Pediatric Tumors — MCQs
A 3-year-old boy is found to have spontaneous bursts of non-rhythmic conjugate eye movements in various directions, as well as hypotonia and myoclonus. Physical examination also reveals an abdominal mass. A CT scan shows a mass in the adrenal gland. Which of the following statements is false regarding the patient's condition?
A renal mass seen on prenatal check-up is most probably:
Opsoclonus-myoclonus is a phenomenon seen in:
Which of the following childhood tumor uses N-myc gene amplification for its prognosis?
What is the most common malignant tumor of the kidney in children?
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?
On USG, a mass was found in the abdomen which was displacing the kidney laterally in a 1-year-old child. What is the most likely diagnosis?
Most common malignant tumour in childhood:
Which one of the following regarding abdominal pediatric surgery is correct?
Most common cause of acute intestinal obstruction in children is
Pediatric Tumors Indian Medical PG Practice Questions and MCQs
Question 1: A 3-year-old boy is found to have spontaneous bursts of non-rhythmic conjugate eye movements in various directions, as well as hypotonia and myoclonus. Physical examination also reveals an abdominal mass. A CT scan shows a mass in the adrenal gland. Which of the following statements is false regarding the patient's condition?
- A. Immunohistochemical detection of chromogranin is not useful for diagnosis. (Correct Answer)
- B. 70-80% of tumors are associated with elevated production of catecholamines.
- C. Rearrangement or deletion of short arm of chromosome 1 is seen in 25-35% of cases.
- D. The most common site of tumor is adrenal medulla.
Explanation: ***Immunohistochemical detection of chromogranin is not useful for diagnosis.*** - This statement is **false** because **neuroblastoma** cells, which originate from neural crest cells, commonly express **chromogranin A** and C, along with other neuroendocrine markers like **synaptophysin** and **neuron-specific enolase (NSE)** [1]. - **Immunohistochemical staining** for chromogranin is thus a **useful diagnostic tool** to confirm the neuroendocrine differentiation of the tumor [1]. *The most common site of tumor is adrenal medulla.* - This statement is **true**. Approximately **50% of neuroblastomas** originate in the **adrenal glands**, specifically the adrenal medulla, because it is derived from neural crest cells, the precursor cells for neuroblastoma [1]. - Other common sites include the paraspinal ganglia, such as the posterior mediastinum, pelvis, and neck. *70-80% of tumors are associated with elevated production of catecholamines.* - This statement is **true**. Neuroblastoma cells often retain the ability to synthesize and secrete **catecholamines** (**epinephrine, norepinephrine, dopamine**) [1]. - Elevated levels of **vanillylmandelic acid (VMA)** and **homovanillic acid (HVA)**, which are the **breakdown products (metabolites) of catecholamines**, are detected in the urine of 70-80% of patients and serve as **important diagnostic and prognostic markers** [1]. *Rearrangement or deletion of short arm of chromosome 1 is seen in 25-35% of cases.* - This statement is **true**. **Deletion** or **rearrangement** of the **short arm of chromosome 1 (1p36)** is a common **cytogenetic abnormality** found in 25-35% of neuroblastomas. - This genetic alteration is often associated with **poor prognosis** and more aggressive disease behavior. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-487.
Question 2: A renal mass seen on prenatal check-up is most probably:
- A. Neuroblastoma
- B. Wilms' tumor
- C. Mesoblastic nephroma (Correct Answer)
- D. Renal sarcoma
Explanation: ***Mesoblastic nephroma*** - This is the most common **congenital renal tumor** and is often detected antenatally or in the **neonatal period**. - It is typically a **benign tumor** that presents as a solid, well-circumscribed renal mass. *Neuroblastoma* - While it can present as an abdominal mass in infants, **neuroblastoma** usually arises from adrenal glands or sympathetic ganglia, not primarily the kidney itself. - It often shows features of calcification and can be associated with elevated **catecholamines**. *Wilms' tumor* - **Wilms' tumor** is the most common childhood renal malignancy, but it is typically diagnosed in older infants and young children (peak incidence at 2-3 years), less commonly detected definitively on prenatal check-ups. - Prenatal detection of **Wilms' tumor** is rare, and it usually grows rapidly postnatally. *Renal sarcoma* - **Renal sarcoma** is an extremely rare primary renal tumor in children, making it a much less likely diagnosis than mesoblastic nephroma for a prenatal renal mass. - These tumors tend to be aggressive but are statistically much less common in the prenatal period.
Question 3: Opsoclonus-myoclonus is a phenomenon seen in:
- A. Wilms' tumor
- B. Meningioma
- C. Cortical tuberculoma
- D. Neuroblastoma (Correct Answer)
Explanation: ***Neuroblastoma*** - **Opsoclonus-myoclonus syndrome (OMS)** is a rare paraneoplastic neurological disorder primarily associated with childhood neuroblastoma. - It is characterized by rapid, irregular eye movements (**opsoclonus**), brief, involuntary muscle jerks (**myoclonus**), ataxia, and irritability. *Wilm's tumor* - Wilms' tumor (nephroblastoma) is a kidney tumor of childhood and is not typically associated with opsoclonus-myoclonus syndrome. - While it is also a pediatric cancer, its paraneoplastic manifestations are different and do not include OMS. *Meningioma* - Meningiomas are typically slow-growing tumors arising from the meninges in adults, and are not associated with opsoclonus-myoclonus. - Paraneoplastic syndromes are rare with meningiomas, and OMS is not one of them. *Cortical tuberculoma* - A cortical tuberculoma is a granulomatous lesion in the brain caused by Mycobacterium tuberculosis, often seen in individuals with tuberculosis. - While it can cause neurological symptoms like seizures, headaches, and focal deficits, it does not cause opsoclonus-myoclonus syndrome.
Question 4: Which of the following childhood tumor uses N-myc gene amplification for its prognosis?
- A. Neuroblastoma (Correct Answer)
- B. Nephroblastoma
- C. Retinoblastoma
- D. Rhabdomyosarcoma
Explanation: ***Neuroblastoma*** - **N-myc gene amplification** is a crucial **prognostic indicator** in neuroblastoma, correlating with aggressive disease and poor outcomes [1]. - Neuroblastoma is a **childhood cancer of neural crest origin**, often presenting as an adrenal mass or in sympathetic ganglia. *Nephroblastoma* - Also known as **Wilms tumor**, it is a childhood kidney cancer. - Its prognosis is more strongly associated with histology (e.g., **anaplasia**) and specific gene mutations like **WT1**, not N-myc amplification. *Retinoblastoma* - This is a **childhood eye cancer**. - Its prognosis is primarily linked to the presence of **RB1 gene mutations** and the extent of retinoblastoma gene protein (pRB) expression, not N-myc. *Rhabdomyosarcoma* - An aggressive **childhood soft tissue sarcoma** with skeletal muscle differentiation. - Prognostic factors often include clinical staging, histology (e.g., **alveolar vs. embryonal**), and specific genetic translocations like **PAX-FOXO1**, rather than N-myc amplification. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 486-487.
Question 5: What is the most common malignant tumor of the kidney in children?
- A. Wilms tumor (Correct Answer)
- B. Clear cell sarcoma of the kidney
- C. Rhabdoid tumor of the kidney
- D. Neuroblastoma
Explanation: ***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.
Question 6: 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 7: On USG, a mass was found in the abdomen which was displacing the kidney laterally in a 1-year-old child. What is the most likely diagnosis?
- A. Neuroblastoma (Correct Answer)
- B. Wilms' tumor
- C. Renal cell carcinoma
- D. All of the options
Explanation: ***Neuroblastoma*** - A retroperitoneal mass displacing the kidney laterally in an infant is highly characteristic of **neuroblastoma**, which originates from neural crest cells in the adrenal gland or sympathetic ganglia. - The key finding is **extrarenal origin** - the mass pushes the kidney aside rather than arising from within it. - Most common extrarenal abdominal mass in children under 2 years. *Wilms' tumor* - This is an **intrarenal mass** that originates within the kidney parenchyma. - Wilms' tumor **expands and distorts the kidney** rather than displacing it laterally from outside. - Most common renal tumor in children (peak age 3-4 years), presenting with abdominal mass, hematuria, and hypertension. *Renal cell carcinoma* - Exceedingly **rare in a 1-year-old child** - primarily an adult malignancy (typically >40 years). - Would be an intrarenal mass, not an extrarenal mass displacing the kidney laterally. *All of the options* - Incorrect because the specific imaging finding of **lateral kidney displacement** indicates an extrarenal origin, which is pathognomonic for neuroblastoma, not the intrarenal tumors listed.
Question 8: Most common malignant tumour in childhood:
- A. CNS tumours
- B. Rhabdomyosarcoma
- C. Neuroblastoma
- D. Leukaemia (Correct Answer)
Explanation: ***Leukaemia*** - **Leukaemia** is the **most common paediatric malignancy**, accounting for approximately 30% of all childhood cancers. - It involves the uncontrolled proliferation of abnormal white blood cells, primarily in the **bone marrow** and blood. - **Acute lymphoblastic leukaemia (ALL)** is the most common subtype. *CNS tumours* - **CNS tumours** (brain and spinal cord tumours) are the **second most common** paediatric malignancy, accounting for 20-25% of childhood cancers. - Include medulloblastoma, gliomas, and ependymomas. - While common, they are less frequent than leukaemia. *Rhabdomyosarcoma* - **Rhabdomyosarcoma** is a malignant tumour of **mesenchymal origin**, typically affecting skeletal muscle. - While it is the most common soft tissue sarcoma in children, it is not the most common overall childhood malignancy. *Neuroblastoma* - **Neuroblastoma** is a malignant tumour derived from **neural crest cells**, often arising in the adrenal glands or sympathetic nervous system. - It is one of the most common solid extracranial tumours in children but is less common than leukaemia.
Question 9: Which one of the following regarding abdominal pediatric surgery is correct?
- A. Transverse abdominal incision is always used
- B. Bowel must be always anastomosed in double layer
- C. Skin over abdomen can never be closed with subcuticular sutures
- D. Incision can be closed with absorbable suture (Correct Answer)
Explanation: ***Incision can be closed with absorbable suture*** - **Absorbable sutures** are commonly used in pediatric abdominal surgery for closing deeper layers and sometimes skin, as they degrade over time and do not require removal. - This is particularly beneficial in children to avoid the trauma and discomfort of suture removal and to promote good cosmetic outcomes. *Transverse abdominal incision is always used* - While **transverse incisions** are often preferred in pediatric abdominal surgery for their good cosmetic results and lower incidence of incisional hernias, they are not *always* used. - Other incisions, such as **vertical midline incisions**, may be utilized depending on the surgical exposure required, the specific pathology, or the surgeon's preference. *Bowel must be always anastomosed in double layer* - **Bowel anastomoses** in pediatric surgery can be performed using either a **single-layer** or **double-layer** technique. - The choice depends on surgeon preference, the specific bowel segment involved, and the patient's condition, with both methods demonstrating comparable outcomes in many situations. *Skin over abdomen can never be closed with subcuticular sutures* - **Subcuticular sutures** are frequently used for skin closure in pediatric abdominal surgery, especially for their excellent cosmetic results and to avoid external suture removal. - This technique places the suture material under the skin surface, minimizing scarring and being well-suited for a child's healing skin.
Question 10: Most common cause of acute intestinal obstruction in children is
- A. Inguinal hernia
- B. Intussusception (Correct Answer)
- C. Volvulus
- D. None of the options
Explanation: ***Intussusception*** - **Intussusception** is the most common cause of **acute intestinal obstruction** in children, particularly between 3 months and 3 years of age. - It occurs when a segment of the intestine telescopes into an adjacent segment, leading to obstruction and potentially **ischemia**. *Inguinal hernia* - While an **incarcerated inguinal hernia** can cause intestinal obstruction, it is less common than intussusception as the primary cause of acute obstruction in children generally. - It is more frequent in **neonates and infants** but overall incidence of obstruction is lower than intussusception. *Volvulus* - **Volvulus** refers to a twisting of the intestine on its mesentery, often associated with **malrotation**, leading to obstruction and vascular compromise. - While a serious cause of obstruction, especially in neonates, it is less common overall than intussusception in the pediatric population. *None of the options* - This option is incorrect because **intussusception** is a recognized and frequent cause of acute intestinal obstruction in children.
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