CNS Tumors

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Peds CNS Tumors: Overview - Brainy Bunch Intro

  • Epidemiology:
    • Most common solid tumors in children.
    • 2nd most common pediatric malignancy overall (after leukemia).
  • Common Locations:
    • Younger children (<2 yrs): Infratentorial (posterior fossa) > Supratentorial.
    • Older children/adolescents: Supratentorial predominance ↑.
  • Key Predisposing Genetic Syndromes:
    • Neurofibromatosis Type 1 (NF1)
    • Neurofibromatosis Type 2 (NF2)
    • Von Hippel-Lindau (VHL)
    • Li-Fraumeni Syndrome (TP53)
    • Tuberous Sclerosis Complex (TSC)

Pediatric brain tumors: supra vs infratentorial

⭐ Brain tumors are the most common solid tumors in children and the second most common pediatric malignancy after leukemia.

Infratentorial Tumors - Posterior Fossa Foes

Histopathology of pediatric posterior fossa tumors

  • Most common site for pediatric brain tumors. Key types:
TumorLocationKey HistoNotes
MedulloblastomaCerebellar vermisHomer-Wright rosettesMC malignant, CSF seeding, MYC amp (↓ prog)
Pilocytic Astro.CerebellumRosenthal fibers, GFAP+MC benign, cystic + mural nodule, good prog
Ependymoma4th ventriclePerivascular pseudorosettesVariable prog, true rosettes
-   Located in pons.
-   Highly aggressive, infiltrative.
-   Poor prognosis. 📌 Pons + Palsies = Poor Prognosis.

⭐ Medulloblastoma, the most common malignant pediatric brain tumor, typically arises in the cerebellar vermis and can disseminate via CSF (drop metastases), often presenting with signs of hydrocephalus.

Supratentorial Tumors - Cerebral Command Crashers

  • Craniopharyngioma:
    • Rathke's pouch origin.
    • Suprasellar calcification, cystic ('machine oil' fluid).
    • Endocrine (↓GH), visual (bitemporal hemianopsia).

    ⭐ Craniopharyngiomas classically show suprasellar calcification on imaging and are notorious for causing endocrine dysfunction (e.g., growth hormone deficiency) and bitemporal hemianopsia.

  • Germ Cell Tumors (GCTs):
    • Pineal/suprasellar location.
    • Markers: ↑AFP, ↑$\beta$-hCG.
    • Germinoma (common, radiosensitive), teratoma.
  • Supratentorial Astrocytomas/Gliomas:
    • Common pediatric brain tumors.
    • Varied grades (WHO I-IV); seizures, focal deficits, ↑ICP.
    • Incl. Pilocytic (optic pathway), GBM. MRI of suprasellar craniopharyngiomaoka

Peds CNS Tumors: Dx & Tx - Brain Battle Plan

  • Clinical Presentation:
    • Raised Intracranial Pressure (ICP): Morning headache, vomiting (often projectile, unrelated to meals), papilledema, lethargy.
    • Focal Neurological Deficits: Ataxia, cranial nerve palsies, seizures, endocrinopathy (e.g., diabetes insipidus, growth failure).
  • Diagnosis:
    • MRI with gadolinium contrast: Gold standard imaging.
    • CSF cytology: For suspected leptomeningeal dissemination.
    • Biopsy: Essential for histological diagnosis and grading.
  • Management Principles:
    • Maximal safe surgical resection.
    • Radiotherapy (RT): Adjuvant; avoid/delay in children < 3 years due to neurocognitive sequelae.
    • Chemotherapy: Adjuvant, neoadjuvant, or salvage; specific protocols vary by tumor type.

⭐ The 'triad' of morning headaches, vomiting (often projectile and unrelated to meals), and lethargy strongly suggests raised intracranial pressure (ICP), a common presentation of pediatric brain tumors.

High‑Yield Points - ⚡ Biggest Takeaways

  • CNS tumors: most common solid tumors in children; infratentorial more frequent.
  • Pilocytic astrocytoma: most common pediatric brain tumor; often cerebellar, Rosenthal fibers.
  • Medulloblastoma: most common malignant; cerebellar (vermis); Homer Wright rosettes, radiosensitive.
  • Craniopharyngioma: from Rathke's pouch; suprasellar calcification; visual defects.
  • Ependymoma: often in 4th ventricle; causes hydrocephalus; perivascular pseudorosettes.
  • Key symptoms: morning headache, vomiting, ataxia, papilledema.
  • Brainstem gliomas (e.g., DIPG) have a dismal prognosis.

Practice Questions: CNS Tumors

Test your understanding with these related questions

Which of the following is the most common cause of a mixed cystic and solid suprasellar mass seen on cranial MR scan of a 10-year-old child:

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Flashcards: CNS Tumors

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What is the most common primary malignant tumor of childhood?_____

TAP TO REVEAL ANSWER

What is the most common primary malignant tumor of childhood?_____

Wilms tumor

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