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Mediastinal primitive neuroectodermal tumor

Imaging findings

Contrast-enhanced chest CT shows an extremely large, highly hypervascular mass in the superior mediastinum. The mass causes significant mass effect, displacing the aortic arch vessels, trachea, and esophagus, and is supplied by hypertrophied intercostal and bronchial artery branches.

Key takeaways

Primitive neuroectodermal tumors are rare, highly aggressive small round cell malignancies that belong to the Ewing sarcoma family and are extremely uncommon in the adult mediastinum. They are typically hypervascular, show rapid growth, respond poorly to radiotherapy or chemotherapy, and primary surgical resection is the treatment of choice.

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