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

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Question 1 of 5

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: The correct answer is E because it is the one that does not accurately describe renal cell carcinoma (RCC) in children. In pediatric cases, RCC is not associated with von Hippel-Lindau disease, which is more commonly seen in adults. Option A is correct as patients with RCC in children can present with symptoms like hematuria, flank pain, and a palpable mass. Option B is also accurate since RCC can be asymptomatic and incidentally detected on imaging studies. Option C is true as RCC in children has a propensity to metastasize to the lungs, bone, liver, and brain. Educationally, understanding the presentation and characteristics of RCC in children is crucial for pediatric nurses to provide timely and appropriate care. Recognizing the differences between RCC in children and adults, such as the absence of von Hippel-Lindau disease association, helps in accurate diagnosis and management. This knowledge ensures comprehensive care and support for pediatric patients with RCC.

Question 2 of 5

In Langerhans cell histiocytosis (LCH), which organ is LEAST likely affected?

Correct Answer: D

Rationale: In Langerhans cell histiocytosis (LCH), the organ that is LEAST likely affected is the middle nodes, making option D the correct answer. LCH is a rare disease characterized by the overproduction of histiocytes, which are a type of white blood cell. LCH commonly affects the skin, bones, and other organs but rarely involves the lymph nodes, particularly the middle nodes. Option A, the skin, is often affected in LCH, presenting as skin rashes or lesions. Option B, the ear, can also be affected in LCH, leading to symptoms like hearing loss or ear discharge. Option C, bones, are commonly involved in LCH, causing bone pain or fractures due to the infiltration of histiocytes. Understanding the typical organ involvement in LCH is crucial for pediatric nurses as they care for children with this condition. Recognizing the less common sites of involvement, like the lymph nodes, helps in early detection and appropriate management. By knowing the disease manifestations, nurses can provide holistic care and support to pediatric patients and their families affected by LCH.

Question 3 of 5

Of the following, the genetic syndrome MOST likely associated with increased risk of optic glioma is

Correct Answer: B

Rationale: The correct answer is B) neurofibromatosis. Neurofibromatosis, specifically type 1 (NF1), is a genetic syndrome associated with an increased risk of optic glioma, a type of tumor that occurs along the optic nerve. This condition commonly presents in childhood and can lead to vision problems if left untreated. Option A) Down syndrome is a genetic disorder caused by the presence of an extra chromosome 21. While individuals with Down syndrome may have an increased risk of certain health conditions, optic glioma is not typically associated with this syndrome. Option C) Monosomy refers to a genetic condition where a cell has only one copy of a particular chromosome instead of the usual two. This is not specifically linked to an increased risk of optic glioma. Option D) Bloom's syndrome is a rare genetic disorder characterized by short stature, sun sensitivity, and an increased risk of cancer. While some individuals with Bloom's syndrome may develop tumors, optic glioma is not a common feature of this condition. Educational Context: Pediatric nursing practice requires a thorough understanding of various genetic syndromes and their associated health risks. Recognizing the signs and symptoms of conditions like neurofibromatosis is crucial for early detection and intervention to prevent potential complications such as optic glioma. By understanding the distinctive features of different genetic syndromes, nurses can provide comprehensive care and support to pediatric patients and their families.

Question 4 of 5

Which chromosomal abnormality is often characteristic of infantile ALL?

Correct Answer: A

Rationale: In pediatric nursing, understanding chromosomal abnormalities associated with different conditions is crucial for accurate diagnosis and treatment. In the case of infantile Acute Lymphoblastic Leukemia (ALL), the chromosomal abnormality t(4;11) is often characteristic. This translocation results in the fusion of the MLL gene on chromosome 11 with various partner genes on chromosome 4, leading to the overexpression of MLL fusion proteins that play a role in leukemogenesis in infants. Option A, t(4;11), is the correct answer because it is specifically linked to infantile ALL. This translocation is a common genetic feature in this age group and is associated with a poorer prognosis compared to other subtypes of ALL. Options B, C, and D are incorrect for infantile ALL. Option B, t(12;21), is typically associated with a better prognosis 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 a subtype of pre-B cell ALL. Educationally, understanding the genetic basis of diseases like ALL enhances nurses' ability to provide comprehensive care, including recognizing potential complications and collaborating with the healthcare team to develop individualized treatment plans. This knowledge also informs patient and family education, helping them better understand the condition and its implications. Consequently, being well-versed in chromosomal abnormalities in pediatric oncology is essential for pediatric nurses to deliver high-quality care to their young patients.

Question 5 of 5

Tumor lysis syndrome is a common complication during treatment of pediatric malignancies. Which malignancy is more likely to manifest such complication?

Correct Answer: D

Rationale: Tumor lysis syndrome (TLS) is a potentially life-threatening complication that can occur during the treatment of pediatric malignancies. It is characterized by metabolic disturbances such as hyperuricemia, hyperkalemia, hyperphosphatemia, and hypocalcemia resulting from the rapid breakdown of tumor cells. In this scenario, Burkitt lymphoma is more likely to manifest TLS compared to the other malignancies listed. Burkitt lymphoma is a highly proliferative B-cell non-Hodgkin lymphoma that is very sensitive to chemotherapy. The rapid cell turnover in Burkitt lymphoma leads to a higher risk of TLS due to the release of intracellular contents into the bloodstream during treatment initiation. Acute myelogenous leukemia (AML) is associated with a high tumor burden but does not typically cause the same degree of rapid cell lysis seen in Burkitt lymphoma. Nephroblastoma (Wilms tumor) and neuroblastoma are pediatric solid tumors that, although they can be large, are less likely to lead to TLS compared to highly proliferative hematologic malignancies like Burkitt lymphoma. Educationally, understanding the risk factors and manifestations of TLS in pediatric oncology is crucial for nurses caring for children with cancer. Recognizing the signs and symptoms of TLS, monitoring laboratory values closely, and implementing preventive measures are essential in the management of pediatric patients at risk for this serious complication.

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