Ruptured intratumoral arteriovenous malformation in a patient with dysembryoplastic neuroepithelial tumor: A case report

Background: Dysembryoplastic neuroepithelial tumor (DNET) is a World Health Organization grade I brain tumor, which tends to cause epileptic seizures in children and young adults. Intracranial hemorrhage seldom occurs in DNET. Here, we report a rare case of intratumoral hemorrhage caused by a ruptured intratumoral arteriovenous malformation (AVM) in a patient with DNET. Case Description: A 6-year-old girl underwent subtotal resection of DNET in the right frontal lobe, which had caused persistent clonic seizures. Although the seizures improved, a new contrast-enhanced lesion appeared in the residual tumor 3 years after the initial surgery. One month later, an intratumoral hematoma occurred in the patient. Cerebral angiography demonstrated right frontal AVM in the residual tumor. Surgical removal of AVM was performed. Postoperatively, the AV shunt lesion completely disappeared. However, there was recurrence of an enhanced lesion at the 4 years postoperative follow-up. Cerebral angiography showed an recurrent AVM in the tumor. The patient underwent another resection. No recurrence has been observed at the subsequent follow-up. Conclusion: An acquired AVM can develop after the resection of DNET. Appropriate vascular evaluation is important when the new enhanced lesion is observed.