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 (absence of the iris) and hemihypertrophy (asymmetrical overgrowth of one side of the body) are features of WAGR syndrome, which includes Wilms tumor as a significant risk. Wilms tumor is a type of kidney cancer that primarily affects children. Understanding this association is crucial for pediatric critical care nurses as early detection and intervention are vital for improving outcomes in children with Wilms tumor. Option A) rhabdomyosarcoma is a soft tissue tumor that is not typically associated with aniridia and hemihypertrophy. Option B) hepatoblastoma is a liver cancer that is not linked to the specific features seen in WAGR syndrome. Option D) medulloblastoma is a type of brain tumor and is not commonly associated with aniridia and hemihypertrophy. Educationally, knowing these associations helps nurses in identifying potential risks in pediatric patients presenting with these physical characteristics. It underscores the importance of a comprehensive assessment in pediatric patients to ensure timely diagnosis and appropriate management of any associated conditions.

Correct Answer: C

Rationale: In this question, the correct answer is C) anaplastic histology. Explanation: Wilms tumor relapse is more common in cases with unfavorable histology, such as anaplastic histology. Anaplastic Wilms tumor is associated with a higher risk of relapse and poorer outcomes compared to other histological subtypes. Therefore, this option is incorrect in the context of relapse in Wilms tumor. A) Low stage (I/II) at diagnosis is incorrect because relapse can occur regardless of the initial stage of the tumor. B) No prior radiotherapy is incorrect because relapse can still occur even if radiotherapy was not part of the initial treatment plan. D) More than 12 months from nephrectomy is incorrect because relapse can occur at any time post-surgery, not exclusively within a specific timeframe. Educational Context: Understanding the risk factors and outcomes associated with Wilms tumor relapse is crucial for healthcare providers caring for pediatric oncology patients. Recognizing the factors that may contribute to relapse can aid in monitoring and managing these patients effectively. Anaplastic histology is a significant risk factor for relapse in Wilms tumor, emphasizing the importance of histological classification in treatment planning and prognostication.

Correct Answer: B

Rationale: In the context of pediatric melanoma risk factors, the correct answer is option B) dark-skinned child. Melanoma is more commonly associated with fair-skinned individuals due to their reduced ability to produce protective melanin in response to UV radiation exposure. Dark-skinned individuals have more melanin, which provides some level of protection against melanoma development. Option A) positive family history of melanoma is a well-known risk factor as genetic predisposition can play a role in the development of melanoma. Option C) hairy nevus and option D) dysplastic nevus are also risk factors as these types of moles can potentially transform into melanoma. Educationally, understanding pediatric melanoma risk factors is crucial for healthcare providers working with children. By recognizing these risk factors, healthcare professionals can educate families on preventive measures, conduct regular screenings, and facilitate early detection and treatment if necessary. Emphasizing the significance of risk factors like family history and specific types of moles can help in identifying children at higher risk for melanoma and taking appropriate actions to mitigate that risk.

Correct Answer: C

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 children with Down syndrome actually have a better prognosis when they develop leukemia, with a higher survival rate compared to children without Down syndrome. Option A is incorrect because acute leukemia does occur more frequently in children with Down syndrome due to genetic factors. Option B is incorrect because children with Down syndrome are actually more likely to develop ALL (Acute Lymphoblastic Leukemia) rather than AML (Acute Myeloid Leukemia). In an educational context, it is important for medical students to understand the specificities of leukemia in children with Down syndrome as it impacts their management and prognosis. Providing accurate information about the incidence, types, and outcomes of leukemia in this population is crucial for delivering quality care to these patients.

Correct Answer: B

Rationale: Neuroblastoma, a common childhood cancer, can be associated with paraneoplastic syndromes, which are conditions caused by the production of hormones or antibodies by the tumor. In this question, option B, cerebellar ataxia and increased body coordination, is the correct answer as it is not typically associated with paraneoplastic syndromes seen in neuroblastoma. Uncontrollable jerking movements (option A) can be seen in opsoclonus-myoclonus syndrome, unilateral ptosis, myosis, and anhidrosis (option C) can be seen in Horner syndrome, and profound secretory diarrhea (option D) can be a manifestation of vasoactive intestinal peptide (VIP) secretion, all of which can be paraneoplastic syndromes associated with neuroblastoma. Educationally, understanding the various paraneoplastic syndromes associated with neuroblastoma is crucial for pediatric critical care nurses as they care for children with this condition. Recognizing these syndromes can aid in prompt diagnosis and appropriate management of complications, ultimately improving patient outcomes.