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Case #3
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患者背景: 7 歳, 男性
適応: Screening MRI in patient with tuberous sclerosis

Case #3

所見

  • Interval enlargement of an enhancing subependymal nodule near the right foramen of Monro measuring 1.5 x 1 cm
  • Multiple additional subcentimeter enhancing subependymal nodules
  • Multiple T2/FLAIR hyperintense cortical and juxtacortical lesions in the bilateral cerebral hemispheres
  • Dilated perivascular spaces in the right greater than left frontal white matter
  • No hydrocephalus
  • Right maxillary sinus mucous retention cyst. Mucosal thickening of bilateral ethmoid air cells

注釈付き画像とイラスト

Enhancing subependymal nodule near the right foramen of Monro (red arrows) which demonstrates growth over a 3-year period, concerning for a subependymal giant cell astrocytoma in this patient with tuberous sclerosis.

Enhancing subependymal nodule near the right foramen of Monro (red arrows) which demonstrates growth over a 3-year period, concerning for a subependymal giant cell astrocytoma in this patient with tuberous sclerosis.

診断

Subependymal giant cell astrocytoma (SEGA)

主要な画像所見

  • SEGAs are enhancing subependymal masses, commonly located near the foramen of Monro
  • While SEGAs can be difficult to distinguish from subependymal nodules, growth over time and size exceeding 1 cm favor SEGA

鑑別診断

  • No differential diagnosis in a patient with known tuberous sclerosis

考察

  • Subependymal giant cell astrocytomas (SEGAs) are almost exclusively seen in patients with tuberous sclerosis
  • They are thought to arise from subependymal nodules and typically present in later childhood or adolescence
  • The primary concern with SEGAs is the development of hydrocephalus due to ventricular outflow obstruction at the foramen of Monro

臨床のポイント

  • In a patient with tuberous sclerosis, an enlarging subependymal nodule exceeding 1 cm in size near the foramen of Monro is highly concerning for a SEGA
  • Watch closely for associated obstructive hydrocephalus

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