Tumors of the pineal region in children often belong to 2 categories, namely germ cell tumors and pineal parenchymal tumors. Very rare pathologies have previously been reported in this region. Most of these tumors may be similar radiologically, while their management differs. The present series reports 2 children with pineal region tumors, each one being a rare pathological entity by itself, namely an embryonal tumor with abundant neuropil and true rosettes (ETANTR) and a rosette-forming glioneuronal tumor (RGNT). Very few such cases in each pathology have been reported in the literature for the pediatric age group up to now. Our series consists of 2 children, both presenting with a raised intracranial pressure of short duration. Imaging revealed lesions in the pineal region with similar radiological features. Both ETANTR and RGNT demonstrated mild enhancement. The 2 patients underwent surgical decompression either by Poppen's approach (n = 1) or a supracerebellar infratentorial approach (n = 1). The patient with ETANTR was advised radiotherapy, while the child with RGNT was advised a regular follow-up. This series presents some rare pathologies which can occur in the posterior third ventricular region with similar radiological features. Management differs based on the histology of the case.

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