Which one of the following statements is true regarding brain tumors in childhood?
Q222
A healthy appearing 8-year-old boy is evaluated for an abdominal mass, felt by his mother during a bath. What is the most likely diagnosis?
Q223
Which condition is characterized by the clinical features of periorbital ecchymosis (raccoon eyes) due to metastatic spread to the orbit?
Oncology Indian Medical PG Practice Questions and MCQs
Question 221: Which one of the following statements is true regarding brain tumors in childhood?
A. Most tumours are below the tentorium (Correct Answer)
B. Papilloedema is infrequent
C. Is a rare form of malignancy
D. Hemiparesis is the most common presenting feature
Explanation: ***Most tumours are below the tentorium***
- In children, approximately 60% of primary brain tumors are **infratentorial**, located in the cerebellum, brainstem, and fourth ventricle.
- This predominance of posterior fossa tumors in children contrasts with adults, where **supratentorial tumors** are more common.
*Is a rare form of malignancy*
- Brain tumors are the **most common solid tumors** in children and the second most common childhood malignancy overall, after leukemia.
- While individually rare, as a group, they represent a significant proportion of childhood cancers.
*Hemiparesis is the most common presenting feature*
- **Hemiparesis** is NOT the most common presentation in pediatric brain tumors.
- Most common presentations include **headaches, vomiting, ataxia, and cranial nerve palsies**, reflecting the predominance of **infratentorial tumors**.
- Hemiparesis occurs more commonly with supratentorial lesions, which represent the minority of childhood brain tumors.
*Papilloedema is infrequent*
- **Papilledema**, or swelling of the optic disc due to increased intracranial pressure, is a **frequent finding** in children with brain tumors.
- It often develops as a result of tumor mass effect, hydrocephalus, or obstruction of CSF flow.
Question 222: A healthy appearing 8-year-old boy is evaluated for an abdominal mass, felt by his mother during a bath. What is the most likely diagnosis?
A. Wilms' tumor (Correct Answer)
B. Hodgkin's lymphoma
C. Embryonal rhabdomyosarcoma
D. Stage 4 neuroblastoma
Explanation: ***Wilms' tumor***
- **Wilms' tumor**, or **nephroblastoma**, is the most common primary renal malignancy in children, typically presenting as an asymptomatic abdominal mass.
- The peak incidence is between ages 2 and 5, but it can occur up to 8 years old; a **palpable abdominal mass** is the classic presentation.
*Hodgkin's lymphoma*
- Hodgkin's lymphoma usually presents with **lymphadenopathy** (enlarged lymph nodes), often in the neck or supraclavicular region, along with systemic symptoms like fever and weight loss.
- While it can occur in childhood, an isolated abdominal mass without other typical signs makes it less likely.
*Embryonal rhabdomyosarcoma*
- **Rhabdomyosarcoma** is a soft tissue sarcoma and can occur in various locations, but an isolated abdominal mass in an 8-year-old is less commonly embryonal rhabdomyosarcoma without other localizing symptoms.
- It usually presents with symptoms related to the primary site, such as a painful mass, swelling, or organ dysfunction if it invades structures.
*Stage 4 neuroblastoma*
- **Neuroblastoma** typically presents in infants and young children, often with an abdominal mass but also with signs of metastasis (e.g., bone pain, periorbital ecchymosis, opsoclonus-myoclonus syndrome).
- While it can manifest as an abdominal mass, the "healthy appearing" aspect makes stage 4 less likely, as advanced neuroblastoma often causes systemic symptoms.
Question 223: Which condition is characterized by the clinical features of periorbital ecchymosis (raccoon eyes) due to metastatic spread to the orbit?
A. Pheochromocytoma
B. Adrenal incidentaloma
C. Neuroblastoma (Correct Answer)
D. Medullary thyroid cancer
Explanation: ***Neuroblastoma***
- **Periorbital ecchymosis** (raccoon eyes) is a **pathognomonic sign** of **neuroblastoma with orbital metastases**
- Most common **extracranial solid tumor in children** (median age 18 months)
- Originates from **neural crest cells** in the adrenal medulla or sympathetic ganglia
- Orbital metastases occur via **hematogenous spread** to periorbital bones, causing hemorrhage and the characteristic "raccoon eyes" appearance
- Other metastatic features: **skull bone lesions**, hepatomegaly, and bone marrow infiltration
- Elevated **catecholamines** (VMA, HVA in urine) and **N-myc amplification** are key diagnostic markers
*Pheochromocytoma*
- Arises from **chromaffin cells** of the adrenal medulla
- Presents with **episodic hypertension**, headache, palpitations, and diaphoresis due to **catecholamine excess**
- Rare in children; when present, often associated with **genetic syndromes** (VHL, MEN 2, NF1)
- Does **not metastasize to orbit** or cause periorbital ecchymosis
- "Rule of 10s": 10% bilateral, 10% extra-adrenal, 10% malignant
*Adrenal incidentaloma*
- **Asymptomatic adrenal mass** discovered incidentally on imaging (CT/MRI) done for unrelated reasons
- May be non-functional or hormonally active (requires biochemical workup)
- Does **not present with clinical symptoms** like periorbital ecchymosis
- Management depends on size, imaging characteristics, and hormonal activity
*Medullary thyroid cancer*
- Arises from **parafollicular C cells** of the thyroid, secreting **calcitonin**
- Associated with **MEN 2A/2B** syndromes (along with pheochromocytoma)
- Typically presents with **thyroid nodule**, cervical lymphadenopathy
- Metastasizes to **lymph nodes, lungs, liver, and bones** but **not characteristically to orbit**
- Does not cause periorbital ecchymosis
