A 5-year-old child, recently diagnosed with ileocecal Burkitt lymphoma; lab investigations reveal: serum uric acid 12 mg/dl, serum sodium, 145 meq/dl; serum potassium, 4.5 meq/dl; serum phosphate 4.4 meq/dl; serum calcium, 8.9 mg/dl; blood urea, 22 mg/dl; serum creatinine, 0.8 mg/dl. Of the following, the MOST effective treatment is

Correct Answer: D

Rationale: In this scenario, the most effective treatment is option D) recombinant urate oxidase. This is because the child has ileocecal Burkitt lymphoma, a malignancy associated with rapid cell turnover leading to tumor lysis syndrome (TLS). The elevated uric acid level of 12 mg/dl indicates TLS, which can lead to renal failure and other complications if not addressed promptly. Recombinant urate oxidase (rasburicase) is the treatment of choice for TLS as it rapidly breaks down uric acid into a more soluble form, preventing uric acid crystallization and subsequent renal damage. This therapy is crucial in preventing acute kidney injury and other TLS-related complications. Option A) excessive hydration is not the most effective treatment as it alone may not be sufficient to rapidly reduce the dangerously high uric acid levels seen in TLS. Option B) sodium bicarbonate is used to alkalinize the urine and prevent uric acid crystallization but is not as effective as recombinant urate oxidase in rapidly lowering uric acid levels. Option C) xanthine oxidase inhibitor (such as allopurinol) is used for chronic management of hyperuricemia but is not as effective as recombinant urate oxidase in the acute setting of TLS. Educationally, understanding the pathophysiology of TLS and the appropriate management is crucial for healthcare providers caring for pediatric oncology patients. Recognizing the urgency of treating elevated uric acid levels in TLS with recombinant urate oxidase can prevent severe complications and improve patient outcomes.

Correct Answer: A

Rationale: In nasopharyngeal carcinoma (NPC) patients, an elevated lactate dehydrogenase (LDH) level carries the worst outcome. LDH is a marker of tissue damage and its elevation indicates more aggressive disease and poorer prognosis in cancer patients. Elevated LDH levels are associated with increased tumor burden, metastasis, and overall disease progression in NPC. Advanced disease (option B) is a significant factor affecting prognosis in NPC, but it is not as specific or indicative of poor outcome as elevated LDH levels. Extensive cervical lymph node involvement (option C) is also a negative prognostic factor in NPC, but it is more related to disease stage rather than a direct marker of poor outcome. Evidence of Epstein-Barr virus (EBV) DNA (option D) is commonly seen in NPC patients but does not necessarily correlate with worse prognosis as much as elevated LDH levels. In an educational context, understanding the significance of different prognostic factors in NPC is crucial for healthcare professionals involved in the care of these patients. Recognizing the impact of elevated LDH levels on prognosis can guide treatment decisions and help in optimizing patient outcomes. Students and practitioners need to be aware of the specific markers and factors that influence prognosis in NPC to provide comprehensive care to these patients.

Correct Answer: A

Rationale: The correct answer is A) intensive chemotherapy. In this scenario, the presence of blast cells in the peripheral blood of a 20-day-old infant with hepatomegaly and abnormal blood counts suggests a high likelihood of acute myeloid leukemia (AML) or acute lymphoblastic leukemia (ALL), both of which require intensive chemotherapy for treatment. Option B) low dose chemotherapy pulses would not be the best approach as aggressive treatment is usually required for acute myeloproliferative disorders to achieve remission and prevent disease progression. Option C) bone marrow transplantation is typically reserved for cases of refractory or relapsed disease, not as initial management. Option D) close follow-up alone is insufficient as immediate treatment is necessary in the presence of blast cells and abnormal blood counts in a pediatric patient. From an educational standpoint, it is crucial for healthcare providers to recognize the urgency of appropriate management in pediatric patients presenting with concerning findings like blast cells in the blood. Understanding the treatment approaches for pediatric leukemias is essential for providing timely and effective care to these vulnerable patients.

Correct Answer: D

Rationale: In this case, the correct answer is D) stage IV S. This is because the scenario describes a 6-month-old girl with a neuroblastoma tumor that has already metastasized extensively, with involvement in the bone marrow, subcutaneous tissue, and a massive abdominal mass. The absence of N-myc amplification suggests a more favorable prognosis than if N-myc were amplified. Option A) stage I is incorrect because there is already metastasis present in this case. Option B) stage II A is also incorrect as the tumor has spread beyond the primary site. Option C) stage III is not the correct choice because of the extensive metastasis described in the scenario. Educationally, understanding the staging of neuroblastoma is crucial for pediatric practitioners as it guides treatment decisions and prognostication. This case highlights the importance of recognizing the extent of disease spread in neuroblastoma cases and how specific genetic markers can influence prognosis and treatment strategies. This knowledge is essential for providing optimal care to pediatric patients with neuroblastoma.

Correct Answer: B

Rationale: The correct answer is B) type of pure epithelial histology because hepatoblastomas with pure epithelial histology have been associated with the most favorable outcomes compared to other histological types. Pure epithelial hepatoblastomas typically respond better to treatment and have a higher survival rate. Option A) mixed type of pure epithelial and mesenchymal elements is incorrect because the presence of mesenchymal elements can indicate a more aggressive tumor behavior. Option C) type of mixed fetal and embryonal histology and Option D) type of undifferentiated histology are also incorrect as they are associated with poorer prognoses and more aggressive disease progression. In an educational context, understanding the histological classification of hepatoblastoma is crucial for pediatric oncology practitioners to make informed treatment decisions and predict patient outcomes. This question highlights the importance of recognizing the different histological types and their implications for patient management and prognosis in pediatric oncology.