Adult Primary Brain Tumors

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Questions and Answers

A patient presents with a brain tumor that crosses the corpus callosum, forming a 'butterfly glioma'. Which genetic abnormality is most closely associated with this tumor type?

  • Inactivation of mismatch repair genes (MMR)
  • Overexpression of the RB protein
  • EGFR amplification (correct)
  • Loss of function of tumor suppressor gene TP53

A 55-year-old female is diagnosed with a meningioma. Imaging reveals a well-circumscribed, extra-axial tumor with a dural attachment. Which of the following is the most likely origin of this tumor?

  • Oligodendrocytes
  • Ependymal cells
  • Astrocytes
  • Arachnoid cap cells (correct)

A pathologist examines a brain tumor biopsy under the microscope and observes cells with 'fried egg' appearance surrounding a 'chicken-wire' capillary pattern. What is the most likely diagnosis?

  • Glioblastoma
  • Ependymoma
  • Oligodendroglioma (correct)
  • Medulloblastoma

A patient presents with cerebellar dysfunction and secondary polycythemia. Imaging reveals a cerebellar mass. Which associated systemic condition should be suspected?

<p>Von Hippel-Lindau syndrome (B)</p> Signup and view all the answers

A patient is diagnosed with a prolactinoma. Which of the following hormonal imbalances is most likely to be observed in this patient?

<p>Increased Prolactin, decreased estrogen, and decreased libido (C)</p> Signup and view all the answers

A 40-year-old female presents with galactorrhea and amenorrhea. Further investigation reveals a pituitary adenoma. Which class of medications would be most appropriate for initial treatment?

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

A patient presents with hearing loss, tinnitus, and unsteady gait. MRI reveals a tumor in the cerebellopontine angle. Which cranial nerve is most likely affected?

<p>Facial nerve (CN VII) (D)</p> Signup and view all the answers

A patient is diagnosed with a vestibular schwannoma. Genetic testing reveals a mutation in the NF2 gene. What is the significance of finding a bilateral vestibular schwannoma?

<p>It is a characteristic feature of Neurofibromatosis type 2 (NF2). (D)</p> Signup and view all the answers

A pathologist examines a tumor sample and notes the presence of psammoma bodies arranged in a whorled pattern. Which of the following tumors is most likely to exhibit these histological features?

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

Which of the following primary brain tumors is most likely to be associated with a mutation in a gene that encodes a cytoskeletal protein?

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

Flashcards

Glioblastoma

Highly malignant primary brain tumor (~1-year median survival). Crosses corpus callosum, forming a "butterfly glioma". Associated with EGFR amplification.

Oligodendroglioma

Relatively rare, slow-growing tumor often in frontal lobes. Characterized by "fried egg" cells and a "chicken-wire" capillary pattern.

Meningioma

Typically benign tumor, more common in females. Arises from arachnoid cells, often attached to the dura ("tail"). May be asymptomatic or present with seizures.

Hemangioblastoma

Often cerebellar; associated with von Hippel-Lindau syndrome. Can produce erythropoietin, leading to secondary polycythemia.

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Pituitary Adenoma

Tumor of the pituitary gland. May be hormone-producing (e.g., prolactinoma) or nonfunctioning (causing mass effect).

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Schwannoma

Benign tumor classically at cerebellopontine angle involving cranial nerves V, VII, and VIII. Can cause hearing loss, tinnitus, and unsteady gait. Composed of Schwann cells.

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Study Notes

  • Adult primary brain tumors are discussed

Glioblastoma

  • Glioblastoma is a common and highly malignant primary brain tumor
  • The median survival is around 1 year
  • Glioblastoma is found in the cerebral hemispheres
  • It can cross the corpus callosum, forming what's known as a "butterfly glioma"
  • Glioblastoma is associated with EGFR amplification
  • Histologically it is of astrocyte origin, GFAP+
  • "Pseudopalisading" pleomorphic tumor cells border central areas of necrosis, hemorrhage, and microvascular proliferation

Oligodendroglioma

  • Oligodendroglioma is relatively rare, and slow growing
  • It is most often found in the frontal lobes
  • Oligodendroglioma is often calcified
  • Histologically the cell of origin is the oligodendrocyte.
  • "Fried egg" cells are round, with clear cytoplasm
  • "Chicken-wire" capillary pattern

Meningioma

  • Meningioma is common and typically benign, and more frequent in females than males
  • It occurs along the surface of the brain or spinal cord
  • Meningioma is extra-axial and may have a dural attachment ("tail" sign)
  • It is well circumscribed, with a spherical or lobular shape
  • Meningioma can often be asymptomatic, but may present with seizures or focal neurologic signs
  • Treatment includes resection and/or radiosurgery
  • Histologically it is of arachnoid cell origin
  • Spindle cells are concentrically arranged in a whorled pattern
  • Psammoma bodies are laminated calcifications

Hemangioblastoma

  • Hemangioblastoma is most often cerebellar
  • It is associated with von Hippel-Lindau syndrome when found with retinal angiomas
  • Hemangioblastoma can produce erythropoietin, leading to secondary polycythemia
  • Histologically it is of blood vessel origin
  • Closely arranged, thin-walled capillaries with minimal intervening parenchyma

Pituitary Adenoma

  • Pituitary adenomas may be nonfunctioning (silent) or hyperfunctioning (hormone-producing)
  • Nonfunctional tumors may present with mass effect, such as Bitemporal hemianopia due to pressure on the optic chiasm
  • Pituitary apoplexy can lead to hypopituitarism
  • Prolactinoma classically presents as galactorrhea, amenorrhea, and decreased bone density due to suppression of estrogen - also decreased libido and infertility in males
  • Treatment for prolactinoma includes dopamine agonists and transsphenoidal resection
  • The histology is a hyperplasia of only one type of endocrine cells found in the pituitary
  • They most commonly arise from lactotrophs, leading to hyperprolactinemia
  • Less commonly, from somatotrophs (GH), leading to acromegaly and gigantism or corticotrophs (ACTH) causing Cushing disease
  • Rarely, from thyrotrophs causing excess TSH or gonadotrophs causing excess FSH and LH

Schwannoma

  • Schwannoma is classically found at the cerebellopontine angle
  • It is benign, involving cranial nerves V, VII, and VIII, but can be along any peripheral nerve
  • Often localized to CN VIII in the internal acoustic meatus, leading to vestibular schwannoma
  • Vestibular schwannoma can cause hearing loss, tinnitus, and unsteady gait
  • Bilateral vestibular schwannomas are found in NF-2
  • Treatment includes resection or stereotactic radiosurgery
  • Histologically of Schwann cell origin, S-100+
  • Biphasic, dense, hypercellular areas containing spindle cells alternating with hypocellular, myxoid areas

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