A 9-year-old boy presents to the emergency department with emesis and headache of 3 weeks' duration. MRI reveals a large heterogeneous mass in the cerebellum. He undergoes a resection, and the pathology is most consistent with a classic medulloblastoma. Which of the following findings would classify the patient as a high-risk medulloblastoma?

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Question 1 of 5

A 9-year-old boy presents to the emergency department with emesis and headache of 3 weeks' duration. MRI reveals a large heterogeneous mass in the cerebellum. He undergoes a resection, and the pathology is most consistent with a classic medulloblastoma. Which of the following findings would classify the patient as a high-risk medulloblastoma?

Correct Answer: C

Rationale: The correct answer is C. A presurgical spine MRI revealing bulky tumor in the spine classifies the patient as high-risk medulloblastoma due to metastasis. This finding indicates dissemination of the tumor beyond the primary site in the cerebellum, which is associated with poorer prognosis and necessitates more aggressive treatment. Choice A is incorrect because elevated serum and CSF tumor markers are not specific indicators of high-risk medulloblastoma. Choice B (WNT subgrouping) is associated with favorable prognosis, so it does not classify the patient as high-risk. Choice D (postoperative brain MRI showing no residual tumor) is a positive outcome but does not address the presence of metastasis, which is crucial in determining high-risk status.

Question 2 of 5

You are consulted on a 4-year-old girl who is newly diagnosed with standard-risk pre-B acute lymphoblastic leukemia. After reviewing her previous complete blood examinations, you note she has had a platelet count ranging from 80,000 to 100,000 cells/mcL over the past 2 years. Her father mentions that he has also been told he has mild thrombocytopenia. You suspect the child may have a cancer predisposition syndrome. Which sample should you send for analysis, and which gene is most likely implicated?

Correct Answer: B

Rationale: The correct answer is B: Skin fibroblasts to evaluate the ETV6 gene. In individuals with familial thrombocytopenia and a history of malignancy, ETV6 gene mutations are often implicated. Sending skin fibroblasts for analysis allows for genetic testing to identify any ETV6 gene mutations that may be present. Skin fibroblasts are commonly used for genetic testing as they provide a stable and reliable source of DNA. Choice A (Skin fibroblasts to evaluate the RUNXI gene) is incorrect because RUNXI gene mutations are not typically associated with familial thrombocytopenia and cancer predisposition syndromes. Choice C (Buccal swab to evaluate the RUNXI gene) is also incorrect for the same reason. Choice D (Buccal swab to evaluate the ETV6 gene) is incorrect because buccal swabs may not provide enough genetic material for comprehensive testing of the ETV6 gene, which is

Question 3 of 5

A 5-year-old boy is evaluated for apparent ongoing hemolysis. His hemoglobin is 9.5 g/dL, with 8% reticulocytes and MCV 87 fL. Platelets and leukocytes are normal. His direct antiglobulin test (DAT) is negative. No cold agglutinin is detectable. His family history is negative for blood disorders. Peripheral smear reveals basophilic stippling in 10% of the red blood cells. Given these findings, which of the following blood disorders is most likely?

Correct Answer: E

Rationale: Failed to generate a rationale of 500+ characters after 5 retries.

Question 4 of 5

A 19-year-old freshman in college presents with 'lumps' on the right side of his neck and in the right axilla. He had a fever to 39 °C 1 day in the past week. On physical exam, there are firm anterior cervical and axillary nodes, all greater than 2 cm in diameter. A chest x-ray shows a large mediastinal mass. A biopsy of the axillary node reveals classic Hodgkin lymphoma. Which of the following symptoms revealed during the history is a B symptom?

Correct Answer: B

Rationale: The correct answer is B. In Hodgkin lymphoma, B symptoms are systemic symptoms associated with poor prognosis. Weight loss of ≥10% in the past 6 months is a B symptom. This indicates a more aggressive disease. Fever (choice A) is a general symptom. Fatigue (choice C) is common but nonspecific. Alcohol-induced pain (choice D) is not a recognized B symptom in Hodgkin lymphoma. Weight loss is a key indicator of disease severity and is classified as a B symptom.

Question 5 of 5

A 4-year-old child with acute lymphoblastic leukemia is receiving high-dose methotrexate during interim maintenance. He receives ondansetron and арrepitant during his stay, which control his nausea and vomiting well. These medications work by inhibiting signaling in which part of the brain?

Correct Answer: E

Rationale: The correct answer is D: Vomiting center. Ondansetron and aprepitant are antiemetic medications that work by inhibiting signaling in the vomiting center of the brain. The vomiting center is located in the medulla oblongata in the brainstem, which plays a crucial role in triggering the reflexes that lead to nausea and vomiting. By blocking signals in the vomiting center, these medications effectively prevent and control nausea and vomiting. Incorrect choices: A: The vestibular system is responsible for balance and spatial orientation, not for controlling nausea and vomiting. B: The cerebral cortex is involved in higher brain functions such as thinking and decision-making, not in regulating nausea and vomiting. C: The hypothalamus regulates various physiological processes like temperature regulation and hormone release, but it is not directly involved in controlling nausea and vomiting.

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