Oncology Practice Questions

Q: Which of the following is post-chemotherapy based staging system in Wilms' tumor?

SIOP staging system. ***SIOP staging system (Post-chemotherapy)*** - The **SIOP (International Society of Paediatric Oncology)** staging system is unique for Wilms' tumor as it incorporates **preoperative chemotherapy**. - This system stages the tumor *after* initial chemotherapy, allowing assessment of tumor response and better surgical planning. - SIOP protocol is widely used in **Europe and many international centers**. *National Wilms' tumor staging system (NWTS)* - The **NWTS** staging system is based on **surgical findings at the time of initial nephrectomy**. - It does not involve **preoperative chemotherapy** - staging occurs *before* any chemotherapy. - Predominantly used in **North America**. *AJCC TNM* - The **AJCC (American Joint Committee on Cancer) TNM staging system** is a general staging system used for many adult cancers. - While it has broad applicability, it is **not the primary or specific system** used for Wilms' tumor, especially for post-chemotherapy assessment. *Chadwick* - There is **no widely recognized cancer staging system** named Chadwick for Wilms' tumor or other cancers. - This option is **irrelevant** in the context of cancer staging.

Q: Wilms tumor commonly presents as:

Abdominal mass. ***Abdominal mass*** - Wilms tumor, or **nephroblastoma**, is the most common primary renal malignancy in children, and it typically presents as an **asymptomatic, firm, unilateral abdominal mass** in preschool-aged children. - The mass is often discovered incidentally by a parent during bathing or by a physician during a routine check-up, and it can grow quite large before other symptoms develop. *Haematuria* - While **hematuria** can occur in about 20-30% of children with Wilms tumor due to tumor extension into the renal collecting system or rupture, it is not the most common presenting symptom. - The presence of blood in the urine, in isolation, might also suggest other conditions like urinary tract infections or glomerulonephritis. *Intracranial metastasis* - **Metastasis to the brain** is rare at presentation in Wilms tumor, occurring in less than 1% of cases. - The most common sites for metastasis are the **lungs** (about 80-90% of cases) and the liver. *Abdominal pain* - **Abdominal pain** is a less common presenting symptom, occurring in about 30-40% of cases and is often associated with rapid tumor growth, hemorrhage within the tumor, or compression of surrounding structures. - It is typically not the initial symptom that brings the child to medical attention compared to the accidental discovery of a mass.

Q: A 2-year-old child presents with a testicular lump and elevated AFP levels. What is the most likely diagnosis?

Yolk sac tumor. ***Yolk sac tumor*** - This is the most common testicular tumor in children, especially prevalent before the age of 3, and is highly associated with **elevated alpha-fetoprotein (AFP)** levels. - Histologically, it is characterized by the presence of **Schiller-Duval bodies**, which are pathognomonic structures. *Choriocarcinoma* - This tumor is known for producing very high levels of **human chorionic gonadotropin (hCG)**, not AFP, and is rare in pure form in children. - It often metastasizes early and aggressively, typically presenting in older males. *Seminoma* - This is the most common testicular cancer in adult males, typically occurring in the third to fourth decades of life, making it highly unlikely in a 2-year-old. - Seminomas usually present with normal AFP levels, although some may have mildly elevated beta-hCG. *Teratoma* - While teratomas can occur in children, pure teratomas in prepubertal males are typically benign and usually do not cause elevated AFP, unless there are elements of other germ cell tumors such as yolk sac tumor within them. - Teratomas are characterized by the presence of tissues derived from all three germ layers.

Q: The most common malignancy in childhood is

ALL (Acute Lymphoblastic Leukemia). ***ALL (Acute Lymphoblastic Leukemia)*** - **ALL** is the most common malignancy diagnosed in children, accounting for approximately 25-30% of all childhood cancers. - It arises from **lymphoid progenitor cells** in the **bone marrow** and can rapidly spread to the blood, lymph nodes, spleen, liver, and central nervous system. *Bone tumor* - While bone tumors, such as **osteosarcoma** and **Ewing sarcoma**, do occur in children and adolescents, they are less common than leukemia. - Their incidence peaks in the teenage years, whereas ALL is most common in early childhood. *Retinoblastoma* - **Retinoblastoma** is the most common **intraocular malignancy** of childhood, typically presenting before the age of 5. - Although it's a significant childhood cancer, its overall incidence is much lower than that of ALL. *Wilms tumor* - Also known as **nephroblastoma**, Wilms tumor is the most common **kidney cancer** in children. - It usually presents in children aged 2-5 years but is considerably less frequent than ALL.

Q: All of the following are good prognostic factors for pediatric acute lymphoblastic leukemia, except:

CNS disease at diagnosis. ***CNS disease at diagnosis*** - The presence of **central nervous system (CNS) disease at diagnosis** in pediatric acute lymphoblastic leukemia (ALL) signifies a more aggressive form of the disease. - This involvement indicates a higher risk of relapse and is associated with a **poorer prognosis**, requiring more intensive treatment strategies. - CNS involvement is classified as a **high-risk feature** in ALL risk stratification protocols. *Hyperdiploidy* - **Hyperdiploidy**, specifically a **DNA index > 1.16** (>50 chromosomes), is considered a **favorable prognostic factor** in pediatric ALL. - It is associated with increased sensitivity to chemotherapy and thus a **better treatment outcome**. - High hyperdiploidy accounts for ~25% of pediatric ALL cases and confers excellent prognosis. *Initial WBC count <50,000/cumm* - An **initial WBC count <50,000/cumm** at diagnosis is a well-established **good prognostic factor** in pediatric ALL. - Lower WBC counts indicate lower tumor burden and are associated with **better treatment response and survival**. - WBC ≥50,000/cumm is classified as high-risk, making values below this threshold favorable. *t(12;21)* - The chromosomal translocation **t(12;21)(p13;q22)**, which results in the **ETV6-RUNX1 (TEL-AML1) fusion gene**, is the most common translocation in pediatric ALL (~25% of cases). - This genetic abnormality is indicative of a **favorable prognosis** with high rates of complete remission and a **reduced risk of relapse**. - It is associated with excellent long-term survival rates in pediatric ALL.

Q: Earliest symptom of Wilms' tumor:

Abdominal mass/ tumour. ***Abdominal mass/tumour*** - The most common initial presentation of **Wilms' tumor** is a palpable, asymptomatic **abdominal mass**. This mass is often discovered incidentally by a parent during bathing or dressing the child. - The tumor grows rapidly and can become quite large before other symptoms develop, making the mass the **earliest and most frequent finding** (present in 80-90% of cases at diagnosis). *Hematuria* - While hematuria (blood in urine) can occur in Wilms' tumor, it is a less common symptom, occurring in only **15-25% of cases**. - It results from invasion into the **renal collecting system** and is typically a later finding, not the **first symptom** noticed by caregivers. *Pyrexia* - Fever (pyrexia) is a non-specific symptom that can be associated with various conditions, including malignancy, but it is **not typically the earliest or most frequent initial symptom** of Wilms' tumor. - When present, it often indicates inflammation or systemic effects rather than the primary manifestation. *Respiratory symptoms* - Respiratory symptoms (such as cough, dyspnea, or chest pain) occur due to **pulmonary metastases**, which are present in 10-15% of cases at diagnosis. - These represent **advanced disease** and are late findings, occurring long after the primary abdominal mass would have been detectable. - The lungs are the most common site of metastatic spread in Wilms' tumor.

Q: All are true about hepatoblastoma except:

Prognosis is very poor with pulmonary metastases.. ***Prognosis is very poor with pulmonary metastases.*** - While pulmonary metastases in hepatoblastoma are a concerning finding and indicate a more advanced stage, complete cure is still possible with **aggressive multimodal treatment**, including chemotherapy and surgical resection of both primary tumor and metastases. - The prognosis is generally **better than in hepatocellular carcinoma** with metastases, as hepatoblastoma is often more responsive to chemotherapy. *Most cases occur in children less than 18 months of age.* - This statement is **true**; hepatoblastoma is the most common primary liver malignancy in children and typically presents before the age of 3, with a median age of diagnosis around 1 year. - About **two-thirds of cases** are diagnosed in children under 2 years old, making early childhood the peak incidence period. *Associated with FAP.* - This statement is **true**; hepatoblastoma has a known association with several genetic syndromes, including **Familial Adenomatous Polyposis (FAP)**, Beckwith-Wiedemann syndrome, and trisomy 18. - Children with FAP have a significantly **increased risk** of developing hepatoblastoma early in life. *Treatment is chemotherapy followed by surgical resection.* - This statement is **true** and describes the standard approach for resectable hepatoblastoma, where **neoadjuvant chemotherapy** is often used to shrink the tumor prior to surgical removal. - Postoperative chemotherapy may also be given depending on the tumor's response and staging to improve outcomes and **prevent recurrence**.

Q: Opsoclonus-myoclonus syndrome is seen in -

Neuroblastoma. ***Neuroblastoma*** - Opsoclonus-myoclonus syndrome (OMS) is a **rare paraneoplastic neurological syndrome** most commonly associated with **neuroblastoma** in children [1]. - The syndrome is characterized by **opsoclonus** (chaotic, multidirectional eye movements), **myoclonus** (brief, involuntary muscle jerks), and ataxia. *Melanoma* - Melanoma is rarely associated with OMS; however, it can be linked to other paraneoplastic syndromes like **Lambert-Eaton myasthenic syndrome** or **cerebellar degeneration** [2]. - Its primary neurological manifestations usually involve brain metastases rather than paraneoplastic syndromes. *Retinoblastoma* - Retinoblastoma is an intraocular malignancy of childhood that typically does not cause paraneoplastic syndromes like OMS. - Its neurological complications are usually related to **direct tumor extension** or leptomeningeal metastasis. *Nephroblastoma* - Nephroblastoma, also known as **Wilms' tumor**, is a kidney cancer in children. - It is not typically associated with paraneoplastic neurological syndromes like OMS; its paraneoplastic manifestations might include syndromes like **hypercalcemia** due to ectopic hormone production.

Q: Most common renal tumor in infants?

Congenital mesoblastic nephroma. ***Congenital mesoblastic nephroma*** - This is the **most common renal tumor in infants less than 3 months of age**, often detected antenatally or presenting shortly after birth. - It is a **benign tumor** originating from mesenchymal tissue, frequently seen as a large, solitary renal mass. *Neuroblastoma* - While common in infants and young children, **neuroblastoma** usually originates from **neural crest cells** in the adrenal gland or sympathetic ganglia, not primarily the kidney itself. - Though it can present as an abdominal mass, it is not primarily a renal tumor. *Rhabdoid tumor* - This is a rare, highly aggressive renal tumor that typically occurs in infants and young children, but it is **less common** than congenital mesoblastic nephroma or Wilms tumor. - Characterized by mutations in the **SMARCB1 gene** and a very poor prognosis. *Nephroblastoma* - Also known as **Wilms tumor**, nephroblastoma is the **most common primary malignant renal tumor** in children, but it typically peaks between **2 and 5 years of age**. - While it can occur in infants, congenital mesoblastic nephroma is more prevalent in the *infant* age group, especially in the first few months of life.

Q: A 1-year-old child presented with a swelling in the left flank with episodes of flushing, diarrhea, sweating and bone pain. The diagnosis is -

Neuroblastoma. ***Neuroblastoma*** - The combination of a **flank mass** in a 1-year-old child, along with paraneoplastic symptoms like **flushing, diarrhea, and sweating** (due to catecholamine secretion), and **bone pain** (indicating metastatic disease), is highly characteristic of neuroblastoma. - Neuroblastoma is a common extracranial solid tumor of childhood arising from **neural crest cells**, often presenting with abdominal mass and systemic symptoms. *Wilms tumor* - While Wilms tumor also presents as a **flank mass** in young children, it typically does not cause the systemic symptoms of flushing, diarrhea, or sweating, nor significant bone pain from metastasis. - Patients with Wilms tumor often present with **asymptomatic abdominal mass** detected incidentally. *Pheochromocytoma* - **Pheochromocytoma** can cause symptoms like flushing, sweating, and episodic hypertension due to catecholamine release, but it is **rare in children** and typically presents as an adrenal mass, not a flank mass with bone pain. - It usually presents with **hypertension crises**, which are not mentioned here. *Medulloblastoma* - **Medulloblastoma** is a **brain tumor** and would present with neurological symptoms such as headache, vomiting, and ataxia, not a flank mass or systemic symptoms like flushing, diarrhea, and sweating. - It is a **malignant tumor** of the cerebellum and does not cause peripheral masses or bone metastases in this manner.

Question 1

Which of the following is post-chemotherapy based staging system in Wilms' tumor?

Accepted Answer

SIOP staging system

Answer

National Wilms' tumor staging system (NWTS)

Answer

AJCC TNM

Answer

Chadwick

Question 2

Wilms tumor commonly presents as:

Accepted Answer

Abdominal mass

Answer

Haematuria

Answer

Intracranial metastasis

Answer

Abdominal pain

Question 3

A 2-year-old child presents with a testicular lump and elevated AFP levels. What is the most likely diagnosis?

Accepted Answer

Yolk sac tumor

Answer

Choriocarcinoma

Answer

Seminoma

Answer

Teratoma

Question 4

The most common malignancy in childhood is

Accepted Answer

ALL (Acute Lymphoblastic Leukemia)

Answer

Bone tumor

Answer

Retinoblastoma

Answer

Wilms tumor

Question 5

All of the following are good prognostic factors for pediatric acute lymphoblastic leukemia, except:

Accepted Answer

CNS disease at diagnosis

Answer

Hyperdiploidy

Answer

t(12;21)

Answer

Initial WBC count <50,000/cumm

Question 6

Earliest symptom of Wilms' tumor:

Accepted Answer

Abdominal mass/ tumour

Answer

Hematuria

Answer

Pyrexia

Answer

Respiratory symptoms

Question 7

All are true about hepatoblastoma except:

Accepted Answer

Prognosis is very poor with pulmonary metastases.

Answer

Most cases occur in children less than 18 months of age.

Answer

Associated with FAP.

Answer

Treatment is chemotherapy followed by surgical resection.

Question 8

Opsoclonus-myoclonus syndrome is seen in -

Accepted Answer

Neuroblastoma

Answer

Melanoma

Answer

Retinoblastoma

Answer

Nephroblastoma

Question 9

Most common renal tumor in infants?

Accepted Answer

Congenital mesoblastic nephroma

Answer

Neuroblastoma

Answer

Rhabdoid tumor

Answer

Nephroblastoma

Question 10

A 1-year-old child presented with a swelling in the left flank with episodes of flushing, diarrhea, sweating and bone pain. The diagnosis is -

Accepted Answer

Neuroblastoma

Answer

Wilms tumor

Answer

Pheochromocytoma

Answer

Medulloblastoma

Oncology — MCQs

Oncology — MCQs

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