Oncology Practice Questions

Q: Most common site of extramedullary relapse of ALL in a 6-year-old boy is

CNS (Central Nervous System). ***CNS (Central Nervous System)*** - The **central nervous system** is the **most common site of extramedullary relapse** in pediatric acute lymphoblastic leukemia (ALL), accounting for the majority of extramedullary relapses. - CNS is an immunologically privileged sanctuary site where chemotherapy penetration is limited due to the blood-brain barrier. - CNS relapse presents with symptoms like headache, vomiting, cranial nerve palsies, or signs of increased intracranial pressure and requires intrathecal chemotherapy and cranial irradiation. - Prophylactic CNS therapy is a standard component of ALL treatment protocols to prevent CNS relapse. *Testes* - **Testes** are the **second most common** site of extramedullary relapse and the most common **solid organ** site in boys with ALL. - Like the CNS, testes are immunologically privileged sites with limited chemotherapy penetration. - Testicular relapse presents as painless testicular enlargement (unilateral or bilateral) and requires testicular radiation plus systemic therapy intensification. *Liver* - While **hepatic infiltration** can occur in ALL, the liver is not a common site for **isolated extramedullary relapse**. - Hepatic involvement typically indicates widespread systemic disease rather than a primary relapse site. *Lung* - **Pulmonary involvement** in ALL is rare as an isolated extramedullary relapse site. - Lung findings in ALL patients are more commonly due to infection, leukostasis in hyperleukocytosis, or disseminated disease.

Q: What is the most common soft tissue sarcoma found in children?

Rhabdomyosarcoma. ***Rhabdomyosarcoma*** - **Rhabdomyosarcoma** is the most prevalent soft tissue sarcoma in pediatric populations, accounting for a significant portion of childhood soft tissue malignancies. - It arises from **mesenchymal cells** that fail to differentiate into mature muscle cells and can occur in various sites, including the head and neck, genitourinary tract, and extremities. *Synovial sarcoma* - While it can occur in children and young adults, **synovial sarcoma** is more common in adolescents and young adults, typically affecting the extremities near large joints. - It is not the most common soft tissue sarcoma observed across all pediatric age groups. *Liposarcoma* - **Liposarcoma**, a malignant tumor of adipose tissue, is exceedingly rare in children. - It primarily affects adults, particularly those in middle to older age. *Fibrosarcoma* - **Fibrosarcoma** is a rare malignant tumor of fibroblasts that can occur in children, but it is much less common than rhabdomyosarcoma. - There are different types, and while some variants show up in congenital or infantile forms, overall incidence is low.

Q: A malignant tumor of childhood that metastasizes to bones most often is -

Neuroblastoma. ***Neuroblastoma*** - **Neuroblastoma** is a common childhood cancer that frequently **metastasizes to bones**, bone marrow, and lymph nodes. - It arises from **neural crest cells** and often presents with symptoms related to bone pain or masses in other sites. *Wilm's tumor* - **Wilm's tumor** (nephroblastoma) is a kidney cancer of childhood that primarily metastasizes to the **lungs** and liver. - While it can, in rare cases, involve bones, it is not the most common site of bone metastasis for childhood tumors. *Rhabdomyosarcoma* - **Rhabdomyosarcoma** is a soft tissue sarcoma that can occur in various body sites, including the head, neck, and genitourinary tract. - While it can metastasize, its most common sites of metastasis include the lungs, lymph nodes, and bone marrow, but **not predominantly bones**. *Granulosa cell tumor of the ovary* - **Granulosa cell tumor** is a rare ovarian tumor that primarily affects girls and young women. - It typically metastasizes locally within the abdomen or to regional lymph nodes, and **does not commonly metastasize to bones**.

Q: Hodgkin lymphoma in children is predominantly seen at what age?

> 14 years. ***> 14 years*** - Hodgkin lymphoma in children shows **peak incidence in adolescence**, specifically between **10-19 years of age**, with the highest peak at **15-19 years**. - Among pediatric cases, the predominant age group is **adolescents over 14 years**, making this the correct answer. - It is one of the most common malignancies in the **teenage years** and remains within the pediatric age spectrum (up to 18-21 years). * 14 years" the more accurate answer for predominant pediatric occurrence. *< 4 years* - Hodgkin lymphoma is **extremely rare** in children younger than 4 years old. - Other pediatric malignancies like **neuroblastoma** or **acute leukemia** are more commonly seen in this age group. - This age range accounts for a **negligible proportion** of pediatric Hodgkin lymphoma cases. *< 1 year* - Hodgkin lymphoma is **virtually non-existent** in infants under 1 year of age. - **Congenital anomalies**, **neuroblastoma**, or **infantile leukemia** are more common diagnoses in this age group. - There are only rare case reports of Hodgkin lymphoma in infancy.

Q: Mrs. Neena noted an abdominal mass on the right side of her 6-month-old child, which showed calcification near the right kidney. What is the likely cause?

Neuroblastoma. ***Neuroblastoma*** - This is the most likely diagnosis due to the presence of an **abdominal mass** with **calcification** in a 6-month-old child, as neuroblastoma frequently arises from the adrenal medulla and often presents with calcifications. - Neuroblastoma is typically diagnosed in **early childhood** and is known for its tendency to metastasize widely. *Leukemia* - Leukemia is a **hematologic malignancy** that primarily affects the blood and bone marrow, not typically presenting as a solid abdominal mass with calcifications. - While it can manifest with various symptoms, an isolated **calcified abdominal mass** is not a characteristic presentation. *Renal cell carcinoma* - Renal cell carcinoma is a **kidney cancer** that is rare in infants and young children, typically affecting older adults. - Although it can present as a renal mass, **calcification** is less common than in neuroblastoma in this age group, and the primary tumor originates from renal parenchymal cells. *Lymphoma* - Lymphoma is a cancer of the **lymphatic system** and can cause abdominal masses, but these are typically non-calcified lymphadenopathy rather than a mass with distinct calcification. - While it can occur in children, the presence of **calcification near the kidney** is not a hallmark feature of lymphoma in infants.

Q: A 5-year-old child is admitted with headache, vomiting, and difficulty in walking. Physical findings include truncal ataxia, papilloedema, and left lateral rectus palsy. No finger-to-nose ataxia could be detected on either the left or right side. The most likely diagnosis is –

Medulloblastoma. ***Medulloblastoma*** - The combination of **headache, vomiting** (due to increased ICP), **difficulty walking, truncal ataxia**, and **papilledema** in a 5-year-old child strongly suggests a posterior fossa tumor. **Lateral rectus palsy** indicates an abducens nerve (CN VI) compression, common with raised ICP. - The absence of **finger-to-nose ataxia** specifically points away from a primary cerebellar hemispheric lesion, making a vermian tumor like **medulloblastoma** (arising from the cerebellar vermis) a prime candidate for explaining the truncal ataxia. *Dandy-Walker malformation* - This congenital brain malformation involves agenesis or hypoplasia of the **cerebellar vermis**, cystic dilation of the fourth ventricle, and an enlarged posterior fossa. - While it can cause ataxia and hydrocephalus with associated symptoms, it is a congenital condition typically presenting earlier in infancy or with more static neurological deficits rather than the progressive symptoms described. *Syringomyelia* - This condition involves a **fluid-filled cyst (syrinx)** within the spinal cord, leading to symptoms like pain, weakness, and sensory deficits, often in the upper extremities. - It does not typically cause the combination of **headache, vomiting, papilledema**, and **truncal ataxia** observed, as these are primarily signs of increased intracranial pressure and posterior fossa involvement. *Arnold-Chiari malformation type I* - This malformation involves the **downward displacement of cerebellar tonsils** into the foramen magnum, often causing headache, neck pain, and sensory deficits due to brainstem and spinal cord compression. - While it can cause ataxia, it typically presents in adolescence or adulthood and does not usually cause the acute or rapidly progressive signs of **increased intracranial pressure** (papilledema, vomiting, progressive headache) seen in this young child.

Q: Wilms' tumor: What is the most common presenting symptom?

Asymptomatic abdominal mass. ***Asymptomatic abdominal mass*** - Wilms' tumor, or **nephroblastoma**, commonly presents as an **abdominal mass** discovered incidentally by a parent or during a routine physical examination. - This mass is typically firm, smooth, and located in the **flank**, and is usually not painful in its early stages. *Haematuria* - While haematuria (blood in the urine) can occur in Wilms' tumor, it is a **less common** initial symptom compared to an abdominal mass. - Its presence often suggests a more advanced stage or involvement of the **pelvic collecting system**. *Abdominal pain* - Abdominal pain may be present in children with Wilms' tumor, but it is **not the most common initial presenting symptom**. - When present, it can indicate rapid tumor growth, hemorrhage, or obstruction, and is usually a **secondary complaint** to the palpable mass. *Headache* - Headache is **not a typical presenting symptom** of Wilms' tumor. - If a headache were present, it would more likely suggest **metastatic disease** involving the brain, which is rare at initial presentation, or an unrelated issue.

Q: Most common intra-abdominal solid organ tumor in children is

Wilms tumor. ***Wilms tumor*** - **Wilms tumor**, also known as **nephroblastoma**, is the most common primary malignant renal tumor of childhood and the most common intra-abdominal solid organ tumor in children. - It typically presents as an asymptomatic abdominal mass, often discovered incidentally. *Neuroblastoma* - **Neuroblastoma** is the most common extracranial solid tumor in childhood and the most common cancer in infancy. - While it frequently occurs in the abdomen (adrenal gland accounts for ~40% of cases), its origin in **neural crest cells** distinguishes it from kidney tumors like Wilms tumor. *Rhabdomyoblastoma* - This term is likely a misspelling or misunderstanding; the correct term for a malignant tumor of skeletal muscle is **rhabdomyosarcoma**. - **Rhabdomyosarcoma** is a common soft tissue sarcoma in children but is not typically classified as the most common intra-abdominal solid organ tumor overall. *Hypernephroma* - **Hypernephroma** is an older term for **renal cell carcinoma (RCC)**. - While RCC is the most common kidney cancer in adults, it is extremely rare in children compared to Wilms tumor.

Q: What is the most common malignant tumor of the kidney in children?

Wilms tumor. ***Wilms tumor*** - Wilms tumor, or **nephroblastoma**, is the most common type of kidney tumor in children, typically presenting with an **abdominal mass** [2]. - Most patients are diagnosed between the ages of **2 and 5** [3], and it is often associated with syndromic conditions like **WAGR syndrome** [1]. *PCKD* - **Polycystic Kidney Disease (PCKD)** is a genetic disorder that leads to kidney enlargement but is not classified as a tumor. - While it can lead to complications like hypertension, it does not typically present as a **neoplastic mass** in children. *Angioliposarcoma* - This is a rare tumor that occurs primarily in adults, characterized by **fatty tissue** and vascular infiltrations, making it an uncommon kidney tumor in children. - Typically, renal tumors in children are **Wilms tumor** rather than **sarcomas** like angioliposarcoma. *Neuroblastoma* - Neuroblastoma is an **extrarenal tumor** that commonly arises from the adrenal glands or sympathetic nervous tissue [3], rather than directly from the kidney. - It presents with **abdominal masses** but is distinct from kidney tumors, which makes it less relevant in the context of renal tumors in children. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 487-488. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 488-490. [3] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-484.

Q: Treatment of choice for stage 4S neuroblastoma is

Observation. ***Observation*** - Stage 4S neuroblastoma is a **unique subset** affecting infants under 1 year, characterized by spontaneous regression in many cases. - Due to its often self-limiting nature, initial **observation with close monitoring** is the preferred management strategy. *Surgery* - While surgery may be used for symptom control or biopsy, it is **not the primary treatment** of choice for early-stage neuroblastoma or stage 4S, which has a favorable prognosis with observation. - **Complete surgical resection** is often difficult or unnecessary in stage 4S, given the potential for spontaneous regression. *Chemotherapy* - Chemotherapy is typically reserved for **high-risk neuroblastoma** or cases of stage 4S that show progression or fail to regress spontaneously. - It carries significant side effects and is **not indicated as initial therapy** for most stage 4S patients. *Radiotherapy* - Radiotherapy is generally used for **local control** in advanced or recurrent neuroblastoma and to manage painful bone metastases. - It is **not a standard initial treatment** for stage 4S due to its systemic nature and the excellent prognosis with observation.

Question 1

Most common site of extramedullary relapse of ALL in a 6-year-old boy is

Accepted Answer

CNS (Central Nervous System)

Answer

Testes

Answer

Liver

Answer

Lung

Question 2

What is the most common soft tissue sarcoma found in children?

Accepted Answer

Rhabdomyosarcoma

Answer

Liposarcoma

Answer

Synovial sarcoma

Answer

Fibrosarcoma

Question 3

A malignant tumor of childhood that metastasizes to bones most often is -

Accepted Answer

Neuroblastoma

Answer

Wilm's tumor

Answer

Rhabdomyosarcoma

Answer

Granulosa cell tumor of the ovary

Question 4

Hodgkin lymphoma in children is predominantly seen at what age?

Accepted Answer

> 14 years

Answer

< 4 years

Answer

< 14 years

Answer

< 1 year

Question 5

Mrs. Neena noted an abdominal mass on the right side of her 6-month-old child, which showed calcification near the right kidney. What is the likely cause?

Accepted Answer

Neuroblastoma

Answer

Leukemia

Answer

Renal cell carcinoma

Answer

Lymphoma

Question 6

A 5-year-old child is admitted with headache, vomiting, and difficulty in walking. Physical findings include truncal ataxia, papilloedema, and left lateral rectus palsy. No finger-to-nose ataxia could be detected on either the left or right side. The most likely diagnosis is –

Accepted Answer

Medulloblastoma

Answer

Arnold-Chiari malformation type I

Answer

Dandy-Walker malformation

Answer

Syringomyelia

Question 7

Wilms' tumor: What is the most common presenting symptom?

Accepted Answer

Asymptomatic abdominal mass

Answer

Haematuria

Answer

Abdominal pain

Answer

Headache

Question 8

Most common intra-abdominal solid organ tumor in children is

Accepted Answer

Wilms tumor

Answer

Rhabdomyosarcoma

Answer

Hypernephroma

Answer

Neuroblastoma

Question 9

What is the most common malignant tumor of the kidney in children?

Accepted Answer

Wilms tumor

Answer

Clear cell sarcoma of the kidney

Answer

Rhabdoid tumor of the kidney

Answer

Neuroblastoma

Question 10

Treatment of choice for stage 4S neuroblastoma is

Accepted Answer

Observation

Answer

Surgery

Answer

Chemotherapy

Answer

Radiotherapy

Oncology — MCQs

Oncology — MCQs

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