Classification Explorer & 3D Link

Benign nerve sheath tumor arising from Schwann cells, most commonly on the vestibular branch of cranial nerve VIII. Causes unilateral hearing loss and tinnitus.

Mixed nerve sheath tumor strongly associated with Neurofibromatosis Type 1 (NF1). Plexiform variants carry risk of malignant transformation.

Aggressive soft-tissue sarcoma of nerve sheath origin. Roughly half arise in NF1 patients. Carries poor prognosis with high metastatic potential.

The molecular subgroup with the best prognosis (>95% long-term survival). Defined by CTNNB1 mutation and monosomy 6. Arises in the cerebellar vermis.

Hedgehog pathway-driven subgroup. In infants it typically has excellent prognosis; TP53-mutant forms in older children carry very poor outcomes.

The most aggressive medulloblastoma subgroup. Associated with MYC amplification, high metastatic rate, and the worst overall survival.

The most common medulloblastoma subgroup (~35%). Often presents with isochromosome 17q. Intermediate prognosis between WNT and Group 3.

Highly aggressive infant tumor defined by loss of SMARCB1 (INI1) or SMARCA4. Can occur in posterior fossa or supratentorially. Median age at diagnosis is under 2 years.

The most common intracranial germ cell tumor. Highly radiosensitive with >90% cure rate. Shows strong male predilection in the pineal region.

Contains mature tissues from all three germ layers (ectoderm, mesoderm, endoderm). AFP-negative. Curable with surgery when fully mature.

Contains incompletely differentiated fetal-type tissues. Graded by amount of immature neuroectodermal elements. Requires chemotherapy.

Malignant non-germinomatous GCT producing alpha-fetoprotein (AFP). Aggressive behavior requiring intensive multimodal therapy.

Extremely rare, highly malignant GCT producing beta-hCG. Characteristically hemorrhagic and carries the worst prognosis among intracranial GCTs.

The most aggressive primary brain tumor in adults. Defined by rapid growth, central necrosis, and microvascular proliferation. Median survival is approximately 15 months with standard therapy.

Diffuse infiltrative glioma driven by IDH1/2 mutations. Lower-grade forms grow slowly but inevitably progress. Homozygous CDKN2A/B deletion upgrades it to Grade 4 regardless of histology.

Characterized by whole-arm 1p/19q co-deletion and IDH mutation. Often presents with seizures. Relatively chemosensitive, with better prognosis than astrocytoma.

A devastating pediatric glioma (formerly DIPG) that arises in midline structures — pons, thalamus, or spinal cord. Currently lacks effective treatment options.

The most common childhood brain tumor. Slow-growing, well-circumscribed, and often curable with surgery alone. Typically forms a cyst with a bright mural nodule.

Arises from ependymal lining of ventricles or spinal canal. Posterior fossa ependymomas in children are molecularly distinct from supratentorial forms in adults.

A mixed glioneuronal tumor most common in the temporal lobe. Strongly associated with chronic epilepsy. Surgical resection often cures both the tumor and seizures.

The most common primary intracranial tumor (~37% of all). Arises from arachnoid cap cells. Slow-growing and usually curable with complete surgical removal.

Intermediate-grade meningioma with elevated mitotic activity (≥4/10 HPF) or specific histologic features. Has a higher recurrence rate than Grade 1.

Aggressive meningioma defined by high mitotic count (≥20/10 HPF), TERT promoter mutation, or CDKN2A/B homozygous deletion. Carries significant mortality risk.

Highly vascular tumor classically found in the cerebellum. Associated with von Hippel-Lindau (VHL) disease. Often presents with erythrocytosis due to erythropoietin secretion.

Formerly called hemangiopericytoma. Dural-based mesenchymal tumor defined by the NAB2::STAT6 fusion. Higher grades carry extracranial metastatic risk.

Low-grade but locally destructive tumor arising from notochord remnants at the clivus or sacrum. Characterized by Brachyury (TBXT) expression. High recurrence rate.

The most common intracranial tumors overall. Most frequently originate from lung, breast, melanoma, renal cell, and colorectal primaries. Found at gray-white matter junctions.

Diffuse tumor seeding of the subarachnoid space and basilar cisterns. Associated with breast cancer, lung cancer, and melanoma. Presents with cranial neuropathies and hydrocephalus.

Metastatic deposits involving the dura mater, often mimicking meningioma on imaging. Most common from breast and prostate carcinoma.

Accounts for >90% of CNS lymphomas. Classically periventricular with "butterfly" pattern. Highly responsive to methotrexate-based chemotherapy. MYD88 L265P mutation found in >70%.

EBV-driven angiocentric lymphoproliferative disorder. Primarily pulmonary but can involve the CNS. Ranges from indolent to aggressive behavior.

Slow-growing tumor of mature pineocytes. Well-circumscribed with excellent prognosis after surgical resection.

Highly aggressive embryonal tumor of the pineal gland. Can disseminate via cerebrospinal fluid. Associated with RB1 loss (trilateral retinoblastoma).

Rare neuroepithelial tumor arising near the posterior commissure. Shows variable biological behavior; recurrence is common after incomplete resection.

Formerly known as pituitary adenoma. Most common sellar tumor, classified by transcription factors (PIT1, TPIT, SF1). Can cause hormonal excess or visual field defects.

Epithelial tumor from Rathke pouch remnants. Has characteristic calcifications and "machinery oil" cyst fluid. Driven by CTNNB1 mutations in children.

Adult-onset variant driven by BRAF V600E mutation (>95%). Solid, non-calcified, and often responds to targeted therapy.