Pineoblastoma, RB1-Altered

Preferred Label : Pineoblastoma, RB1-Altered;

NCIt synonyms : Pineoblastoma-RB1; PB, RB1-Altered; PB-RB1;

NCIt definition : Pineoblastoma characterized by alterations in RB1 gene. It may develop in the setting of congenital retinoblastoma, also known as trilateral retinoblastoma. It affects young children and is associated with a dismal prognosis.;

Neoplastic status : Malignant;

Details

Origin ID

C201969;

Disease may have findings
- Aggressive Clinical Course [NCIt concept]
- Flexner-Wintersteiner Rosette Formation [NCIt concept]
- Hemorrhage [NCIt concept]
- High Mitotic Activity [NCIt concept]
- Homer Wright Rosette Formation [NCIt concept]
- Microcalcification [NCIt concept]
- Necrotic Change [NCIt concept]
Excludes anatomical site(s)
- Spinal Cord [NCIt concept]
Has associated anatomic sites
- Brain [NCIt concept]
- Central Nervous System [NCIt concept]
- Nervous System [NCIt concept]
- Pineal Gland [NCIt concept]
- Supratentorial Brain [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Indolent Clinical Course [NCIt concept]
- Pineocytomatous Rosette Formation [NCIt concept]
- Well-Circumscribed Lesion [NCIt concept]
disease_excludes_normal_tissue_origin
- Germinal Layer [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Malignant Neuroectodermal Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Neuroepithelial Cell [NCIt concept]
- Neoplastic Neuroepithelial Cell and Neoplastic Perineural Cell [NCIt concept]
- Neoplastic Small Immature Neuroepithelial Cell [NCIt concept]
- Neoplastic Small Neuroepithelial Cell [NCIt concept]
disease_has_finding
- Aggressive Clinical Course [NCIt concept]
- Increased Cellularity Present [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
disease_has_grade
- Grade 4 [NCIt concept]
disease_has_molecular_abnormality
- RB1 Gene Mutation [NCIt concept]
disease_has_normal_cell_origin
- Embryonic Cell [NCIt concept]
- Neuroepithelial Cell [NCIt concept]
- Neuron, Neuroepithelial Cell, and Supporting Cell of the Nervous System [NCIt concept]
- Pineocyte [NCIt concept]
disease_has_normal_tissue_origin
- Embryonic Tissue [NCIt concept]
- Nerve Tissue, Neuroepithelial Tissue, and Nerve Sheaths [NCIt concept]
- Neuroepithelial Tissue [NCIt concept]
- Pineal Parenchyma [NCIt concept]
disease_has_primary_anatomic_site
- Brain [NCIt concept]
- Central Nervous System [NCIt concept]
- Pineal Gland [NCIt concept]
disease_mapped_to_gene
- RB1 Gene [NCIt concept]
disease_may_have_associated_disease
- Trilateral Retinoblastoma [NCIt concept]
pathogenesis_of_disease_involves_gene
- RB1 Gene [NCIt concept]
- RB1 wt Allele [NCIt concept]

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