Space Occupying Lesions Flashcards
CNS tumour symptoms?
Clinical examination ; Head aches ,cognitive changes, epileptic seizures ,hemiplegia
Neurological examination papillodeama hemiplegia weakness
Associated disease carcinoma of lung , bowel ,breast ,skin lesion
CT scan single ring enhancing lesion with gadollinium contrast odema and
Biopsy to identify tumour ? Histological classification grade progress treatment
brain tumours?
Primary
Glial = Astrocyte Oligodendrocytes ependymal
Neurons = embryonic tumours
Meninges =meningiomas
Secondary
Metastatic tumours
tumour grading?
Histology ; therefore require a biopsy
Atypia of nuclei & pleiomorphism ( abnormal cell shape)
Vessel wall microvascular proliferation
Mitotic figures
Tumour necrosis
World Health Organization classification of CNS tumours 2007
Grading based upon histological appearances
WHO grades I II III IV
WHO I , II (LOW grades) WHO III , IV (MALIGNANT grades)
Treatment based upon this grading scheme
symptoms of mass?
Local brain injury = focal neurological signs
Increased intracranial pressure = global signs
GCS falls slow vs rapid fall (Depends on tumour grade
Depends upon Rate of tumour growth
WHO grades I II ( slow growth ) or Malignant WHO III IV grades ( rapid growth )
diffuse astrocytoma?
Young adults
Slow growth gradual space occupying effects
Supratentorial-
In anatomy, the supratentorial region of the brain is the area located above the tentorium cerebelli. The area of the brain below the tentorium cerebelli is the infratentorial region. The supratentorial region contains the cerebrum, while the infratentorial region contains the cerebellum.
WHO II
Survival 6-8 years
Malignant transformation into anaplastic astrocytoma about 50% at 5years
anaplastic astrocytoma?
Short history rapid growth and increasing space occupying effect
Contrast enhancing on MRI
Atypical cells no necrosis usually mitotic figures
Vessel wall changes microvascular proliferation
Survival 2-3years
WHO III
Glioblastoma?
Short history with neurological signs confusion ,stroke seizures
Contrast ring enhancing and odema ?
Infiltrating crosses midline
Commonest primary CNS tumour in adults
Tumour necrosis (as well as all the other histological changes ) very important finding WHO grade IV ; very poor survival at 18m -24m
Pilocytic astrocytoma?
Children
Midline brain stem optic nerve ,cerebellum IIIventricle occasionally temporal lobe
Slow growth
MRI hyperintense on T2 can be cystic
Unlikely to have mitosis
Can spread in CSF NF1???
WHO I excellent survival at 10years
Oligodendroglioma?
Young adult with seizures
Hypodense lesion in Frontal lobes OR Contrast enhancement
Oligodendroglioma WHO II
Typical cellular appearance can have calcification
Slow growth , but transforms into anaplastic variety after 3-5years
WHO II
Anaplastic Oligodendroglioma
Nuclear atypia ,multi nucleated , loose clear cytoplasm
Mitotic figures and microvascular changes
Necrosis
WHO IIII
Ependymomas?
Babies ,young adults infratentorial spreads along ventricular system
Some contrast enhancement cystic blocks CSF flow
Hydrocephalus
Ependymoma
Well delineated ,pseudorossettes ,fibrillary cytoplasm of tumour cells occasional mitosis no necrosis
WHO II
Anaplastic Ependymoma
Increased atypical cellularity ,mitotic figures
WHO III
Difficult to predict biological behaviour age <3 y resection CSF spread
embryonal tumours medulloblastoma?
Young children
Midline in Posterior fossa solid intensely enhancing lesion
Hydrocephalus blocks 4th ventricle
Primitive/undifferentiated neurons become malignant
CSF seeding CSF cytology
Survival 50% at 5years
meningiomas?
Dural tumour
Meningioma WHO I but does infiltrate brain
Anaplastic Meningioma WHOIII
malignant cytology and recurrent +++.
metastatic brain tumours?
Single or multiple sites in the CNS
From
Breast Adenocarcinoma
Bronchus Squamous cell carcinoma
Bowel Adenocarcinoma
Melanoma skin
brain tumour therapy?
Await events tumour CT/MRI biopsy or operate when clinically indicated
Depends upon site ie dominant non dominant lobe
Biopsy LARGE TUMOUR and plan chemo or radiotherapy
Resection and give post operative radio therapy
Inoperable give palliative radiotherapy
common symptoms?
Mechanisms of injury by a mass lesion Clinical features of raised intracranial pressure Patterns of altered focal neurological function (supra and infra-tentorial) Common causes of intracranial mass lesions Hydrocephalus
supratentorial region?
The cortex (Excluding the cerebellum)
infretentorium region?
The cerebelum only
Supretentorial masses?
Related to Raised ICP From mass effect (tumour ± oedema) Hydrocephalus (HCP) Progressive focal neurological deficit Destruction of brain by tumour invasion Compression of brain Compression of cranial nerve Weakness → Speech (40-60% left-sided tumours) → Visual deficit →
Headache Worse in morning Associated with Valsalva Nausea & vomiting Seizures Investigate any 1st seizure > 20 yrs old Mental status change Depression, lethargy, apathy, confusion
infretentorial symptoms?
Related to Raised ICP 2nd to hydrocephalus Headache Nausea & vomiting Gait disturbance Vertigo Diplopia Papilloedema
Mass effect in posterior fossa Cerebellar hemisphere → dysmetria, DDK, intention tremor Vermis → Ataxic gait, truncal ataxia Brainstem multiple cranial nerve long-tract nystagmus
calculate the volume of the raniospinal contents?
v.intracranial (constant) = v.brain + v.CSF + v.blood + v.mass lesion
patient assessment?
General examination Lumps Evidence of primary malignancy
Cushing’s triad - HTN (hypertension) Bradycardia Respiratory irregularity
neurocutaneous disorders?
Unique cutaneous + neurological manifestations Familial tendencies Dysplasias of a number of organ systems All responsible genes have been identified 1. Neurofibromatosis 2. Tuberous Sclerosis 3. Von Hippel Lindau disease 4. Sturge Weber Syndrome 5. Ataxia-Telangiectasia 6. Osler-Rendu-Weber Syndrome
what is neurofibromatosis?
Neurofibromatosis (commonly abbreviated NF; neurofibromatosis type 1 is also known as von Recklinghausen disease) is a genetically-inherited disorder in which the nerve tissue grows tumors (neurofibromas) that may be benign and may cause serious damage by compressing nerves and other tissues. The disorder affects all neural crest cells (Schwann cells, melanocytes and endoneurial fibroblasts). Cellular elements from these cell types proliferate excessively throughout the body, forming tumors; melanocytes also function abnormally in this disease, resulting in disordered skin pigmentation and café au lait spots. The tumors may cause bumps under the skin, colored spots, skeletal problems, pressure on spinal nerve roots, and other neurological problems.[1][2]
what is tuberous sclerosis?
Tuberous sclerosis or tuberous sclerosis complex (TSC) is a rare multi-system genetic disease that causes non-malignant tumors to grow in the brain and on other vital organs such as the kidneys, heart, eyes, lungs, and skin. A combination of symptoms may include seizures, developmental delay, behavioral problems, skin abnormalities, lung and kidney disease. TSC is caused by a mutation of either of two genes, TSC1 and TSC2, which code for the proteins hamartin and tuberin respectively. These proteins act as tumor growth suppressors, agents that regulate cell proliferation and differentiation.[1]
The name, composed of the Latin tuber (swelling) and the Greek skleros (hard), refers to the pathological finding of thick, firm and pale gyri, called “tubers,” in the brains of patients postmortem. These tubers were first described by Désiré-Magloire Bourneville in 1880; the cortical manifestations may sometimes still be known by the eponym Bourneville’s disease.
what is von hippel lindau disease?
Von Hippel–Lindau (VHL) disease is a rare, autosomal dominant genetic condition that predisposes individuals to benign and malignant tumours. The most common tumours found in VHL are central nervous system and retinal hemangioblastomas, clear cell renal carcinomas, pheochromocytomas, pancreatic neuroendocrine tumours, pancreatic cysts, endolymphatic sac tumors and epididymal papillary cystadenomas.[1][2] VHL results from a mutation in the von Hippel–Lindau tumor suppressor gene on chromosome 3p25.3.[3]
Signs and symptoms associated with VHL disease include headaches, problems with balance and walking, dizziness, weakness of the limbs, vision problems, and high blood pressure. Conditions associated with VHL disease include angiomatosis, hemangioblastomas, pheochromocytoma, renal cell carcinoma, pancreatic cysts (pancreatic serous cystadenoma) and café au lait spots.[5] Angiomatosis occurs in 37.2% of patients presenting with VHL disease and usually occurs in the retina. As a result, loss of vision is very common. However, other organs can be affected: strokes, heart attacks, and cardiovascular disease are common additional symptoms.[3] Approximately 40% of VHL disease presents with CNS hemangioblastomas and they are present in around 60-80%. Spinal hemangioblastomas are more specific for VHL disease as 80% are found in the disease.[6][7]
what is sturge weber syndrome?
Sturge–Weber syndrome, sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma. It is characterized by abnormal blood vessels on the brain surface. Normally, only one side of the brain is affected.
Sturge-Weber is an embryonal developmental anomaly resulting from errors in mesodermal and ectodermal development. Unlike other neurocutaneous disorders (phakomatoses), Sturge-Weber occurs sporadically (i.e., does not have a hereditary etiology).
what is ataxia telanhiectasia?
Ataxia telangiectasia (A-T) (also referred to as Louis–Bar syndrome) is a rare, neurodegenerative, inherited disease causing severe disability. Ataxia refers to poor coordination and telangiectasia to small dilated blood vessels, both of which are hallmarks of the disease.[1]
A-T affects many parts of the body:
It impairs certain areas of the brain including the cerebellum, causing difficulty with movement and coordination.
It weakens the immune system causing a predisposition to infection.
It prevents repair of broken DNA, increasing the risk of cancer.
Symptoms most often first appear in early childhood (the toddler stage) when children begin to walk. Though they usually start walking at a normal age, they wobble or sway when walking, standing still or sitting, and may appear almost as if they are drunk. In late pre-school and early school age they develop difficulty moving the eyes in a natural manner from one place to the next (oculomotor apraxia). They develop slurred or distorted speech, and swallowing problems. Some have an increased number of respiratory tract infections (ear infections, sinusitis, bronchitis, and pneumonia). Because not all children develop in the same manner or at the same rate, it may be some years before A-T is properly diagnosed. Most children with A-T have stable neurologic symptoms for the first 4–5 years of life, but begin to show increasing problems in early school years.
A-T is caused by a defect in the ATM gene,[2] which is responsible for managing the cell’s response to multiple forms of stress including double-strand breaks in DNA. In simple terms, the protein produced by the ATM gene recognizes that there is a break in DNA, recruits other proteins to fix the break, and stops the cell from making new DNA until the repair is complete.[3]
what is osler rendu weber syndrome?
Hereditary hemorrhagic telangiectasia (HHT), also known as Osler–Weber–Rendu disease and Osler–Weber–Rendu syndrome, is a genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, and often in organs such as the lungs, liver and brain.[1][2]
It may lead to nosebleeds, acute and chronic digestive tract bleeding, and various problems due to the involvement of other organs. Treatment focuses on reducing bleeding from blood vessel lesions, and sometimes surgery or other targeted interventions to remove arteriovenous malformations in organs. Chronic bleeding often requires iron supplements and sometimes blood transfusions. HHT is transmitted in an autosomal dominant fashion, and occurs in one in 5,000 people.[1][2]
The disease carries the names of Sir William Osler, Henri Jules Louis Marie Rendu and Frederick Parkes Weber, who described it in the late 19th and early 20th centuries.[3]
glasgow coma scale?
pupillary resonse to light?
Bilateral small pupils Opiates, metabolic encephalopathies, pontine lesions Unilteral dilated pupil expanding lesion Bilateral dilated pupils inadequate cerebral perfusion
karnofsky score?
100% - Normal, no complaints, no signs of disease
90% - Capable of normal activity, few symptoms or signs of disease
80% - Normal activity with some difficulty, some symptoms or signs
70% - Caring for self, not capable of normal activity or work
60% - Requiring some help, can take care of most personal requirements
50% - Requires help often, requires frequent medical care
40% - Disabled, requires special care and help
30% - Severely disabled, hospital admission indicated but no risk of death
20% - Very ill, requiring urgent admission, supportive measures or treatment
10% - Moribund, rapidly progressive fatal disease processes
0% - Dead
investigations?
CT Head MRI Head MR Spectroscopy Functional MRI Tractography CT Chest/Abdomem
who 2007?
Tumours of neuroepithelial tissue Tumours of cranial and paraspinal nerves Tumours of the meninges Lymphomas and haematopoietic neoplasms Germ cell tumours Tumours of the sellar region Metastatic tumours
types of neuroepithelial tumours?
Astrocytic tumours - Pilocytic astrocytoma (grade I), Diffuse astrocytoma (grade II), Anaplastic astrocytoma (grade III), Glioblastoma (grade IV)
Oligodendroglial tumours - Oligodendroglioma, Anaplastic oligodendroglioma
Oligoastrocytic tumours
common locations for tumours?
age distribution of tumours?
astrocytic brain tumours?
Pilocytic astrocytoma (grade I) - Children only, curable
Diffuse astrocytoma (grade II) - Slowly growing, May transform to high grade, Prognosis 2 - 20 years
Anaplastic astrocytoma/oligodendroglioma - prognosis 1 – 7 years
Glioblastoma multiforme - Prognosis 3 months - 2 years
prognostic factors?
Tumour grade Age (young better, over 70 poor) Performance status (Karnofsky) Extent of surgical resection
treatment options?
Surgery Radiotherapy Stereotactic radiosurgery (SRS) Chemotherapy
