Childhood Primary Brain Tumors

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Questions and Answers

A child presents with cerebellar involvement, truncal ataxia, and noncommunicating hydrocephalus. Which tumor is most likely indicated by these clinical findings?

  • Pilocytic astrocytoma
  • Craniopharyngioma
  • Medulloblastoma (correct)
  • Ependymoma

A supratentorial tumor in a child is found to have calcifications on imaging. The tumor is compressing the optic chiasm, resulting in bitemporal hemianopia. This clinical presentation is most consistent with which type of tumor?

  • Ependymoma
  • Medulloblastoma
  • Pilocytic astrocytoma
  • Craniopharyngioma (correct)

Microscopic examination of a brain tumor reveals bipolar neoplastic cells with hairlike projections, microcysts, and Rosenthal fibers. Which of the following tumors is most likely?

  • Ependymoma
  • Pilocytic astrocytoma (correct)
  • Medulloblastoma
  • Craniopharyngioma

A tumor found in the fourth ventricle of a child is causing noncommunicating hydrocephalus. Which of the following tumors is most likely, given its location and effect?

<p>Ependymoma (D)</p> Signup and view all the answers

A child presents with Parinaud syndrome, characterized by upward gaze palsy, convergence-retraction nystagmus, and light-near dissociation. Imaging reveals noncommunicating hydrocephalus. Which type of tumor is most likely responsible for these findings?

<p>Pineal gland tumor (A)</p> Signup and view all the answers

Histological analysis of a tumor reveals Homer-Wright rosettes. This is a characteristic feature of which tumor?

<p>Medulloblastoma (D)</p> Signup and view all the answers

Which of the following tumors is derived from remnants of Rathke's pouch?

<p>Craniopharyngioma (C)</p> Signup and view all the answers

A brain tumor in a child is described as having anucleate squamous cells, also known as 'ghost cells', forming keratinous nodules with dystrophic calcifications and cholesterol crystals in 'motor oil'-like fluid. Which tumor type does this microscopic description suggest?

<p>Craniopharyngioma (B)</p> Signup and view all the answers

A primary brain tumor in a child is found to be sending 'drop metastases' to the spinal cord. This pattern of metastasis is most characteristic of which tumor?

<p>Medulloblastoma (C)</p> Signup and view all the answers

Which of the following childhood brain tumors is most likely to present with perivascular pseudorosettes on histological examination?

<p>Ependymoma (A)</p> Signup and view all the answers

Flashcards

Pilocytic astrocytoma

Most common primary brain tumor in childhood, often in the posterior fossa. Cystic appearance with a mural nodule. Benign with good prognosis.

Medulloblastoma

Most common malignant brain tumor in childhood, involving the cerebellum. Can compress the 4th ventricle, leading to noncommunicating hydrocephalus.

Ependymoma

Typically found in the 4th ventricle. Associated with noncommunicating hydrocephalus and poor prognosis.

Craniopharyngioma

Most common childhood supratentorial tumor. Arises along the pituitary stalk, potentially causing bitemporal hemianopia. High recurrence rate.

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Pineal Gland Tumors

Presents with noncommunicating hydrocephalus and Parinaud syndrome (upward gaze palsy, convergence-retraction nystagmus, and light-near dissociation).

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Study Notes

Childhood Primary Brain Tumors

Pilocytic Astrocytoma

  • Most common primary brain tumor in childhood
  • Usually well-circumscribed
  • In children, it's often found in the posterior fossa (e.g., cerebellum)
  • May be supratentorial
  • Has a cystic appearance with mural nodule
  • Generally benign with good prognosis
  • Originates from astrocytes and is GFAP-positive
  • Displays bipolar neoplastic cells with hairlike projections
  • Associated with microcysts and Rosenthal fibers (eosinophilic, corkscrew fibers)

Medulloblastoma

  • Most common malignant brain tumor in childhood
  • Commonly involves the cerebellum
  • Can involve the cerebellar vermis, leading to truncal ataxia
  • Can compress the 4th ventricle, causing noncommunicating hydrocephalus, headaches, and papilledema
  • Can send "drop metastases" to the spinal cord
  • It's a primitive neuroectodermal tumor (PNET)
  • Characterized by Homer-Wright rosettes (small, round, blue cells surrounding a central area of neuropil)
  • Synaptophysin-positive

Ependymoma

  • Most commonly found in the 4th ventricle
  • Can cause noncommunicating hydrocephalus
  • Has a poor prognosis
  • Originates from ependymal cells
  • Shows characteristic perivascular pseudorosettes
  • Rod-shaped blepharoplasts (basal ciliary bodies) are found near the nucleus

Craniopharyngioma

  • Most common childhood supratentorial tumor
  • Calcification is common
  • Typically arises along the pituitary stalk, leading to compression of the optic chiasm and bitemporal hemianopia (may be confused with pituitary adenoma)
  • Has a high recurrence rate
  • Derived from remnants of Rathke pouch (ectoderm)
  • Anucleate squamous cells ("ghost cells") form keratinous nodules with dystrophic calcifications
  • Cholesterol crystals are found in "motor oil"-like fluid within the tumor

Pineal Gland Tumors

  • Most commonly extragonadal germ cell tumors
  • Incidence is higher in males
  • Presents with noncommunicating hydrocephalus (due to compression of the cerebral aqueduct) and Parinaud syndrome (due to compression of the dorsal midbrain)
  • Parinaud syndrome triad includes upward gaze palsy, convergence-retraction nystagmus, and light-near dissociation
  • Similar to testicular seminomas

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