or detecting residual disease, not for acute infection workup in febrile neutropenia. - This would cause critical delay in diagnosis and treatment of a potentially life-threatening infection and is completely inappropriate in this emergency scenario.

Question 197: A 3-year-old child presents with an abdominal mass and hypertension. Laboratory tests show high catecholamines, imaging reveals an adrenal mass, and biopsy shows small round blue cells. What is the likely diagnosis?

A. Neuroblastoma (Correct Answer)
B. Pheochromocytoma
C. Wilms tumor
D. Adrenocortical carcinoma

Explanation: ***Neuroblastoma*** - Characterized by the presence of **small round blue cells** and **high catecholamines**, indicating a **neuroendocrine origin**, typically seen in adrenal masses in children [1]. - The child's age and symptoms align with the **most common extra-cranial solid tumor** in pediatric populations, making neuroblastoma the likely diagnosis [1]. *Wilm's tumor* - Usually presents with a unilateral **renal mass** in children, not typically associated with **hypertension** or elevated catecholamines. - Histologically, it features **triphasic differentiation** and does not show small round blue cells. *Pheochromocytoma* - Generally affects adults more than children and is characterized by **episodic hypertension** and **paroxysmal symptoms** like sweating, not typically an abdominal mass in young children. - While it also shows high catecholamine levels, it lacks the small round blue cell component found in neuroblastoma [1,2]. *Adrenocortical carcinoma* - Less common in children and presents with more **indolent symptoms**, often associated with virilization or Cushing's syndrome due to steroid production [3]. - The biopsy typically shows **dramatic nuclear atypia**, which differs from the small round blue cells seen in neuroblastoma. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-486. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Liver And Biliary System Disease, pp. 419-420. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Endocrine System, pp. 1135-1137.

Question 198: A 4-year-old child presents with abdominal pain, a palpable mass, and hypertension. Urinalysis reveals hematuria. What is the most likely diagnosis?

A. Wilms tumor (Correct Answer)
B. Neuroblastoma
C. Hepatoblastoma
D. Lymphoma

Explanation: **Wilms tumor** - **Wilms tumor**, also known as **nephroblastoma**, is a common kidney cancer in children, presenting with an **abdominal mass**, abdominal pain, and often **hematuria**. - **Hypertension** can result from tumor compression of renal vasculature or renin production by the tumor itself. *Neuroblastoma* - While also a common pediatric cancer and presenting with an **abdominal mass**, neuroblastoma typically originates from **neural crest cells** in the adrenal medulla or sympathetic ganglia. - Classic symptoms often include **periorbital ecchymosis** (raccoon eyes) due to orbital metastases, and it is less commonly associated with gross hematuria directly from the tumor. *Hepatoblastoma* - **Hepatoblastoma** is a liver cancer in children, manifesting as an abdominal mass in the right upper quadrant. - While it can cause abdominal pain, it is not typically associated with **hematuria** or **hypertension**, as it does not involve the kidneys. *Lymphoma* - **Lymphoma** in children can present with an abdominal mass (e.g., Burkitt lymphoma), but it is a cancer of the lymphatic system. - It would not typically cause **hematuria** unless there was direct renal involvement, and **hypertension** is not a common primary symptom.

Question 199: A 5-year-old child presents with fever, weight loss, and bone pain. Laboratory tests reveal anemia and leukocytosis. A bone marrow biopsy shows hypercellularity with blast cells. What is the most likely diagnosis?

A. Acute lymphoblastic leukemia (Correct Answer)
B. Osteosarcoma
C. Langerhans cell histiocytosis
D. Neuroblastoma

Explanation: ***Acute lymphoblastic leukemia*** - The combination of **fever**, **weight loss**, **bone pain**, **anemia**, and **leukocytosis** in a child are classic symptoms of ALL, highly suggestive of bone marrow involvement. - A bone marrow biopsy showing **hypercellularity with blast cells** is the definitive diagnostic finding for ALL, indicating uncontrolled proliferation of immature lymphocytes. *Osteosarcoma* - Osteosarcoma is a **primary bone tumor** that primarily causes localized bone pain and may present with a palpable mass, but it is not typically associated with systemic symptoms like fever, weight loss, or characteristic peripheral blood abnormalities such as leukocytosis and anemia from bone marrow infiltration. - While it can cause bone pain and potential pathologic fractures, it does not typically show diffuse **blast cells in the bone marrow biopsy**. *Langerhans cell histiocytosis* - This condition involves proliferation of **Langerhans cells** and can cause bone lesions and pain, but it is usually associated with **lytic bone lesions** and infiltration of various organs rather than a diffuse **hypercellular marrow with blasts** and marked leukocytosis as the primary presenting feature. - Systemic symptoms like fever and weight loss can occur, but the characteristic bone marrow finding is not blast accumulation. *Neuroblastoma* - **Neuroblastoma** is a solid tumor originating from neural crest cells, often presenting as an **abdominal mass** or with symptoms related to metastatic disease (e.g., bone pain due to metastases). - While it can metastasize to bone marrow and cause bone pain and cytopenias, the bone marrow biopsy would show **small, round blue cells** forming rosettes, not a diffuse infiltration of **blast cells** characteristic of leukemia.

Question 200: A 5-year-old child presents with fatigue, pallor, bruising, bone pain, and recurrent infections. Examination reveals hepatosplenomegaly and petechiae. Laboratory findings show pancytopenia and blasts. Bone marrow biopsy indicates acute lymphoblastic leukemia (ALL). What is the next step?

A. Chemotherapy (Correct Answer)
B. Surgical intervention
C. Supportive care for anemia
D. Supportive care with steroids

Explanation: ***Chemotherapy (Correct Answer)*** - Given the diagnosis of **acute lymphoblastic leukemia (ALL)**, the primary treatment involves **multi-agent chemotherapy** to eradicate the leukemic blasts and achieve remission. - The combination of **fatigue, pallor, bruising, bone pain, recurrent infections, hepatosplenomegaly, petechiae, pancytopenia, and blasts** are classic signs and symptoms of ALL, necessitating immediate aggressive treatment. - Standard ALL protocols include **induction, consolidation, and maintenance phases** with multiple chemotherapy agents. *Supportive care with steroids (Incorrect)* - While **steroids** (prednisone or dexamethasone) are a component of ALL treatment protocols, they are typically used in conjunction with other chemotherapy agents, not as standalone "supportive care." - Monotherapy with steroids would not be sufficient to achieve remission or cure ALL and would only provide temporary relief of some symptoms. *Surgical intervention (Incorrect)* - **Surgical intervention** is generally not a primary treatment for leukemia, which is a systemic disease affecting the bone marrow and blood. - Surgery might be considered in very rare instances for complications such as a localized mass causing life-threatening compression, but it does not address the underlying systemic malignancy. *Supportive care for anemia (Incorrect)* - **Supportive care for anemia** (e.g., blood transfusions) is often necessary in patients with ALL due to reduced red blood cell production from bone marrow infiltration. - However, treating only the anemia addresses a symptom, not the underlying leukemia, and would not lead to cure or remission.

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Leukemias

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Lymphomas

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CNS Tumors

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Neuroblastoma

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Wilms Tumor

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Rhabdomyosarcoma

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Bone Tumors

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Retinoblastoma

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Histiocytosis Syndromes

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Principles of Pediatric Chemotherapy

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Hematopoietic Stem Cell Transplantation

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Late Effects of Cancer Treatment

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Oncology — MCQs

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