You are evaluating a 6-mo-old girl with a firm right suprarenal mass. Histologically, there is no bony involvement, 10% bone marrow involvement, subcutaneous nodules involvement, and massive abdominal mass. The N-myc oncogene is not amplified. According to the international neuroblastoma staging system, the infant is stratified as

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Burns Pediatric Primary Care 7th Edition Test Bank Questions

Question 1 of 5

You are evaluating a 6-mo-old girl with a firm right suprarenal mass. Histologically, there is no bony involvement, 10% bone marrow involvement, subcutaneous nodules involvement, and massive abdominal mass. The N-myc oncogene is not amplified. According to the international neuroblastoma staging system, the infant is stratified as

Correct Answer: D

Rationale: In this scenario, the correct answer is D) stage IV S. This infant's presentation aligns with stage IV neuroblastoma according to the international neuroblastoma staging system. The presence of a massive abdominal mass, subcutaneous nodules, bone marrow involvement, and absence of N-myc oncogene amplification are indicative of a high-risk, advanced-stage disease. Option A) stage I is incorrect because this stage is characterized by a localized tumor without dissemination. Option B) stage II A is also incorrect as it involves unilateral tumor with incomplete resection. Option C) stage III is not applicable as it typically includes unresectable tumor in patients older than 1 year. Educationally, understanding the staging of neuroblastoma is crucial for healthcare providers working in pediatric care. Recognizing the features associated with each stage is vital for appropriate risk stratification, treatment planning, and prognostication. This case underscores the importance of considering multiple factors in determining the stage of neuroblastoma to guide optimal management strategies.

Question 2 of 5

You are meeting with parents of a 10-year-old child who recently develops acute lymphoblastic leukemia (ALL). Which of the following is LEAST likely to increase the risk of CNS relapse in children with ALL?

Correct Answer: A

Rationale: The correct answer is A) first traumatic lumbar puncture (LP) is least likely to increase the risk of CNS relapse in children with ALL. This is because a traumatic LP does not introduce leukemia cells into the cerebrospinal fluid (CSF), which is the main route for CNS relapse in ALL. T-cell leukemia (option B) is associated with a higher risk of CNS relapse compared to B-cell leukemia due to its higher propensity for CNS involvement. Cranial nerve involvement at diagnosis (option C) and the presence of lymphoblasts in the CSF during treatment (option D) are both indicators of CNS disease and increase the risk of CNS relapse in children with ALL. In an educational context, this question highlights the importance of understanding risk factors for CNS relapse in children with ALL. It emphasizes the significance of differentiating between factors that increase the risk of CNS involvement and those that do not. By grasping these nuances, healthcare providers can make informed decisions regarding the management and monitoring of pediatric patients with ALL to optimize outcomes and reduce the risk of relapse.

Question 3 of 5

Childhood primary brain stem tumors are a heterogeneous group of tumors; the outcome usually depends on the tumor location. Which tumor, depending on the site of tumor, carries the worst prognosis?

Correct Answer: D

Rationale: The correct answer is D) diffuse intrinsic. Childhood primary brain stem tumors are a complex group, and the prognosis depends largely on the tumor location. Diffuse intrinsic pontine gliomas (DIPG) are located in the brainstem, making them difficult to treat surgically. They are highly aggressive, infiltrative, and have a poor prognosis due to their location near vital structures that control essential bodily functions. Option A) focal dorsally exophytic tumors are generally associated with a better prognosis as they can be more accessible for surgical intervention compared to deep-seated intrinsic tumors like DIPG. Option B) cervicomedullary diffuse intrinsic tumors are also deep-seated and pose challenges for treatment, but they may have a slightly better prognosis compared to pontine gliomas due to their location at the junction of the brainstem and upper spinal cord. Option C) none of the above is incorrect as the prognosis does vary depending on the specific type and location of the brain stem tumor. Understanding the prognosis of childhood primary brain stem tumors is crucial for healthcare providers working in pediatric primary care to provide accurate information to families, make appropriate referrals to specialists, and offer support throughout the treatment process. Knowledge of tumor types and their implications on prognosis can guide treatment decisions and help manage expectations for both healthcare providers and families.

Question 4 of 5

Of the following, the WORST prognostic factor in pediatric osteosarcoma is

Correct Answer: B

Rationale: In pediatric osteosarcoma, the worst prognostic factor is a poor histologic response to treatment, which is the correct answer (B). A poor response to treatment indicates that the tumor is not responding well to the prescribed therapy, leading to a higher risk of disease progression and poorer outcomes. This factor is crucial in determining the success of treatment and overall prognosis in osteosarcoma cases. Option A, primary pelvic bone tumor, is not the worst prognostic factor as the primary tumor location alone does not necessarily dictate the treatment response or overall prognosis. Osteosarcoma can occur in various bones in the body, and the location itself does not always determine the severity of the disease. Option C, bony metastases at the time of diagnosis, is also a significant factor in the prognosis of osteosarcoma. However, the presence of metastases does not automatically indicate a worse prognosis compared to a poor histologic response to treatment. Response to treatment directly reflects the effectiveness of therapy on the tumor itself. Option D, lung metastases at the time of diagnosis, is a concerning factor in osteosarcoma but may not be the worst prognostic factor. While lung metastases can impact prognosis, the ability of the tumor to respond to treatment is more critical in determining outcomes. In an educational context, understanding prognostic factors in pediatric osteosarcoma is essential for healthcare providers involved in the care of these patients. Recognizing the significance of treatment response and metastatic spread helps in making informed decisions regarding treatment strategies and setting realistic expectations for patients and their families. Monitoring response to therapy and adjusting treatment plans accordingly are crucial in improving outcomes for pediatric osteosarcoma patients.

Question 5 of 5

The physical manifestations of fetal alcohol syndrome include:

Correct Answer: C

Rationale: In the context of pediatric primary care, understanding the physical manifestations of fetal alcohol syndrome is crucial for early identification and appropriate management. The correct answer, option C, includes microcephaly, short philtrum, and intrauterine growth restriction, which are classic features of fetal alcohol syndrome. Option A, abnormal facial tissue, cleft lip, and cleft palate, are not specific to fetal alcohol syndrome but can be seen in other conditions like genetic syndromes or teratogen exposure. Option B, hepatomegaly, hypotonia, and microphthalmia, are not typical features of fetal alcohol syndrome but may be seen in other congenital disorders. Option D, hyperbilirubinemia, jaundice, and failure to thrive, are also not consistent with the characteristic physical findings of fetal alcohol syndrome. Educationally, emphasizing the unique physical characteristics of fetal alcohol syndrome can help healthcare providers in early recognition, appropriate counseling of families, and referral for further evaluation and interventions. Understanding these key features can also aid in the holistic care of children affected by prenatal alcohol exposure, promoting better outcomes and quality of life.

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