Cribriform neuroepithelial tumor

Cribriform neuroepithelial tumor (CRINET) is a recently recognized central nervous system neoplasm that arises in the ventricles of young children and is characterized by primitive, non-rhabdoid SMARCB1-deficient cells with prominent cribriform architecture.

Twenty-month-old male with intraventricular tumor underwent transcallosal intraventricular tumor resection and endoscopic intraventricular second look stages. The tumor was initially considered choroid plexus carcinoma and histopathological results pointed CRINET. The patient also received Ommaya reservoir for intrathecal chemotherapy employment. The patient's preoperative and postoperative MRI scans and tumor's pathological features are described with a brief history of the disease in the literature.

Results: Lack of SMARCB1 gene immunoreactivity and presence of cribriform non-rhabdoid trabecular neuroepithelial cells led to the CRINET diagnosis. The surgical technique helped us to approach directly into the third ventricle and perform total resection and intraventricular lavage. The patient recovered without any perioperative complications and is consulted pediatric oncology for further treatment planning.

Conclusion: With our limited knowledge on the matter, our presentation may provide an inside to the course and progress of the CRINET as a very rare tumor and may help to set a basis for future investigations focused on its clinical and pathological features. Long courses of follow-up periods are required for establishing treatment modules and assessing the responses to surgical resection techniques and chemotherapy protocols ¹⁾