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 is the commonest childhood cancer –
The ideal timing of radiotherapy for Wilms' tumor after surgery is -
What is the most common site of rhabdomyosarcoma in children?
2-year-old child is seen for a well-child visit in a pediatric clinic. Abdominal examination demonstrates a palpable, non-tender mass on the left side of the abdomen. The mother had no idea where the mass was present and the pediatrician did not note the presence of the mass at the child's 18-month well-child visit. Physical examination is otherwise unremarkable. Abnormalities of which of the following chromosomes have been linked to this disease?
TRUE statement regarding childhood malignancy is-
The most common intracranial tumor in children is –
A baby presents with recurrent ear infections with discharge, seborrheic dermatitis, hepatosplenomegaly, and cystic skull lesions. What is the most likely diagnosis?
A renal mass seen on prenatal check-up is most probably:
Most common malignant tumour in childhood:
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.
Explanation: ***ALL*** - **Acute Lymphoblastic Leukemia (ALL)** is the most common cancer in children, accounting for about 1 in 3 childhood cancers. - It primarily affects **lymphoid progenitor cells** and is characterized by rapid progression. *AML* - **Acute Myeloid Leukemia (AML)** is more common in adults and has a lower incidence in children compared to ALL. - It involves the **myeloid lineage** of white blood cells. *CML* - **Chronic Myeloid Leukemia (CML)** is rare in childhood, primarily affecting adults. - It is often associated with the **Philadelphia chromosome (BCR-ABL fusion gene)**. *CLL* - **Chronic Lymphocytic Leukemia (CLL)** is extremely rare in children and predominantly affects older adults. - It is characterized by the accumulation of **mature B lymphocytes**.
Explanation: ***Within 10 days*** - Postoperative radiotherapy for Wilms' tumor should ideally be initiated within **10 days** of surgery to optimize outcomes and minimize the risk of recurrence. - This **early initiation** helps to control potential microscopic residual disease and is associated with improved outcomes. - **Children's Oncology Group (COG)** protocols recommend starting radiotherapy within **10-14 days**, with 10 days being the preferred timeline. *Any time after surgery* - Delaying radiotherapy beyond the recommended 10-14 day window can significantly increase the risk of **disease progression** and **local recurrence**. - The efficacy of radiotherapy is highest when administered promptly after surgical resection. *Within 2 months* - A delay of two months is considered **suboptimal** and significantly exceeds the recommended timeframe. - This extended delay could lead to progression of any residual tumor cells and compromise treatment outcomes. *Within 2 weeks* - While 2 weeks (14 days) falls within the acceptable **10-14 day window** recommended by most protocols, **10 days is preferred** as the most optimal timing. - The distinction is that initiating within 10 days is considered **ideal**, whereas the full 14-day period represents the outer limit of the acceptable timeframe for standard cases.
Explanation: ***Head and neck region*** - The **head and neck region** is the most common site for rhabdomyosarcoma in children, accounting for approximately **35-40% of cases**. - Within the head and neck, common sub-sites include the **orbit**, **parameningeal sites** (nasopharynx, paranasal sinuses, middle ear), and other **non-parameningeal sites**. *Extremities* - Rhabdomyosarcoma can occur in the **extremities**, but it is less common than in the head and neck, typically accounting for about **20% of cases**. - When present in the extremities, it often involves the **proximal portions** of the limbs. *Genitourinary area* - The **genitourinary (GU) tract** is a significant site for rhabdomyosarcoma, particularly the **bladder** and **prostate**, accounting for approximately **20% of cases**. - While common, it is not the *most* common overall site compared to the head and neck. *Trunk* - Rhabdomyosarcoma in the **trunk** (including the chest wall, abdomen, and retroperitoneum) is less frequent, accounting for about **10-15% of cases**. - These tumors can arise from various muscle groups within the trunk, but are collectively less common than head and neck involvement.
Explanation: ***Correct: Option 11*** - The presentation of a **palpable, non-tender abdominal mass** in a 2-year-old child, especially one that has grown since the last visit, is highly suggestive of **Wilms tumor (nephroblastoma)**. - **Wilms tumor** is associated with genetic abnormalities, particularly on **chromosome 11**, involving genes such as **WT1** (11p13) and **WT2** (11p15.5). - These genetic mutations are found in approximately **10-15% of Wilms tumors**, and chromosome 11 abnormalities are the most well-established genetic link to this disease. *Incorrect: Option 15* - While other chromosomal abnormalities can be associated with childhood cancers, **chromosome 15** is not typically implicated in the primary genetic etiology of Wilms tumor. - Specific genetic syndromes linked to chromosome 15 (e.g., Prader-Willi, Angelman syndrome) have distinct phenotypic presentations unrelated to a solitary renal mass. *Incorrect: Option 5* - Abnormalities on **chromosome 5** are not primarily linked to the development of Wilms tumor. - **Cri-du-chat syndrome** (deletion on chromosome 5p) is a known genetic condition associated with chromosome 5 abnormalities, but it presents with distinct craniofacial features and developmental delays, not an abdominal mass. *Incorrect: Option 3* - **Chromosome 3** abnormalities are more commonly associated with certain adult cancers, such as renal cell carcinoma, or other developmental anomalies. - It does not have a direct, well-established link to the pathogenesis of **Wilms tumor** in children.
Explanation: ***Neuroblastoma typically occurs before 5 years of age*** - **Neuroblastoma** is predominantly a malignancy of **early childhood**, with approximately **90% of cases** diagnosed **before the age of 5 years**. - The **median age at diagnosis** is around **17-22 months**, with about **40% occurring in the first year of life**. - It originates from **neuroblasts** in the adrenal medulla or sympathetic ganglia, which are rapidly developing tissues in infants and young children. *AML is the most common malignancy* - **Acute Lymphoblastic Leukemia (ALL)**, not Acute Myeloid Leukemia (AML), is the **most common childhood malignancy**, accounting for about **25-30% of all childhood cancers** and **75-80% of childhood leukemias**. - While AML does occur in children, its incidence is **significantly lower than ALL**. *Wilm's tumour is the commonest* - **Wilms' tumor** (nephroblastoma) is the **most common primary malignant renal tumor** in children, but it is **not the most common childhood malignancy overall**. - **Leukemias** (specifically ALL) are considerably more common than Wilms' tumor. *None of the options* - This option is incorrect because the statement regarding **neuroblastoma** is accurate, making it a true statement. - Therefore, at least one of the options provided is correct.
Explanation: ***Glioma*** - **Gliomas** are the most common type of intracranial tumor in children, accounting for approximately **50-60%** of all pediatric brain tumors. This broad category includes tumors like astrocytomas (e.g., **pilocytic astrocytoma**), ependymomas, and brainstem gliomas. - The most common subtype of glioma in children is the **pilocytic astrocytoma**, which often arises in the cerebellum or optic pathway. - Other common gliomas include **ependymomas** (arising in the ventricular system) and **diffuse brainstem gliomas**. *Meningioma* - **Meningiomas** are tumors arising from the meninges, the membranes surrounding the brain and spinal cord, and are **rare** in children. - These tumors are typically seen in **middle-aged to older adults**, and their occurrence in pediatric populations is uncommon, accounting for less than **2%** of pediatric brain tumors. *Lymphangioma* - A **lymphangioma** is a benign malformation of the lymphatic system, most commonly found in the **head and neck region** (e.g., cystic hygroma), not within the brain parenchyma. - It is a tumor of lymphatic vessels and is **not an intracranial brain tumor** in the typical sense. *Medulloblastoma* - **Medulloblastoma** is the most common **malignant** brain tumor in children and the most common **posterior fossa tumor** in the pediatric population. - However, when considering **all intracranial tumors** (both benign and malignant), **gliomas as a category** are more common overall than medulloblastomas alone. - Medulloblastomas arise from the cerebellum and account for approximately **15-20%** of pediatric brain tumors.
Explanation: ***Langerhans cell histiocytosis*** - This classic presentation involves a constellation of symptoms including **seborrheic dermatitis-like rash**, recurrent ear infections with discharge (due to infiltration of the mastoid bone), hepatosplenomegaly, and **cystic skull lesions**, all highly suggestive of Langerhans cell histiocytosis (LCH). - LCH is a rare disorder characterized by the proliferation of abnormal Langerhans cells, which can affect various organs and systems. *Hemophagocytic lymphohistiocytosis* - While patients with Hemophagocytic Lymphohistiocytosis (HLH) can present with **hepatosplenomegaly** and recurrent infections, they typically have high fevers, cytopenias, and poor clinical condition. - HLH does not typically cause **seborrheic dermatitis-like rashes** or **cystic bone lesions**. *Multiple myeloma* - Multiple myeloma is a **plasma cell malignancy** that predominantly affects older adults and is characterized by bone pain, anemia, renal failure, and hypercalcemia. - It does not present with recurrent ear infections, seborrheic dermatitis, or hepatosplenomegaly in this manner, and is exceedingly rare in infants. *Acute lymphoblastic leukemia (ALL)* - ALL is the most common childhood cancer and can cause **hepatosplenomegaly**, recurrent infections (due to neutropenia), and sometimes bone pain. - However, **seborrheic dermatitis-like rash** and **cystic skull lesions** are not typical features of ALL.
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.
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.
Leukemias
Practice Questions
Lymphomas
Practice Questions
CNS Tumors
Practice Questions
Neuroblastoma
Practice Questions
Wilms Tumor
Practice Questions
Rhabdomyosarcoma
Practice Questions
Bone Tumors
Practice Questions
Retinoblastoma
Practice Questions
Histiocytosis Syndromes
Practice Questions
Principles of Pediatric Chemotherapy
Practice Questions
Hematopoietic Stem Cell Transplantation
Practice Questions
Late Effects of Cancer Treatment
Practice Questions
Get full access to all questions, explanations, and performance tracking.
Start For Free