Constellation of aniridia and hemihypertrophy is strongly associated with increased risk of which of the following tumors?

Correct Answer: C

Rationale: The correct answer is C) Wilms tumor. Aniridia-hemihypertrophy syndrome, also known as WAGR syndrome (Wilms tumor, aniridia, genitourinary abnormalities, and mental retardation), is a rare genetic disorder characterized by the presence of Wilms tumor, a type of kidney cancer that primarily affects children. The association between aniridia and hemihypertrophy increases the risk of developing Wilms tumor significantly. Option A) rhabdomyosarcoma is incorrect because it is not strongly associated with aniridia-hemihypertrophy syndrome. Rhabdomyosarcoma is a soft tissue tumor that typically arises in muscles, not commonly linked to this syndrome. Option B) hepatoblastoma is also incorrect as it is a liver tumor that is not commonly associated with aniridia-hemihypertrophy syndrome. Option D) medulloblastoma is a type of brain cancer that is not strongly linked to this specific genetic syndrome. Educationally, understanding the association between certain genetic syndromes and specific tumors is crucial in clinical practice, especially in pediatric oncology. Recognizing these patterns can aid in early detection, appropriate management, and improved outcomes for patients. This knowledge underscores the importance of a comprehensive understanding of pediatric oncology and genetics in nursing practice, particularly for those specializing in critical care for children.

Correct Answer: C

Rationale: In this question, the correct answer is C) anaplastic histology. Anaplastic histology in Wilms tumor is associated with a higher risk of relapse and poorer outcomes compared to other histological subtypes. This is because anaplastic Wilms tumor is more aggressive and less responsive to treatment, leading to a higher likelihood of relapse. Option A) low stage (I/II) at diagnosis is incorrect because even children with low-stage disease can experience relapse, although the risk is lower compared to higher-stage disease. Option B) no prior radiotherapy is incorrect because the absence of prior radiotherapy does not preclude the possibility of relapse in Wilms tumor patients. Option D) more than 12 months from nephrectomy is incorrect because relapse can occur at any time after nephrectomy, not just within a specific timeframe. Educationally, understanding the factors associated with relapse in Wilms tumor is crucial for pediatric oncology nurses and healthcare providers caring for these patients. Recognizing the risk factors for relapse can help guide surveillance strategies and treatment decisions to improve outcomes for children with Wilms tumor.

Correct Answer: B

Rationale: In pediatric CCRN practice, understanding risk factors for melanoma in children is crucial for early detection and management. The correct answer is B) dark-skinned child. Melanoma is predominantly seen in fair-skinned individuals with a history of sun exposure. Dark skin has more melanin, providing natural protection against UV radiation, hence reducing the risk of melanoma. A) A positive family history of melanoma is a well-established risk factor due to genetic predisposition. Individuals with a family history are at a higher risk of developing melanoma themselves. C) A hairy nevus, also known as a congenital mole, is a risk factor for melanoma due to the presence of a higher number of melanocytes. These moles have a potential for malignant transformation. D) Dysplastic nevus, or atypical mole, is considered a risk factor for melanoma as these moles exhibit irregular features that may progress to melanoma. Educationally, it is important to teach healthcare providers to recognize these risk factors to conduct thorough assessments and provide appropriate education to patients and families about sun protection and regular skin checks. Early identification and intervention can significantly impact the prognosis of pediatric melanoma cases.

Correct Answer: C

Rationale: Rationale: The correct answer is C) children with Down syndrome have a slightly inferior outcome ratio of ALL/AML in general. This statement is incorrect because research shows that children with Down syndrome have a better outcome when they develop leukemia compared to children without Down syndrome. This is due to the unique genetic and immunological characteristics of individuals with Down syndrome that make them more responsive to certain treatments. Option A is incorrect because acute leukemia does indeed occur more frequently in children with Down syndrome than in the general population due to the genetic predisposition associated with Down syndrome. Option B is incorrect as AML (Acute Myeloid Leukemia) is more common in children with Down syndrome compared to ALL (Acute Lymphoblastic Leukemia) as Down syndrome predisposes individuals to myeloid leukemia. Option D is incorrect as children with Down syndrome who develop AML actually demonstrate remarkable sensitivity to antimetabolites, making them more responsive to this type of chemotherapy. In an educational context, understanding the specific risks and outcomes of leukemia in children with Down syndrome is crucial for medical students to provide appropriate care and treatment to this unique patient population. By understanding these nuances, future healthcare providers can tailor their approach to effectively manage leukemia in children with Down syndrome.

Correct Answer: B

Rationale: In the context of pediatric CCRN practice questions, understanding neuroblastoma and its associated paraneoplastic syndromes is crucial for nurses working with pediatric patients. In this question, the correct answer is B) cerebellar ataxia and increased body coordination. This is because cerebellar ataxia is a common paraneoplastic syndrome associated with neuroblastoma, leading to issues with coordination and movement. Option A) uncontrollable jerking movements could be associated with opsoclonus-myoclonus syndrome, a paraneoplastic syndrome seen in neuroblastoma. Option C) unilateral ptosis, myosis, and anhidrosis are symptoms of Horner syndrome, which can be seen in neuroblastoma. Option D) profound secretory diarrhea is a characteristic of VIPoma syndrome, another paraneoplastic syndrome associated with neuroblastoma. By understanding the specific paraneoplastic syndromes linked to neuroblastoma, nurses can monitor and manage symptoms effectively, ensuring better outcomes for pediatric patients. This knowledge also highlights the importance of holistic care and multidisciplinary approaches in pediatric oncology nursing.