Which intracranial tumor is associated with 'trilateral retinoblastoma' in children carrying a germline RB1 mutation?
- A Pineoblastoma ✓
- B Medulloblastoma
- C Glioblastoma multiforme
- D Craniopharyngioma
Correct answer: A. Pineoblastoma
Explanation
Trilateral retinoblastoma refers to bilateral hereditary retinoblastoma accompanied by a primitive neuroectodermal tumor of the pineal gland, almost always pineoblastoma. The pineal photoreceptor lineage shares developmental origin with retinal cells, so loss of both RB1 alleles predisposes it to tumorigenesis in the same way as the retina. Medulloblastoma arises in the cerebellum and is linked to other pathways such as SHH signaling, glioblastoma is an adult astrocytic tumor, and craniopharyngioma is not a neuroectodermal malignancy.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.