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AJNR Awards, New Junior Editors, and more. Read the latest AJNR updates

Case of the Month

Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO

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September 2018
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Next case coming October 9th...

SUPRATENTORIAL LIPOMATOUS EMBRYONAL TUMOR WITH GLIOBLASTOMA COMPONENTS

  • Background
    • Embryonal tumors are highly malignant poorly differentiated tumors of neuroepithelial origin, more common in children, rare in adults (0.4%);
    • Formerly known as primitive neuroectodermal tumors (PNET);
    • Rare differentiation into glial, neuronal and specific mature mesenchymal tissues (such as cartilage, bone, skeletal or smooth muscle and adipose tissue) have been reported;
    • Embryonal tumors are small round cells with hyperchromatic nuclei and scant cytoplasm, with synaptophysin (Syn) positivity;
    • Anaplastic cells with GFAP positivity are signs of glial neoplastic tumors. Necrotic foci, endothelial proliferation, and mitores are also common. 
       
  • Clinical Features
    • ​Intracranial hypertension syndrome: e.g. headache and altered mental status;
    • Focal deficits depending on lesion location
       
  • Key Diagnostic Features
    • ​High cellular density on solid portions are compatible with embryonal and high grade glial tumors;
    • T1-hypertense foci, with signal loss on T1 FS sequence, and chemical shift on GRE are MRI signs suggestive of adipose composition, with fat attenuation on CT
       
  • Differential Diagnosis
    • ​Primary CNS tumors with adipose components are rare; few cases of  medulloblastomas, central neurocytomas, ependymomas, and low grade astrocytoma were reported.
       
  • Treatment
    • The treatment options consist of surgical resection when feasible, followed by radiotherapy and chemotherapy.

Suggested Reading:

  1. Ishizawa K, Kan-nuki S, Kumagai H, et al. Lipomatous primitive neuroectodermal tumor with a glioblastoma component: A case report. Acta Neuropathol. 2002;103(2):193-8, 10.1007/s004010100436.
     
  2. Krishnamurthy S, Powers SK, Towfighi J. Primitive neuroectodermal tumor of cerebrum with adipose tissue. Arch Pathol Lab Med. 2001;125(2):264-6, 10.1043/0003-9985.
     
  3. Selassie L, Rigotti R, Kepes JJ, et al. Adipose tissue and smooth muscle in a primitive neuroectodermal tumor of cerebrum. Acta Neuropathol. 1994;87(2):217-22, 10.1007/BF00296193.

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American Journal of Neuroradiology: 46 (6)
American Journal of Neuroradiology
Vol. 46, Issue 6
1 Jun 2025
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