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Primary intracranial malignant ectomesenchymoma (MEM) is a rare proliferative disorder that occasionally involves the central nervous system. So far only 8 cases with confirmed clinicopathological features arising from brain have been reported. The preoperative neruoradiological diagnosis for MEM is still challenging. We presented a 16-year-old patient case of primary MEM located in pineal region. The patient was treated by total surgical resection followed by radiation and experienced no recurrence during the 6-month follow-up. The CT scans revealed a round and smooth-edged hypodensity lesion with sporadic calcification around the circum in right posterior third ventricle infringed on the right metathalamus. And the MRI scans showed hyperintensity on T1WI, T2WI and FLAIR images and hyperintensity on DWI and hypointensity on ADC. The heterogeneously enhanced lesion invaded the right metathalamus and upper mesencephalon and also obstructed the aqueduct of Sylvius. The clinicopathological features of this case raised two points for the first record, as the first regarded the special age and pineal location as well as the second regarded the specific pathological components with malignant large round cells. The histological displays, immunophenotypical characteristics and cytogenesis arrays are the reliable evidence for neuroradiological diagnosis.
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