Oncology Practice Questions

Q: A child presented in the OPD with multiple permeating lesions involving all the bones of the body. Which of the following is the most probable diagnosis?

Histiocytosis X. ***Histiocytosis X*** - **Histiocytosis X**, now known as **Langerhans cell histiocytosis (LCH)**, can present with lytic bone lesions, which may be widespread and "permeating" throughout the skeletal system in children. - The disease involves the proliferation of **Langerhans cells**, and bone involvement is a common manifestation, leading to multiple osteolytic lesions. *Neuroblastoma* - **Neuroblastoma** is a common childhood cancer that frequently metastasizes to bone, leading to lytic lesions; however, it often presents with other symptoms like an abdominal mass, opsoclonus-myoclonus syndrome, or proptosis due to orbital involvement. - While bone metastases can be extensive, the primary tumor is typically extraosseous, originating from neural crest cells. *Metastasis from Wilm's tumour* - **Wilms' tumor** primarily affects the kidney and typically metastasizes to the lungs, liver, and occasionally bone marrow. - While bone metastasis can occur, it's less common than in neuroblastoma and less likely to present as widespread "permeating" osseous lesions throughout all bones, as described. *Metastasis from osteosarcoma* - **Osteosarcoma** is a primary malignant bone tumor, and its metastases usually occur in the lungs. - While it can metastasize to other bones, it generally presents as new, discrete osteoblastic or mixed lesions rather than a widespread permeating pattern across the entire skeletal system.

Q: Most common cancer in children less than 10 years:

Leukemia. ***Leukemia*** - Leukemia, particularly **acute lymphoblastic leukemia (ALL)**, is the most frequently diagnosed cancer in children under 10 years of age. - It arises from the rapid production of abnormal white blood cells that accumulate in the bone marrow, interfering with the production of normal blood cells. *Neuroblastoma* - While a common solid tumor in childhood, particularly in infants, it accounts for a smaller percentage of all childhood cancers compared to leukemia. - Originates from **neuroblasts** (immature nerve cells), typically in the adrenal glands or sympathetic nervous system. *Wilm's tumor* - This is a common **kidney cancer** in children, usually affecting those aged 3-4 years. - Although significant, its incidence is lower than that of leukemia. *Brain tumor* - Brain and other central nervous system tumors are the **second most common type of cancer in children**, after leukemia. - They encompass various types and can occur at any age during childhood.

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: All of the following are true about long-term sequelae of craniospinal radiotherapy for children with CNS tumors except:

Neuropsychological sequelae are independent of radiation dose. ***Neuropsychological sequelae are independent of radiation dose*** - This statement is incorrect; **neuropsychological sequelae** are **highly dependent on the radiation dose** received by the brain, with higher doses generally leading to more severe and frequent cognitive impairments. - The impact of radiation on developing neural tissue is dose-related, affecting **myelination**, **synaptogenesis**, and **neurogenesis**, leading to dose-dependent cognitive deficits. *Musculoskeletal hypoplasia* - **Craniospinal irradiation (CSI)** can lead to musculoskeletal hypoplasia, particularly affecting the **vertebrae** and **long bones**, resulting in **short stature** and **scoliosis**. - Radiation can damage growth plates and bone-forming cells, impairing normal bone development and leading to growth deficiencies. *Neurocognitive dysfunction* - **Radiation to the brain** in children, especially at a young age, can cause significant **neurocognitive dysfunction**, including deficits in **memory**, **attention**, **processing speed**, and **executive functions**. - Damage to **white matter**, particularly secondary to demyelination and vasculopathy, plays a significant role in these cognitive impairments. *Endocrinologic dysfunction* - **CSI** frequently affects the **hypothalamic-pituitary axis**, leading to various **endocrinologic dysfunctions** such as **growth hormone deficiency**, **thyroid dysfunction**, and **gonadal dysfunction**. - The developing endocrine glands and their regulatory centers are particularly sensitive to radiation, impacting hormonal production and regulation.

Question 1

A child presented in the OPD with multiple permeating lesions involving all the bones of the body. Which of the following is the most probable diagnosis?

Accepted Answer

Histiocytosis X

Answer

Neuroblastoma

Answer

Metastasis from Wilm's tumour

Answer

Metastasis from osteosarcoma

Question 2

Most common cancer in children less than 10 years:

Accepted Answer

Leukemia

Answer

Neuroblastoma

Answer

Wilm's tumor

Answer

Brain tumor

Question 3

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 4

Wilms tumor commonly presents as:

Accepted Answer

Abdominal mass

Answer

Haematuria

Answer

Intracranial metastasis

Answer

Abdominal pain

Question 5

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 6

The most common malignancy in childhood is

Accepted Answer

ALL (Acute Lymphoblastic Leukemia)

Answer

Bone tumor

Answer

Retinoblastoma

Answer

Wilms tumor

Question 7

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 8

Earliest symptom of Wilms' tumor:

Accepted Answer

Abdominal mass/ tumour

Answer

Hematuria

Answer

Pyrexia

Answer

Respiratory symptoms

Question 9

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 10

All of the following are true about long-term sequelae of craniospinal radiotherapy for children with CNS tumors except:

Accepted Answer

Neuropsychological sequelae are independent of radiation dose

Answer

Musculoskeletal hypoplasia

Answer

Neurocognitive dysfunction

Answer

Endocrinologic dysfunction

Oncology — MCQs

Oncology — MCQs

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