Updated: 12/26/2021

Craniopharyngioma

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  • Snapshot
    • A 68-year-old woman presents to the emergency room with profound hypovolemia. She is given IV fluids and also complains of a headache that has been plaguing her for a year but attributed it to severe migraines. It is continuous; however, it seems to have gotten worse recently. She also reports recent weight gain. On physical exam, she has notably slow deep tendon reflexes with facial myxedema. Serum labs come back with hypoglycemia and hyperkalemia. Concerned for possibility of a pituitary lesion, she is sent for a CT scan of the head.
  • Introduction
    • Benign brain tumor arising from Rathke pouch
  • Epidemiology
    • Most common childhood supratentorial tumor
    • Bimodal distribution
      • children 5-14 years
      • adults 65-74 years
  • etiology
    • Pathogenesis
      • derived from remnants of Rathke pouch
      • 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
  • Presentation
    • Symptoms/physical exam
      • most commonly headaches
        • progressive, dull, and continuous
        • papilledema from increased intracranial pressure
      • bitemporal hemianopia
        • recall pituitary adenomas present similarly
        • from compression of optic chiasm
      • can compress pituitary gland and cause hypopituitarism
        • hypothyroidism
          • nonpitting edema
          • slow return phase of DTR
          • weight gain
          • fatigue
        • adrenal failure
          • orthostatic hypotension
          • hypoglycemia
          • hyperkalemia
        • diabetes insipidus
          • frequent urination
  • IMAGING
    • CT scan with contrast for diagnosis
      • suprasellar calcified cyst
    • MRI to assess surgical approach
  • STUDIES
    • Histology
      • cholesterol crystals
      • calcifications
    • Assessment of endocrine function if suspicious
      • serum electrolytes
      • serum and urine osmolality
      • thyroid studies
      • growth hormone levels
      • LH and FSH levels
  • 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
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