Malignant congenital tumor of the pineal gland rudiment. Literature review and clinical case

We present a clinical case of an extremely rare congenital brain tumor in a 6-month-old child – pineal anlage tumor (PAT), which occurs in 0.04 % of all brain neoplasms in childhood. Foreign literature describes 20 cases of PAT in children, while there are no such publications in the domestic literature.

At present, this tumor is not yet defined as a separate type in the latest edition of the WHO classification in 2021. In the WHO classification of 2007, PAT is described as a rare variant of pineoblastoma with melanotic, cartilaginous and/or rhabdomyoblast differentiation, characterized by heterogeneous elements of neuroepithelial and ectomesenchymal tissue, but without endodermal structures. Due to the rarity of this type of neoplasm, histologic diagnosis can be difficult for the morphologist.

Specific therapy has not been developed at present, the optimal treatment is the maximal complete surgical removal of the volumetric neoplasm, as well as polychemotherapy according to the protocol of pineoblastoma treatment. However, the efficacy of this strategy remains unclear.

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