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33 Cards in this Set

  • Front
  • Back
Most common brain tumor

Metastasis. Comprise slightly more than half of brain tumors.
Brain metastases solitary vs multiple on MRI

~70% of metastases will be multiple on MRI at time of diagnosis

Which primary brain tumor is the most frequent to metastazise extraneural


Medulloblastoma is the most common primary responsible for extraneural spread: Lung, bone marrow, lymph nodes, abdomen.




Meningioma rarely goes to heart and lungs


Which cancer is the most likely to produce brain metastases


- Lung Ca. followed by breast Ca and melanoma




*Lung Ca + breast Ca account for > 50% of cerebral mets.

Sources of cerebral Mets in pediatrics

Neuroblastoma, rhabdomyosarcoma and Wilm's tumor
Small-cell lung Ca is radiosensitive or radio resistant


Small-cell lung carcinoma (SCLC) is very radiosensitive. (Therefore tx of primary usually not-resected Chemo + XRT).






Which is the most common location of brain metastasis


- Posterior to the sylvian fissure near the juntion of temporal, parietal and occipital lobes (presumably due to embolic spread to terminal MCA branches).


- 75% are parenchymal. 80% of solitary metastasia are located in cerebral hemispheres.


- Cerebellum is a common site of cerebral mets (spread via Batson´s plexus and/or vertebral veins).

Melanoma brain metastasis. Common sites of origin? How does it show on CT


- Skin


- retina


- brain (primary)


- nail bed.




- Melanoma shows hyperdense on CT because of the melanine. AND MRI: T1 hyperintense


and T2 hypointense


**Commonly metatasize 70-90% of pts who died from melanoma in autopsies had brain metastasis


Radiosensitivy of brain metastasis?


Radiosensitive:


- Small-cell lung Ca (SCLC).


- Germ cell tumors


- multiple myeloma


Moderate sensitive


- Breast


Moderate resistant


- Colon


-Non-small lung cell Ca. (NSCLC)


Highly resistant (SRS may be better than WBXRT)


- Melanoma


- thyroid


- Renal cell


- Sarcoma


- AdenoCa.

Radiation therapy for brain metastasis.


1. In pts not considered for surgery dose


2. Pos-op radiation dose


3. Stereotactic radiosurgery usually reserved for what size and how many lesions




** Which cancer receives prophylactic brain radiation

1. Dose for non surgical metastasis is 30 Gy in 10 fractions over two weeks




2. Pos-op is usually 45-50 WBRXT plus a boost the tumor bed bringing the total tx to 55 Gy. All with low fractions of 1.8 - 2.0 Gy




3. Stereotactic RS si usually reserved for lesions < 3cm and usually less than 3 lesions




** Small-cell lung Ca. is treated with prophylactic brain radiation due to its likelyhood to metastazise to the brain

1. With history of treated Ca, which % of brain solitary lesions are mets




2. On the contrary solitary brain mass without Ca. history and negative chest X-ray what percentage are mets


1. 93 %




2. Only 7 % and ~ 87% are primary brain tumors


What is the most important prognostic factor in brain mets


The Karnofsky performance score. Age is also very important.


Karnofsky performance score scale


Score:


100 - Normal, no evidence of disease


90 - Able to carry on normal activity, minor S&S


80 - Normal activity with effort.


70 - Cares for self. Unable to do normal activity or to do active work


60 - Requires occasional assistance.


50 - Requires considerable assistance and f care


40 - Disable: requires special care and assistance


30 - Severely disabled: hospitalized


20 - Very sick: Hospitalized active supportive care


10 - Moribund


0 - Dead

Carcinomatous meningitis (AKA leptomeningeal carcinomatosis). Most common primary tumors? S%S, dg by CSF?


- The most common primary are breast, lung and melanoma.


- S&S usually Simultaneous onset of findings in multiple levels of neuraxis, multiple cranial nerve findings are f. (most common VII, III, V & VI)


- In LP ~ 45% positive in first study but CSF eventually abnormal in > 95% of cases.



Which is the most common foramen magnum tumor

Meningioma


Idiophatic intracranial hypertension (AKA pseudotumor cerebri) is often associated with?


Can cause?


Tx?


Dural sinus thrombosis




- Can cause blindness (often permanent) from optic atrophy


- Tx


- Spontaneous resolution is common.


- Weight loss, diuretics (acetazolamide, etc)


- Optic nerve sheath fenestration (for visual loss without H/A.


- Shunt for H/A associated visual loss.




** likely associated with drugs:keprone, lindane, hypervitamosis A.


Which neurofibromatosis is more common

NF I (AKA von Recklinghausen's disease) > 90 % of cases of neurofibromatosis.

Diagnostic criteria for NF I (von Recklinghausen's disease)


Two or more of the following:


- ≥ 6 café au lait spots (present in ≥ 99%)


- ≥ 2 neurofibromas


- Hyperpigmentation in axila or inguinal areas


- ≥ 2 Lisch nodules = pigmented iris hamartomas


- Distintive osseus abnormality (ej. sphenoid dysplasia, scoliosis)


- A first degree relative with NF I



NF type I tumors associated with


- Schwann-cell tumors on any nerve (but bilateral virtually non-existant)


- Multiple skin neurofibromas


- Hemispheric astrocytomas are the most common IC-tumors.


- gliomas usually pilocytic astros


- 20% develop plexiform neurofibromas


- pheochromocytoma is occasionally present




**Meningiomas are not ass with NF 1

What is a plexiform neurofibroma and is associated with?

Is a tumor from multiple nerve fascicles that grow along the lenght of the nerve is almost pathognomonic of NF type I.
All the neurocutaneous disorders except for ataxia-telangiectasia are inhereted in what way


- Autosomal dominant


- There is also a high rate of spontaneous mutations.


Other associated conditions beside tumors in NF type I


- Aqueductal stenosis


- Macrocephaly


- Unilateral defect in sup. orbit pulsatile exophtalmus.


- 30 to 60 % have mild learning disabilities.


- Kyphoscoliosis


- Syringomielia


- Unidentified bright objects (UBO) in brain/spine MRI.

NF type I has an autosomal dominant inheritance, which gene is the affected one and for which protein does it code


- The NF type I gene is on chromosome 17q11.2 and codes for neurofibromin




** Spontaneous mutation rate is high 30-50%

Diagnostic criteria for NF type 2 (AKA bilateral acoustic NFT)


Definite dx if either:


- Bilateral vestibular schwannomas.


- A first degree relative and either:


a. unilateral VS at age < 30@


b. any two of: meningioma, schwannoma,


glioma (includes astro, ependymoma),


posterior lens opacity (chataracs)


Probable dx:


- Unilateral VS at age < 30@ and any: meningioma, schwannoma, glioma, posterior lens op.


- Multiple meningiomas and either of: schwannoma, glioma or posterior lens opacity

What tumors are associated with NF2


- Toki bilateral VS.


- Multiple intradural spinal tumors are common including intramedullary (specially ependymomas), and extramedullary schwannomas, meningiomas.


- Meningiomas, which can be multiple


- Retinal hamartomas


- Cutaneous schwannomas




** Despite its name is not associated with neurofibromas

Beside tumors other clinical characteristics of NF2


- There are two types the most common, severe form, at younger age of onset 2nd to 3rd decades of life with rapid progression of hearing loss and multiple associated tumors. Milder form presents later in life.




- Chataracts




** Not associated with pheocromocytoma, no intellectual impairment, not associated with Lisch nodules.


NF2 is autosomal dominant due to mutation where and codes for which protein




- NF2 is due to mutation in chromosome 22q12.2, which results in the inactivation of schwannomin (AKA merlin protein).




Sporadic occurrence > 50%

Clinical triad of Tuberous Sclerosis (AKA Bourneville's disease)
Triad: Seizures, mental retardation and sebaceous adenomas. (mm in the face)

Typical CNS finding in Tuberous Sclerosis.




Associated with which other tumor




Characteristic patern in CT


- Subependymal nodule (Tuber) - a hamartoma




TS is commonly associated with Subependymal giant cell astrocytoma.




Can associate with pachygyria or mycrogyria




CT shows intracerebral calcifications (usually subependymal).


Tuberous Sclerosis is characterized by which lesions?


S&S of TS


- TS is characterized by hamartomas of many organs including skin, brain, eyes and kidneys.

Which neurocutaneous disordes (contain unique neurological findings and bening cutaneous lesions) are of importance for neurosurgery


- NF


- Tuberous Sclerosis


- von-Hippel Lindau.


- Sturge Weber Syndrome


- Racemosa angioma (Wyburn-Mason S.): Midbrain and retinal AVMs



In Tuberous Sclerosis what is the chromosomes and proteins involce in the disease

2 tumor supressor genes (Tuberous Sclerosis complex) TSC1 (chromosome 9q34 encoding for hamartin) and TSC2 (chromosome 16p13, coding for tuberlin.

Sturge-Weber Syndrome is characterized by S&S What does X-ray show?


Cardinal signs: 1) Localized cerebral cortical atrophy and calcifications and 2) ipsilateral port-wine facial nevus (usually in distribution of V1)




- Contralateral seizures usually present. usually hemiparesis, hemiatrophy and homonymous hemianopia.




* Plain skull films classically show "tram-tracking" calcifications.




** Lobectomy or hemispherectomy may be needed for refractory seizures.


Most cases are sporadic.