Neuroblastoma — 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?
Opsoclonus-myoclonus is a phenomenon seen in:
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 -
Which of the following childhood tumor uses N-myc gene amplification for its prognosis?
The most common site of metastasis in neuroblastoma is?
Neuroblastoma originates from –
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 1-year-old unimmunised child with HbsAg positivity is having following skin lesions. What is the diagnosis?
What is the most common malignant neoplasm of infancy?
Wilms' tumor is associated with which of the following conditions?
Neuroblastoma 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: 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 3: 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 -
- A. Wilms tumor
- B. Pheochromocytoma
- C. Neuroblastoma (Correct Answer)
- D. Medulloblastoma
Explanation: ***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 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: The most common site of metastasis in neuroblastoma is?
- A. Lung
- B. Liver
- C. Lymph nodes
- D. Bone marrow (Correct Answer)
Explanation: ***Bone marrow*** - **Bone marrow** is the most common site of metastasis in neuroblastoma, occurring in more than half of all patients and being a primary determinant of prognosis. - Metastasis to the bone marrow often leads to **anemia**, **thrombocytopenia**, and sometimes **bone pain**. *Lung* - While possible, lung metastases are relatively **uncommon** in neuroblastoma, especially when compared to bone marrow involvement. - Lung metastases tend to occur in **later stages** or with specific genetic subtypes. *Liver* - Liver metastases, though seen, are more prevalent in **infants** with **Stage 4S neuroblastoma**, where the liver can be massively enlarged [1]. - This specific stage often has a **better prognosis** than other metastatic forms [1]. *Lymph nodes* - **Regional lymph node** involvement is common at diagnosis, but distant lymph node metastasis is less frequent than bone marrow involvement. - Involvement of regional lymph nodes does contribute to staging but is not the most frequent site of **distant metastasis**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, p. 486.
Question 6: Neuroblastoma originates from –
- A. Mediastinum
- B. Adrenals
- C. Neck
- D. All of these (Correct Answer)
Explanation: ***All of these*** - **Neuroblastoma** arises from primitive **sympathetic neural crest cells** and can originate from **any location** where these cells exist during development [1]. - The tumor can develop in the **adrenal medulla**, **sympathetic ganglia** along the paravertebral sympathetic chain (abdomen, chest, neck, pelvis), making all the listed options correct sites of origin [1], [2]. - The anatomical distribution reflects the **embryological migration pattern** of neural crest cells. **Adrenal Glands (Most Common)** - The **adrenal medulla** is the most frequent primary site, accounting for approximately **40-50%** of all neuroblastomas [1]. - This is because the adrenal medulla is derived from sympathetic neural crest cells. - Abdominal neuroblastomas (adrenal and extra-adrenal) together comprise about **65%** of cases. **Mediastinum (Posterior)** - Approximately **15-20%** of neuroblastomas originate in the **posterior mediastinum** from sympathetic ganglia. - These are the **second most common site** and typically present as posterior mediastinal masses on chest imaging. - Often discovered incidentally on chest X-rays. **Neck (Cervical)** - **Cervical neuroblastoma** arises from sympathetic ganglia in the neck region. - This accounts for about **1-5%** of cases and is relatively uncommon. - May present as a palpable neck mass or Horner syndrome if the cervical sympathetic chain is involved. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of Infancy and Childhood, pp. 483-484. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Liver And Biliary System Disease, pp. 419-420.
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: A 1-year-old unimmunised child with HbsAg positivity is having following skin lesions. What is the diagnosis?
- A. Gianotti-Crosti syndrome (Correct Answer)
- B. Erythema infectiosum
- C. Pityriasis alba
- D. Pityriasis rosea
Explanation: ***Gianotti-Crosti syndrome*** - This syndrome is characterized by a **papular acrodermatitis** of childhood, typically presenting as monomorphic, erythematous papules on the face, buttocks, and extensor surfaces of the extremities. - It is strongly associated with **viral infections**, particularly **Hepatitis B virus (HBV)** and Epstein-Barr virus (EBV), which aligns with the HbsAg positivity in this unimmunized child. *Erythema infectiosum* - This condition is caused by **Parvovirus B19** and typically presents with a "slapped cheek" rash on the face, followed by a lacy, reticulated rash on the trunk and extremities. - The rash in the image does not demonstrate the characteristic lacy pattern or typical "slapped cheek" appearance. *Pityriasis alba* - This is a common, mild, and often asymptomatic skin condition characterized by **hypopigmented (lighter) patches** with fine scales, predominantly on the face and arms. - The lesions in the image are erythematous (red) and papular, not hypopigmented. *Pityriasis rosea* - This condition typically begins with a single, larger "herald patch" followed by smaller, oval-shaped, pinkish-red patches with a characteristic **"Christmas tree" pattern** on the trunk and proximal extremities. - The rash in the image does not show a herald patch or the distinct distribution and morphology of pityriasis rosea.
Question 9: What is the most common malignant neoplasm of infancy?
- A. Malignant teratoma
- B. Neuroblastoma (Correct Answer)
- C. Wilms' tumor
- D. Hepatoblastoma
Explanation: **Explanation:** **Neuroblastoma** is the most common extracranial solid tumor of childhood and the **most common malignant neoplasm of infancy** (defined as children <1 year of age). It originates from primordial neural crest cells of the sympathetic nervous system, most commonly occurring in the adrenal medulla. Its high incidence in infancy is attributed to its embryonal nature; in fact, many cases are congenital or detected during prenatal screening. **Analysis of Incorrect Options:** * **Malignant Teratoma:** While teratomas are the most common germ cell tumors in neonates (specifically Sacrococcygeal teratoma), the majority are benign at birth. They do not surpass neuroblastoma in overall malignant frequency during infancy. * **Wilms’ Tumor (Nephroblastoma):** This is the most common primary renal tumor in children, but its peak incidence occurs between **2 to 5 years** of age. It is relatively rare in the first year of life. * **Hepatoblastoma:** This is the most common liver malignancy in children, but it is significantly less common overall than neuroblastoma. **High-Yield Clinical Pearls for NEET-PG:** * **Median age of diagnosis:** 19 months (but it remains the #1 malignancy in the <1-year age group). * **Clinical Feature:** Often presents as a hard, irregular abdominal mass that **crosses the midline** (unlike Wilms’ tumor, which usually does not). * **Opsoclonus-Myoclonus Syndrome:** A classic paraneoplastic syndrome associated with neuroblastoma ("dancing eyes, dancing feet"). * **Biomarker:** Elevated urinary catecholamines (VMA and HVA) are found in 90% of cases. * **Prognosis:** Age is a major prognostic factor; infants (<18 months) generally have a much better prognosis, including the possibility of spontaneous regression (Stage 4S).
Question 10: Wilms' tumor is associated with which of the following conditions?
- A. Hemihypertrophy
- B. Aniridia
- C. Hypertension
- D. Bilateral polycystic kidney disease (Correct Answer)
Explanation: **Explanation:** Wilms' tumor (Nephroblastoma) is the most common primary renal malignancy in children. While it is famously associated with several congenital syndromes, the question asks for a condition **not** typically associated with it, or identifies a distractor in a "except" style format. **Note on the Answer Key:** In standard medical literature, **Bilateral Polycystic Kidney Disease (BPKD)** is **not** a known predisposing factor or associated feature of Wilms' tumor. If the question asks which is associated, Options A, B, and C are all classic features. If the provided key marks D as correct, it implies D is the "odd one out" or the condition **not** associated. * **Hemihypertrophy (Option A):** This is a classic association, often seen in **Beckwith-Wiedemann Syndrome** (macroglossia, omphalocele, hemihypertrophy, and Wilms' tumor). * **Aniridia (Option B):** Absence of the iris is a hallmark of **WAGR Syndrome** (Wilms' tumor, Aniridia, Genitourinary anomalies, and intellectual disability/Range of developmental delays). * **Hypertension (Option C):** Approximately 25% of patients with Wilms' tumor present with hypertension due to increased renin production or compression of the renal artery by the tumor mass. * **Bilateral Polycystic Kidney Disease (Option D):** This is a genetic ciliopathy and is not part of the oncogenic pathways (WT1/WT2 mutations) that lead to Wilms' tumor. **High-Yield Clinical Pearls for NEET-PG:** 1. **WAGR Syndrome:** Associated with deletions on chromosome **11p13** (WT1 gene). 2. **Beckwith-Wiedemann Syndrome:** Associated with mutations on chromosome **11p15.5** (WT2 gene). 3. **Denys-Drash Syndrome:** Characterized by the triad of Wilms' tumor, pseudohermaphroditism, and early-onset nephropathy (glomerulosclerosis). 4. **Clinical Presentation:** Most common presentation is an **asymptomatic, firm, smooth abdominal mass** that does not cross the midline (unlike Neuroblastoma).
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