Oncology Practice Questions

Q: 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?

Nephroblastoma. ***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.

Q: Most common manifestation of Wilms tumor is ________

Abdominal mass. ***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.

Q: Not seen in Neuroblastoma is -

Splenomegaly. ***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.

Q: Opsoclonus is associated with:

Neuroblastoma. ***Neuroblastoma*** - **Opsoclonus-myoclonus syndrome (OMS)**, characterized by rapid, chaotic eye movements (opsoclonus) and myoclonus, is a well-recognized paraneoplastic syndrome associated with **neuroblastoma** in children. - This syndrome is thought to be caused by an **autoimmune response** against neural antigens shared by the tumor and the central nervous system [1]. *Renal cell carcinoma* - While renal cell carcinoma can present with various paraneoplastic syndromes, **opsoclonus** is not typically associated with it. - Common paraneoplastic syndromes for renal cell carcinoma include **polycythemia**, hypercalcemia, and hypertension [2]. *Wilms tumor* - **Wilms tumor**, a common pediatric kidney cancer, is generally not associated with neurological paraneoplastic syndromes like opsoclonus. - It is known for its association with certain **congenital anomalies** (e.g., aniridia, hemihypertrophy) but not opsoclonus. *Retinal tumor* - Retinal tumors primarily affect vision and are associated with **ocular symptoms**, such as vision loss or visual field defects. - They do not typically cause systemic neurological paraneoplastic syndromes like **opsoclonus-myoclonus**.

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

Lung metastasis is rare and occurs late. ***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.

Q: 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?

Neuroblastoma. ***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.

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

Wilms tumor. **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.

Q: 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?

Acute lymphoblastic leukemia. ***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 1

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?

Accepted Answer

Nephroblastoma

Answer

Retinoblastoma

Answer

Hepatoblastoma

Answer

Medulloblastoma

Question 2

Most common manifestation of Wilms tumor is ________

Accepted Answer

Abdominal mass

Answer

Loss of weight

Answer

Hypertension

Answer

Haematuria

Question 3

Not seen in Neuroblastoma is -

Accepted Answer

Splenomegaly

Answer

Bone involvement

Answer

Diarrhoea

Answer

Proptosis

Question 4

Opsoclonus is associated with:

Accepted Answer

Neuroblastoma

Answer

Renal cell carcinoma

Answer

Wilms tumor

Answer

Retinal tumor

Question 5

Which of the following statements regarding Wilms tumor is false?

Accepted Answer

Lung metastasis is rare and occurs late

Answer

Most commonly seen < 5 years of age

Answer

Most patients recover well with chemotherapy and radiotherapy.

Answer

Presents as a renal mass.

Question 6

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?

Accepted Answer

Perform a complete blood count and blood cultures

Answer

Start broad-spectrum antibiotics empirically

Answer

Administer antipyretics and observe

Answer

Order a PET scan to rule out metastasis

Question 7

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?

Accepted Answer

Neuroblastoma

Answer

Pheochromocytoma

Answer

Wilms tumor

Answer

Adrenocortical carcinoma

Question 8

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

Accepted Answer

Wilms tumor

Answer

Neuroblastoma

Answer

Hepatoblastoma

Answer

Lymphoma

Question 9

A 7-year-old child presents with ataxia and slurred speech. An MRI shows a mass in the cerebellum. What is the most likely diagnosis?

Accepted Answer

Astrocytoma

Answer

Brainstem glioma

Answer

Arachnoid cyst

Answer

Medulloblastoma

Question 10

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?

Accepted Answer

Acute lymphoblastic leukemia

Answer

Osteosarcoma

Answer

Langerhans cell histiocytosis

Answer

Neuroblastoma

Oncology — MCQs

Oncology — MCQs

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