Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
September 2018
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.
- 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.