Snapshot A 6-year-old boy has had a daily dull headache for the past 6 months. He says that it is a continuous, dull headache that increases a bit every day. He can’t focus on playing with friends at school anymore due to the pain. On physical exam, his physician is concerned when the patient’s visual field is notably decreased bitemporally. Additionally, physical exam reveals papilledema bilaterally. A stat CT scan is done, revealing suprasellar calcifications. Introduction Childhood tumor in the brain Pathogenesis derived from remnants of Rathke pouch recall that Rathke pouch forms the adenohypophysis in week 4 of gestation arises from surface ectoderm benign on histology slow growing malignant behavior with invasion in local structures may recur after resection metastases rare blood supply typically from anterior circulation of brain tumor characteristics include dystrophic calcification, cyst formation, and hemorrhage Epidemiology most common childhood supratentorial tumor most common cause of pituitary hypofunction in children Presentation Symptoms/physical exam most commonly headaches progressive, dull, and continuous bitemporal hemianopia recall pituitary adenomas present similarly due to compression of optic chiasm can compress pituitary gland and cause hypopituitarism hypothyroidism adrenal failure diabetes insipidus Evaluation CT scan with contrast for diagnosis suprasellar calcified cyst Histology cholesterol crystals calcifications Differential Diagnosis Pituitary adenoma Glioma Medulloblastoma Meningioma Treatment Surgical resection If not completely resectable follow with radiotherapy Prognosis, Prevention, and Complications Prognosis better prognosis for patients < 20 years 5-year-survival rate 99% poorer prognosis for older than 65 years 5-year-survival rate 38% Complications vision loss
QUESTIONS 1 of 4 1 2 3 4 Previous Next Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (M1.EC.13.102) A 9-year-old boy presents with polydipsia, polyuria, and a serum osmolality of 325 mOsm/L. A neurologic examination reveals bitemporal hemianopia. The lesion is believed to be derived from Rathke's pouch remnants. Which of the following is the most likely histologic finding? QID: 100403 Type & Select Correct Answer 1 Liquefactive necrosis 14% (9/65) 2 Cystic spaces 51% (33/65) 3 Lymphocytic infiltrate 18% (12/65) 4 Immune complex deposition 8% (5/65) 5 Branching papillae 6% (4/65) M 2 Question Complexity E Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review Tested Concept Review Full Topic
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