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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The Hematologic System ATI Questions

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 due to leptomeningeal spread. This indicates dissemination of the tumor cells beyond the primary site, leading to a poorer prognosis. Choice A is incorrect as AFP and beta-HCG markers are associated with germ cell tumors, not medulloblastomas. Choice B is incorrect as WNT subgrouping is associated with better outcomes. Choice D is incorrect because the absence of residual tumor postoperatively does not necessarily indicate high-risk disease.

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 this scenario, the child's history of persistent thrombocytopenia and the father's mild thrombocytopenia suggest a possible familial predisposition to hematologic disorders. ETV6 gene mutations are associated with inherited thrombocytopenia and predisposition to hematologic malignancies such as acute lymphoblastic leukemia. Skin fibroblasts are typically used for genetic testing, as they contain the individual's complete genetic information. Choice A is incorrect because the RUNXI gene is not typically associated with thrombocytopenia or leukemia predisposition. Choices C and D are incorrect because buccal swabs may not provide sufficient genetic material for comprehensive analysis, and the ETV6 gene is more relevant in this context.

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: 10% weight loss in the past 6 months. In Hodgkin lymphoma, B symptoms include unexplained weight loss of more than 10% in the past 6 months, fever >38°C, and night sweats. Weight loss is a significant indicator of systemic illness and malignancy. In this case, the patient's weight loss, along with the presence of fever and lymphadenopathy, fulfills the criteria for B symptoms in Hodgkin lymphoma. Choices A, C, and D are incorrect because fever, fatigue, and alcohol-induced pain are not specifically classified as B symptoms in the context of Hodgkin lymphoma. Fever alone does not qualify as a B symptom unless it is associated with other systemic symptoms like weight loss and night sweats. Fatigue can be a nonspecific symptom in many conditions, and alcohol-induced pain is not a recognized B symptom in Hodgkin lymphoma.

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 drugs that work by targeting the vomiting center in the brainstem. The vomiting center coordinates the vomiting reflex in response to various stimuli, including chemotherapy drugs like methotrexate. By inhibiting signaling in the vomiting center, these medications effectively prevent nausea and vomiting. The other choices (A: Vestibular system, B: Cerebral cortex, C: Hypothalamus) are not directly involved in the control of nausea and vomiting in response to chemotherapy.

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