Specific Brain/Spine Tumors

Types of Astrocytoma

Low-grade (I & II) tend to grow slowly

High-grade (III & IV) tend to grow quickly and spread into surrounding normal brain tissue

Astrocytoma is the most common primary brain tumor in children

True

Astrocytoma clinical presentation

Headache

Seizure

Unilateral weakness

Mental changes

Focal presentation related to tumor location

Approximately ___ out of 10 brain tumors are astrocytoma

2

Astrocytoma Routes of Spread

Sometimes spread along CSF pathways

Rarely spread outside of brain

Considered an intracranial tumor

Astrocytoma Treatment

Maximal surgical resection

Chemotherapy shows no proven advantage

Post-op radiation recommended with a dose range of 50.4-60 Gy, utilized when surgery cannot complete the job

Ependymoma Clinical Presentation

Brain

• Morning headaches

• Nausea/vomiting

• Dizziness and coordination problems

• ICP

• Abducens and oculomotor nerve defects

Spine

• Back pain

• Numbness and weakness in their arms, legs or trunk

• Sexual, urinary or bowel problems

Detection and diagnosis of Astrocytoma

May not enhance on CT or MRI

Ependymoma Detection and diagnosis

MRI or CT scan

Lumbar puncture recommended

Ependymoma Treatment

Surgery for total excision should be attempted prior to adjuvant EBRT

Surgery alone may be sufficient for patients with low-grade, noninvasive tumors, but for those with invasive tumors that are amenable only to subtotal resection

Chemotherapy not routinely recommended for ependymoma, however recurrent ependymomas are sensitive to agents such as nitrosoureas, platinum compounds, and procarbazine

Post-op EBRT with doses of 54 to 59.4 Gy

Glioma/Glioblastoma tends to occur in ________ patients

Older

________ is the most common primary adult brain tumor and has the worst prognosis of CNS tumor

GBM

Types of Gliomas

Grade 1: many kinds; Ex: Pilocytic astrocytomas (more common in children)

Grade 2: diffuse glioma (astrocytomas or oligodendrogliomas)

Grade 3: anaplastic glioma (astrocytomas or oligodendrogliomas)

Grade 4: Glioblastomas

Glioma/Glioblastoma clinical presentations

Persistent headaches

Headaches which are worse in the morning due to ICP

Double or blurred vision

Speech problems

Decreased cognitive abilities

Grasp or limb weakness

New seizures

Detection and diagnosis of Glioma/Glioblastoma

CT lesion

MRI lesion

MRI is most common imaging test when Glioma is suspected

Glioma Treatment

Possible Surgery

Radiation Therapy either alone of followed by chemotherapy

Glioblastoma Treatment

Maximal surgical resection

Chemotherapy- temozolomide given during and after radiotherapy

Postoperative EBRT with a dose of 60 Gy in 6 weeks

Medulloblastoma occur more often in …

Children

Medulloblastomas are part of a group of tumors called ________ tumors

Embryonal

Second most common primary tumor in children

Medulloblastoma

Medulloblastoma clinical presentation

Brain

• Morning headaches

• Nausea and Vomiting

• Coordination problems due to ICP

• Blurred and double vision

• Extreme sleepiness

• Confusion

• Seizures and possible fainting

Spine

• Weakness or numbness in the arms and/or legs

• A change in bowel or bladder habits

• Spinal pain

Medulloblastoma pattern of spread

Very fast-growing

Often spread to other areas of CNS through CSF

Can spread outside of CNS (bones, lungs, lymphatics)- happens more often in children or adults with recurrent tumors

Medulloblastoma detection and diagnosis

MRI or CT

Usually appears as a solid mass in the cerebellum

Can block CSF flow

Lumbar puncture recommended

Medulloblastoma treatment

Treatment of choice is surgery (maximal surgical resection)

Chemo usually follows pediatric guidelines and given with EBRT

EBRT standard is post-op radiation to the craniospinal axis of 23.4 Gy

Boost to the posterior fossa of 54-55.8 Gy or tumor bed

Meningioma etiology

Radiation exposure especially during childhood

Neurofibromatosis type 2 (genetic condition)

Meningioma are more common in females, but Grades II and III occur more often in males

True

Meningiomas are most common in white people

False

Meningiomas are most common in black people

True

Incidence in meningiomas ________ with age

Increases

Meningioma classsifications

Grade I- low grade; fast growing

Grade II- atypical; mid-grade tumors; choroid and clear cell meningiomas

Grade III- anaplastic; fast-growing; papillary and rhabdoid meningiomas

Meningioma clinical presentation

Vision changes and focal problems related to tumor location

Loss of hearing or smell

Confusion

Seizures

Localized headaches that are worse in the morning

Detection and diagnosis of Meningioma

Enhancing MRI or CT lesion associated with dura

Meningioma treatment

Small, non-threatening meningiomas can be observed (especially in elderly)

Surgery- complete resection with negative margins

For unresectable or partially resected tumors, EBRT is used (both SRS and conformal)

Post-op radiation is highly recommended for high grade and malignant meningiomas

Dose 54-60 Gy

Systemic therapy does not have a defined role

Which tumor type is diagnosed most often in males and between the ages of 35-44?

Oligodendroglioma

Oligodendroglioma clinical presentation

Insidious headache

Problems with thinking and memorization

Weakness

Numbness

Problems with balance and movement

Focal problems due to location

Detection and diagnosis of oligodendroglioma

Radiographic calcification

Treatment of oligodendroglioma

Maximal surgical resection when feasible

Role of chemo still undefined

Post-op EBRT is considered the standard with doses of 54-59.4 Gy

Craniospinal irradiation is used only in patients with disseminated disease

Detection and diagnosis of brainstem glioma

May be seen only on MRI image

Biopsy may not be appropriate

*Brainstem Glioma

*Classification, Epidemiology, Etiology and Clinical Presentations the same as Glioma/Astrocytoma

Brainstem Glioma Treatment

Corticosteroids to stabilize neurologic symptoms such as severe hydrocephalus

Surgery has limited role due to location

Temozolomide recently incorporated

EBRT is the mainstay of treatment with dose regimen of 54-60 Gy

Spinal tumors can develop in the spinal canal or within the bones of the spine or within the cord

True

Spinal canal tumor factors

Can be benign or metastatic

Location dependent

Genetic factors: NF1 and NF2

Radiation exposure

Spinal canal tumor clinical presentation

Localized pain (75% of patients)

Radicular (nerve) pain reflects root involvement

Weakness (75% )

Sensory changes (65% )

Sphincter dysfunction (15%)

Low grade tumors have longer duration of symptoms than high grade tumors

Increased intracranial pressure (ICP)

Initial bladder and bowel dysfunction relatively uncommon

Cauda equina tumors absence of ankle reflexes, or plantar responses may occur

Impotence

Loss of anal reflexes may be present

Detection and diagnosis of spinal canal tumors

History and Physical (H&P)

Neurologic Exam

Lumbar puncture

Plain x-rays of spine (may show spine abnormalities)

Myelography (injection of radiocontrast agent into cervical or lumbar spine followed by several x-ray projections) has been replaced by MRI, but useful for those unable to undergo MRI scanning

CT

MRI (gold standard)

Intraoperative Ultrasonography (surgical management) after posterior spinal bony elements have been removed

Intramedullary spinal canal tumors

Tumors arising from the intrinsic substance of the spinal cord

Intradural extramedullary spinal canal tumors

Tumors arising from connective tissues, blood vessels or coverings adjacent to the cord or cauda equina

Prognostic indicators for spinal canal tumors

Tumor type, grade, extent, location, age, presenting neurologic function

Tumor resectability, use of radiation

Neurologic function at diagnosis

Young age at diagnosis is associated with a good 5-year recurrence free survival in patients with astrocytoma

Surgical resection may be favored over biopsy alone for certain low- grade malignancies

Spinal canal tumor treatment considerations

Surgical excision is treatment of choice; gross total excision with preservation of neurologic function is the goal

Radiation Therapy - 45.0 Gy to 54 Gy

Chemotherapy remains experimental in a lot of cases; more common in systemic metastatic cancers; limited role in primary spinal tumors

Spinal Cord tumors account for ___% of CNS tumors

20

Spinal cord tumor etiology

Cause is unclear

Defective genes play a role

Prior history of cancer

Linked to syndromes

• Neurofibromatosis

• Von Hippel-Lindau disease

Clinical presentation for spinal cord tumor

Vary dependent on location

Back pain is the first and most common symptom

Back pain that radiates to other parts of your body such as hips, legs, feet, or arms

Difficulty walking, sometimes leading to falls

Decreased sensitivity to pain, heat, cold

Loss of sensation, especially in arms or legs

Muscle weakness that may occur in varying degrees and different parts of your body, depending on which nerves or part of the spinal cord is affected

Loss of bowel or bladder function

Spinal cord tumor detection and diagnosis

H&P

CT

MRI (gold standard)

PET

X-Rays

Angiography

Lumbar puncture

Spinal cord treatment considerations

Monitoring

Surgery

Radiation Therapy

SRS

Chemotherapy (CNS tumor)

Steroids

Spinal cord compression

Spinal cord compression factors

Considered an emergency

Treatment should be initiated immediately to reduce the risk of permanent neurological damage

Complete treatment within several hours of diagnosis

Most commonly caused by metastasis to the spine

Tumors commonly extend posteriorly through the bony canal into space around the spinal cord

Four most common symptoms of spinal cord compression

Pain

Weakness

Autonomic dysfunction (loss of bowel and bladder sphincter control)

Sensory loss

Detection and treatment of spinal cord compression

Neurologic examination

CT

MRI

Corticosteroids

Radiation - 30-40 Gy in two to four weeks

Dexamethasone

Surgery

Craniospinal Treatment

Treatment of the entire brain and spinal cord simultaneously

Technique: Bilateral whole-brain (helmet) and spine fields (PA) at 100 SSD

Dose: 23.4 Gy CSI, followed by a tumor boost 54-55.8 Gy (1.8 Gy/fraction)

The neck is hyperextended to minimize PA spinal field exit through the mandible/oral cavity

Most commonly treated CNS cancer utilizing craniospinal technique?

Medulloblastoma

Craniospinal - when treated in the prone position, why is the neck hyperextended?

Minimize dose through the mandible/oral cavity during PA spinal field

When is the gap shift applied? How may cm is the gap?

0.5 cm around the brain as a safety gap preferred at junction between inferior whole-brain and superior spine fields