Renal cell carcinoma (RCC) is rare in children, accounting for <5% of all renal tumors. All the following are true regarding RCC in children EXCEPT

Correct Answer: E

Rationale: In this question, the correct answer is E, which is not listed as an option. Renal cell carcinoma (RCC) in children is indeed a rare condition, accounting for less than 5% of all renal tumors. The other options listed in the question are all true statements regarding RCC in children. Option A is correct because patients with RCC may present with symptoms such as frank hematuria, flank pain, and/or a palpable mass. Option B is also correct as RCC can be asymptomatic and may be incidentally detected. Option C is true as RCC in children has the potential to metastasize to various organs including the lungs, bone, liver, and brain. Option D is also accurate as RCC can be associated with von Hippel-Lindau disease, a genetic disorder that predisposes individuals to various tumors, including RCC. Educationally, understanding the presentation, diagnosis, and associations of RCC in children is crucial for healthcare providers working in pediatric oncology or nephrology. Recognizing the signs and symptoms, risk factors, and potential metastatic sites can aid in early detection, appropriate management, and improved outcomes for pediatric patients with RCC.

Correct Answer: D

Rationale: In Langerhans cell histiocytosis (LCH), the least likely affected organ is the middle nodes, making option D the correct answer. LCH mainly affects tissues such as the skin, bone, and sometimes the ears. The skin is commonly affected in LCH, presenting as skin lesions or rashes. Bone involvement is also common, leading to bone pain, fractures, or swelling. Ears can be affected, causing symptoms like hearing loss or ear discharge. Educationally, understanding the typical organ involvement in LCH is crucial for pediatric nurses to recognize and manage the condition effectively. By knowing that middle nodes are least likely affected, nurses can focus on assessing and monitoring more commonly involved organs like the skin, bones, and ears in pediatric patients with LCH. This knowledge helps in providing comprehensive care and early intervention for these young patients.

Correct Answer: B

Rationale: In this question, the correct answer is B) neurofibromatosis. Neurofibromatosis is a genetic disorder that can lead to the development of optic gliomas, which are tumors that form on the optic nerve. This association is well-documented in the medical literature, making neurofibromatosis the most likely genetic syndrome linked to an increased risk of optic glioma. Option A) Down syndrome is a chromosomal disorder caused by the presence of an extra chromosome 21, but it is not typically associated with an increased risk of optic glioma. Option C) monosomy refers to a genetic condition where a person is missing a chromosome in their cells, but this is not specifically linked to optic glioma. Option D) Bloom's syndrome is a rare genetic disorder characterized by short stature and increased cancer risk but is not typically associated with optic glioma. In an educational context, understanding the association between genetic syndromes and specific health conditions like optic glioma is crucial for healthcare professionals, especially pediatric nurses. This knowledge helps in early identification, appropriate management, and providing holistic care to pediatric patients with neurofibromatosis and associated conditions like optic glioma. It highlights the importance of genetic assessments and multidisciplinary approaches in pediatric nursing practice.

Correct Answer: A

Rationale: The correct answer is A) t(4;11) for infantile Acute Lymphoblastic Leukemia (ALL). This translocation results in the fusion of the MLL gene on chromosome 11 with various partner genes. This fusion gene plays a key role in leukemogenesis in infantile ALL. Option B) t(12;21) is associated with a better prognosis in pediatric ALL as it results in the formation of the ETV6-RUNX1 fusion gene, which is more common in childhood ALL. Option C) t(9;22) is characteristic of Chronic Myeloid Leukemia (CML) and not infantile ALL. Option D) t(1;19) is associated with pre-B cell ALL, not infantile ALL. Educationally, understanding chromosomal abnormalities in pediatric malignancies is crucial for nurses caring for pediatric oncology patients. Recognizing specific translocations can guide treatment decisions and help predict outcomes, highlighting the importance of this knowledge in pediatric nursing practice.

Correct Answer: D

Rationale: Tumor lysis syndrome (TLS) is a potentially life-threatening complication that can occur during the treatment of malignancies due to the rapid breakdown of cancer cells leading to metabolic imbalances. In pediatric oncology, Burkitt lymphoma is more likely to manifest TLS compared to other malignancies listed. Burkitt lymphoma is a highly proliferative and fast-growing cancer, characterized by rapid cell turnover. When treatment is initiated, the massive destruction of tumor cells can overwhelm the body's ability to clear the metabolic byproducts, leading to TLS. This is why option D (Burkitt lymphoma) is the correct answer. The other options can be eliminated as they are less commonly associated with TLS in pediatric patients. Acute myelogenous leukemia (AML) and nephroblastoma are not typically linked to TLS due to differences in their pathophysiology and cell turnover rates. Neuroblastoma, although it can cause tumor lysis, is less likely to result in TLS compared to Burkitt lymphoma due to differences in tumor burden and cell turnover dynamics. Understanding which malignancies are more prone to TLS is crucial for pediatric nurses caring for oncology patients. Recognizing the predisposing factors can help in early identification, prompt management, and prevention of TLS-related complications, ultimately improving patient outcomes.