Oncology Practice Questions

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.

Q: Which one of the following statements is true regarding brain tumors in childhood?

Most tumours are below the tentorium. ***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.

Q: 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?

Wilms' tumor. ***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.

Q: Opsomyoclonus is encountered as a complication of:

Neuroblastoma. ***Neuroblastoma*** - Opsomyoclonus syndrome (OMS) is a **rare paraneoplastic syndrome** strongly associated with **neuroblastoma** in children. - Neuroblastoma is a common **extracranial solid tumor** of childhood arising from primitive sympathetic neuroblasts. *Meningioma* - Meningiomas are typically **benign tumors** of the meninges in adults and are not associated with paraneoplastic syndromes like opsomyoclonus. - Their symptoms usually arise from **mass effect** on adjacent brain tissue or cranial nerves. *Neurofibromatosis* - Neurofibromatosis is a **genetic disorder** causing tumors to grow on nerve tissue, primarily **neurofibromas**. - It is not associated with opsomyoclonus syndrome, which is a **paraneoplastic** phenomenon. *Wilms tumor* - Wilms tumor is a common **kidney cancer** in children. - While it can be associated with certain genetic syndromes, it is **not known** to cause opsomyoclonus syndrome.

Q: Which condition is characterized by the clinical features of periorbital ecchymosis (raccoon eyes) due to metastatic spread to the orbit?

Neuroblastoma. ***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

Question 1

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 2

Treatment of choice for stage 4S neuroblastoma is

Accepted Answer

Observation

Answer

Surgery

Answer

Chemotherapy

Answer

Radiotherapy

Question 3

Which one of the following statements is true regarding brain tumors in childhood?

Accepted Answer

Most tumours are below the tentorium

Answer

Papilloedema is infrequent

Answer

Is a rare form of malignancy

Answer

Hemiparesis is the most common presenting feature

Question 4

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?

Accepted Answer

Wilms' tumor

Answer

Hodgkin's lymphoma

Answer

Embryonal rhabdomyosarcoma

Answer

Stage 4 neuroblastoma

Question 5

Opsomyoclonus is encountered as a complication of:

Accepted Answer

Neuroblastoma

Answer

Meningioma

Answer

Neurofibromatosis

Answer

Wilms tumor

Question 6

Which condition is characterized by the clinical features of periorbital ecchymosis (raccoon eyes) due to metastatic spread to the orbit?

Accepted Answer

Neuroblastoma

Answer

Pheochromocytoma

Answer

Adrenal incidentaloma

Answer

Medullary thyroid cancer

Oncology — MCQs

Oncology — MCQs

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