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Spine and Spinal cord Tumors
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Spine tumors Benign , primary malignant , and metstatic tumors
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Sign and symptoms: Cord compression and n. deficit
Mechanical instability Structural change like scoliosis Pain ( persistent , at rest and at night) Local pain or mechanical pain Local tenderness and muscle spasm Radicular symptoms due to neural foramen involvement
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15% of primary CNS tumors are intraspinal
Most primary CNS spinal tumors are benign Most present by compression rather than invasion
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Primary Skeletal Neoplasms
Cell of origin Osseous Cartilagenous Fibrous Benign Malignant Neoplasms Neoplasms Osteoid osteoma Osteosareoma Osteoblastoma ( and variants Osteochondroma Enchondroma Chondroblastoma Chondromyxoid- fibroma Chondrosarcoma Primary, secondary Fibrosarcoma Malignant fibrous Histiocytoma Fibroma
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Marrow Other Ewing’s sarcoma Multiple myeloma Lymphoma Metastasis
Giant cell tumor Hemangioma Related lesions Eosinophilic-Granuloma Aneurysmal-Bone cyst
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Osteoid osteomas first discovered by Jaffe in 1935 .
May be seen in the spine. Osteoid osteomas involve males more commonly than females. men to women is 2:1
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Osteoid osteomas are seen
predominantly in childen or young adults from 10 to 25 years of age .
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Osteoid Osteomas Have a strong predilection for the posterior
elements of the vertebra (lamina, pedicle, most frequent sites). Neural arch is affected in about 75%, articular facets in about 18%, vertebral bodies in only 7%.
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Osteoid Osteomas Are slightly more common in
the lumbar area, followed by the cervical, thoracic, sacral regions.
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Osteoid Osteomas All patients will present with
back pain & usually is the primary complaint and is not relieved by rest or heat .
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Pain is worse at night and with
recumbency and is frequently relieved by aspirin or other nonsteroidal drugs.
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More commonly the patients will
present with scoliosis, osteoid osteoma is the most frequent cause of painful scoliosis in adolescents .
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Osteoid osteoma is often diagnosed
late with the delay reported being between 18 & 27 months.
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Early in its courses, plain X-Ray films are negative
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Any back pain of greater than
6 weeks, duration in children and young adults should be studied by a technetium bone scan.
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The bone scan will show increased
radionuclide uptake at the site of the lesion .
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Radiologically , the lesion is
characterized by a radiolucent area with a central nidus and surrounding sclerosis (a lesion about 1.5 cm diameter ).
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Treatement is surgery when lesions
involved the posterior elements, they can usually be completely remove through a posterior approach.
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If the lesion has been completely removed , recurrence is unlikely.
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Osteiod osteomas constitute about
2.6% of all excised primary tumors of bone and about 12.1% of all benign tumors .
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Between 7% and 18% of all osteoid
Location Between 7% and 18% of all osteoid osteomas are located in the spine. Lumbar 40% , thoracic 30%, and cervical 30%.
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Osteeoblastoma Osteoblastoma : is a rare benign
neoplasm of bone that accounts for 3% of all benign bone tumors. Pathogenesis of tumor is unknown.
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osteoblastoma The major clinical symptom of osteoblastoma is dull aching, localized pain over the involved bone.
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Pain is insidious in onset
and may have a duration of months to years before diagnosis.
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As opposed to osteoid osteoma, pain of
an osteoblastomais less severe, not nocturnal, and not relieved by salicylates
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Osteoblastoma located in the lumbar spine may be associated with pain radiation into the legs.
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Pain may be aggravated by activity,
was the presenting symptom in 81% and radicular pain was in 29% of patients with spinal involvement.
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Osteoblastoma appear during
the second or third decade of life
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Osteoblastoma has a predilection
for the spine, approximately 40% are located in the axial skeleton.
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Male to female ratio is 2.5 to 10
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In the past osteoblastoma
has been referred to as an osteogenic fibroma, giant osteoid osteoma,
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Physical examination may
demonstrate local tenderness on palpation with mild swelling over the spine .
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A positive straight leg raising
test is present in bout 25% of patients .
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Osteoblastoma associated with
spinal cord compression with result in abnormalities on sensory and motor examination of the lower extremities.
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Radiographics evaluation
of osteoblastoma are variable and nonspecific.
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In the spine lesions are most
commonly located in the posterior elements of the vertebrae (pedicles, lamina, transverse, & spinous processes).
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Osteoblastoma is locted in the sacrum
or lumbar spine in 40% of the lesions, in the cervical spine in 36%, and in the thoracic spine in 24%
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In x-ray osteobastoma is expansile,
with well-circumscribed margins and homogeneous ossification.
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CT. may provide better localization
of the tumor particularly when when the lesion is obscured on plain roentgenograms.
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demonstrating the extension
MRI. is better than CT. in demonstrating the extension of the bone sclerosis.
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Osteochondroma Is a common benign tumor of bone that occurs in single
or multiple location in the skeleton .
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Osteochondromas represent
up to 36% of all benign bone tumors
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Approximately 60 % of patients
develop the lesion between the second and third decade of life ( in multiple lesions before 20 years )
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is postulated to be related to an abnormality of cartilage growth .
Pathogenesis of osteochondroma is postulated to be related to an abnormality of cartilage growth .
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1% to 2% of osteochondromas are located in the spine
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50% in the Lumbosacral 30% in the thoracic 20% in the cervical
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Osteochondroma is frequently
asymptomatic and is discovered only as a painless prominence of bone.
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If pain is present, it is mild,
deep , pain may increase with activity.
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Osteochondromas attached
to the spinal column have been associated with kyphosis and spondylolisthesis
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Osteochondroma may even
grow large enough to cause nerve or spinal compression .
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In the spine osteochondromas
usually occur in the posterior elements especially the spinous process .
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Radionuclide bone scan
shows increased uptake at the site of the tumor .
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Treatment: Osteochondroma requires no therapy when they are
asymptomatic
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Removal is indicated if the tumor
is causing persistent pain or disability or increase in size, or X-Ray features suggestive of malignancy .
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Osteochondroma may occasionally change into malignant chondrosarcoma ( about 10% in patients with multiple lesion ).
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Aneurysmal bone cyst Unknown origin 1.4-2.3% primary bone neoplasms
12% in spine Present with LBP Most in children and infrequent after 30 y 32% associate with other lesion 60% in post elements of spine Growth pattern : enlarg and bone destruction and n. defecit Treatment : endovascular embolization and surgical curettage
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Spinal Cord Tumors In Adults
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The Spinal Cord Figure 13.29a
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Anatomy of the Spinal Cord
Figure 13.30a
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Anatomy of the Spinal Cord
Figure 13.30b
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Spinal cord tumors in Adults
15% of CNS neoplasm arise from cellular constituent of the: spinal cord Filum terminal Nerve Roots meninges Metastatic involvement: rare
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Spinal cord tumors in Adults
S.C.T are categorized according to their relationship to the spinal cord. 1-Intramedullary (I.M) tumors arise within the substance of the spinal cord (S.C) 2-Extramedullary (E.M.) tumor are extrinsic to the spinal cord. 3-I.M & E.M. S.C.T: Small number Communication through a nerve root entry zone. Conus medularis-filum terminale transition zone. Some I.D. tumors extend through the nerve root to extradural compartment.
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SPINAL CORD TUMORS IN ADULTS
Exteramedullary (2/3 of cases) Nerve sheath tumor (40%) Menengioma (40%) Filum terminal ependymoma (15%) Miscellaneous * (rare) (5%) Intramedullary (1/3 of cases) Ependymoma (45%) Astrocytoma † (40%) Hemangioblastoma (5%) Miscellansous † (10%) *Includes paraganglioma, drop metatasis, and ganglioma. † includes oligodendroglioma, ganglioma, neurocytoma, and subependymoma. †includes metatitic tumor. Inclusion tumor (e.g., lipoma), inflammatory pathology (e.g., abscess, tuberculuma, sarcoid), and vascullar pathology (e.g., cavernous malformation, aneurysm).
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Spinal cord tumors in Adults
Extramedullary (2/3 of cases) Nerve sheath tumor (40%) Meningioma (40%) Filum terminale ependymoma (15%) Miscellaneous (Rare) (5%) Metastases Inclusion tumors Cysts Para gangliomas Melanocytic neoplasm E.M. tumor are almost benign and resectable. Intramedullary tumors (1/3 of case) (more than 80%) are primary glial tumors) most of these are histologically benign. Ependymomas % Astrocytomas 40% Oligodendroglioma, Ganglioglioma Neurocutoma Subependymoma hemangio blastoma % Miscellaneous 10%
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nerve sheath tumors 40% of E.M.S.C.T
Schwannomas From schwann cell neurofibromas Schwann cell, perineural cells & fibroblasts. Neurofibromas and schwannomas have different demographical, histological and biological characterstics.
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Neurofibromas Histological appearance
Abundance of fibrous tissues obvious nerve fibers within the tumor stroma Fusiform (plexiform) enlargement of the involved nerve. multiple neurofibromas establish the diagnosis of Neurofibromatousis
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Schwannomas gross appearance
Smooth globoid mass Do not enlarge nerve balanced eccentrically distinct attachment Histological appearance Elongated bipolar cells Fusiform darkly staining nuclei Arranged in compact interlacing palisade formation (antoni-A) Antoni – B- (less common)
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Nerve sheath tumors 25% of I.D. S.C.T. in adults.
Most are solitary schwannoma Peak incidence; 4th – 6th decade of age Men = women Majority arise from dorsal nerve root Ventral root tumors are more common in Neurofibromas. 10% of nerve sheath tumors are epidural or paraspinal. 1% of nerve sheath tumors are intramedullary Retrograde or Antegrade extension of schwannoma is possible 2.5% of I.D. spinal nerve sheath tumors are malignant ½ in neurofibromatosis Malignant nerve sheath tumors rarely survives 1 year.
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Nerve Sheath Tumors
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Meningiomas Meningiomas= nerve sheath tumors= 40% of E.M.S.C.T
Arise from: (mesodermal origin) Arachnoid cap cells embedded in the dura near the root sleeve Pia Dural fibroblast Peak incidence= year, % occur in women 80% are thoracic Upper cervical and foramen magnum are other common sites ventral position Ventrolateral position
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Meningiomas 90% intradural 10% intra-extradural & entirely extradural
gross characteristic smooth fibrous Histological characteristic : Broad dura attachment Bony involvement does not occurred
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Meningiomas
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FILUM TERMINAL EPENDYMOMA
40% of spinal canal ependymomas arise within filum terminale Proximal portion of filum = most common site. Astrocytoma, oligodendroglioma and para ganglioma may also originate in the filum terminal 3th – 5th decade= most common Men= women Myxopapillary= most common Almost all are biologically benign
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CLINICAL FEATURES Variable clinical presentation
Features slow growing intra spinal mass Venrtra location in upper cervical & foramen magnum Suboccipital pain Distal arm weak ness & atrophy Clumsiness of intrinsic hand muscle ↑I.C.P. & hydrocephallus Differnet cord syndromes (Broun Sequard, hemicord) Long tract sings Bowel and bladder dysfunctional Back pain, radicullar pain Worsening pain on recumbency
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Miscellaneous pathology (Rare)
Neoplastic Inclusion cyst Dermoids Epidermoids Lipomas Teratomas Neurentric cyst Paraganglioma Inclusion tumors Non-neoplastic Arachnoid cyst Intraspinal aneurysm Sarcoidosis Tuberculoma Subdural empyema
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Miscellaneous pathology (Rare)
Neoplastic Cavernous mass effect Hemangioblastoma Gangloneuroma Spinal carcinomatosis (rarely presents as mass lesion) Drap metastasis (from intracranial mass lesion)
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Miscellaneous pathology (Rare)
Iclusion cyst Treatment Excision of mass Release of tetherd cord Excision of sinus tract Total extirpation in some cases is not possible
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Miscellaneous pathology (Rare)
Paraganglioma Rare tumor of neural crest origion Arise from filum terminale or cauda equina Benign non- functioning tumor Histologically resembles extra adrenal paraganglioma (carotid body- glomus jugulare) Well circumscribed vascular tumor Non- secretory granules on electronic microscopy Complete removal is possible is most cases
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Miscellaneous pathology (Rare)
Neoplastic Spinal carcinomatous meningitis (frequently complicates systemic cancers) Malignant intracranial tumors (that appose the subarachnoid space or ventricles are the most likely intracranial tumors to demonstrate CSF drop MX. In to spinal SAS)
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Miscellaneous pathology (Rare)
NON Neoplastic Intramedullary and / or extramedullary mass Arachnoid cyst (well know example/ most common in thoracic region) Intraspinal aneurysm (extremely rare, associated with AVM or coarctation of aorta) Dx: MRI, Selective spinal angiography HIVD: trasgressed HIVD to intradural space Inflamatory pathologies (tuberculoma, sarcoidosis, subdural empyema)
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TREATMENT
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NERVE SHEATH TUMORS TREATMENT (1)
1) Gross total excision: Surgical excision in benign N.ST. Recurrence is rare, if gross total removal has been achieved Small I.D. NST Most dumbbell tumor
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NERVE SHEATH TYMORS TREATMENT (2)
2) Gross subtotal excision : NST embeded in the S.C. or on epipial tissues. Some cervical NST Thoracic & cervical paraspinal extension (dumbbell tumors) Anteriorly located N.S.T. TECNIQUES: 1) standard laminectomy with or without facetectomy. dentate ligament section followed by contra lateral facet fusion and lateral mass plating 2) lateral extracavitary approach for concomitant complex exposure of intraspinal and paraspinal compartments. 3) anterior & posterior dumbbell sacral tumors.
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(Meningiomas) Treatment 1
complete surgical removal= treatment of choice Favorable features in compare to intracranial meningiomas Less difficult ventral exposure requirement Absence of bony involvement Lack of venous sinus or major blood vessel involvement 10-15% recurrence rate of intraspinal meningiomas at 10 years after total or near total removal.
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Meningiomas Treatment 2
technique: 1. standard posterior laminectomy 2. Unilateral laminectomy and facetectomy 3. Costotransversectomy for ventral thoracic tumors 4lateral extra cavitary for ventral thoracic tumor 5. extreme lateral approach (Sen & Sekhar method) for significant tumor components above foramen magnum.
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Meningiomas Treatment 3
6. Always the reflected arachnoid layer over the central tumor surface should be approached. 7. large tumors are debulked and then removed, dural attachement is excised or extensively coagulated. 8. devide archnoid adhesions to prevent A. Tetherig B. delayed syrinx formation C. arachnoiditis D. hydrocephalus
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Meningiomas Treatment 4
Rarery spinal meningiomas may extend through a dural nerve root sleeve & present as dumbbell tumor Management federal base Excision and patch graft Extensive in situ coagulation
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Filum Terminale Treatment
Size and relation ship of tumor to the surrounding roots and cauda equina determine the role of surgery. Gross total en bloc resection should be attempted when ever possible. For small and moderate size tumor Well sircumescribed. Inter and decompression may increase the risk of dissemination . Recurrence after en bloc resection are rate.
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Filum ependymomas treatment)
Larg filum terminal ependymomas can present significant problems for surgical resection. High risk of seminarian through CSF ( have been present for many years) Entire neuro axis should be evaluated. These tumor may reach enormous size. May insinuate among the roots and within the archnoid sheaths of the caud equina (un encapsulated and pliable neoplasm).
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Filum ependymomas treatment)
SurginalTechnique piecemeal & subtotal removal Diminish the tumor build 20% recurrence rate anon if a near total piecemeal removal has been acheaved. Radiotherapy delay in situration in which surgery canbe contem plate For early tumor recurrence in biologically aggressive tumors. Posoperatively: for significant tumor buden or dissemination After repeated surger for tumor recurrence.
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Intramedullary Tumors
Neoplastic primary glial tumors 80% Astrocytomas Ependymomas Gangliogliomas Oligodendrogllioms Subependymomas Hemangioblastoma(3- 6%) Metastatic involvement (<5%) (lung & breast = most common) Inclusion tumors Cycts Metastasis Nerve sheath tumors Neurocytomas melanocytoms
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Intramedullary Tumors
Non-Neoplastic Inflammatory condition Bacterial abscess Tuberculoma Sarcoidosis Acute or sub acute clinical course Is characteristic and evidence of systemic involvement further suggests the diagnosis
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Intramedullary Tumors
Non-Neoplastic DDX Inflammatory or demyelinating conditions of the cord Multiple sclerosis Viral or parainfections myelitis Para neoplastic involvement Many of these conditions are grouped as transverse myelitis Acute or subacute course (few hours to few days)
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Intramedullary Tumors
MRI of an acute M.S. Plaque Focal homogeneous contrast enhancement that may be comfined today white matter. Little or no cord enlargement MRI of viral or parainfectious myelitis Patchy contrast enhancement over several cord segments
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Intramedullary Tumors
astrocytoma (occurrence) 3% of CNS astrocytomas arise within spinal cord. Most prevalent in the first 3 decades of life. Most common pediatric “I.M” S.C.T. 90% of I.M .S.C.T in patient younger than l0 years of age. 60% of IM SCT are in adolescence. 60% in cervical and cervicothoracic spinal cord segments. Less common in: thorasic,, lumbosacral cord & conus medularis.
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Intramedullary Tumors
astrocytomas (occurrence) S.C. Astrocytomas represents a heterogeneous group with respect to Histology. Gross characteristics Biology Natural history
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Intramedullary Tumors
astrocytomas (occurrence) Includes Low grade fibrillary astrocytomas. pilocytic astrocytomas Malignant astrocytomas Gangliogliomas Oligodendrogliomas 90% of pediatric astrocytic tumors are benign Most are grade I or II fibrillary astrocutomas up to 1/3 are juvenile epilocytic astrocytomas or gangliogliomas) 10% of pediatric gliomas are malignant astrocytomas or glioblastomas
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Intramedullary Tumors
astrocytomas (occurs) Fibrillary astrocytomas prevails in adult. Juvenile pilocytic astrocytomas prevail in adulthood. 25% of adult astrocytomas are malignant.
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Intramedullary Tumors
Eependymomas (occurrence) Most common I.M.T in adults. Middle adult year (most frequent) Men and women are equally affected. Variety of histological subtypes. Cellular ependymoma (most common) Epithelial Tanycytic (fibrillary) Sub ependymomas myxopapillary mixed almost All are histologically benign Unencapsulated and well circumscribed glial derived tumor.
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Intramedullary Tumors
Hemangioblastomas (occurrence) 3-8% of I.M. S.C.T 15-25% occur in association with von hippel- lindau Syndrome. Rare in childhood. Benign tumor of vascular origin. Sharply circumscribed not encapsulated. All have a pial attachments Most are dorsally or dorsolaterally located.
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Intramedullary Tumors
miscellaneous pathology (occurrence) Dysembryogenic lesions Lipomas= most common= 1% of intra medullary S.C.T Inclusion tumors= rare Cysts= rare These are not true neoplasm Arise from inclusion of mesenchymal tissue Produce symptom in early and middle adult age. They are juxtamedullary (subpial)
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Intramedullary Tumors
miscellaneous pathology (occurrence) metastases 2% of S.C.T (small size of S.C., remote vascular accessibility to hematogenous tumor emboli) Lung, breast, most common Melanocytoma, fibrosarcoma, myxoma Vascular malformation (cavernous)
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Intramedullary Tumors
Clinical features Variable Early symptoms are non- specific Symptom duration before diagnosis: 2-3 years= Weeks-month= for malignant or metastatic neoplasms Shorter course= intratumoral hemorrhage (associated with ependymomas)
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Intramedullary Tumors
Clinical featurs Symptome Pain= most common Sensory complaints= 1/3 of patients Motor complaints= 1/3 of patients Dysesthesia Paradysesthesia Numbness Back & leg pain
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Intramedullary Tumors
Clinical featurs Signs Different weaknesses Centeral cord syndrome Spasticity and sensory disturbances urogenital dysfunction Anorectal dysfunction
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Intramedullary Tumors
Radiology & imaging plain x Ray: obsolete C.T Scan Myelography MRI: modality of choice for diagnosis and pre operative evaluation Most I.M. S.C.T are isointense or slightly hypointense on T1- weighted images. Often there is only ill-defined Spinal cord enlargment on T1 weighted images.
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Intramedullary Tumors
Radiology imaging T2- weighted images are more sensitive (because most tumors are hypintense to the spinal cord on T2) Contrasted T2 studies are more specific Almost all I.M. SCT uptake contrast Uniform contrast enhancement (ependymomas) Polar cysts Heterogeneous enhancements Variable appearance (astrocytoma) histological diagnosis is not possible on the basis Neuroimaging.
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Intramedullary Tumors
Treatment Treatment priority should be long- term tumor control with preservation of neurosurgical function. Surgery most effective treatment Radical Gross total removal objectively documented on posoperative MRI prolongs event- free survival in some patients, but in others it has no effect for most I.M. S.C.T
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Intramedullary Tumors
Treatment Gross total removal For benign & demarcated tumors (Hemangioblastomas, most Edendymos some well circumscribed Astrocytomas & Gangliogliomas) For intra medullary metastasis Surgical histology verinfication Subtotal removal: judicious subtotal internal decompression (lipoma, inclusion tumors) Radiation Chemotherapy
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Intramedullary Tumors
Treatment Radiation therapy (R.T) Benign tumor Uncertain role Indolent natural history of most benign I.M S.C.T is misinterpreted as benefit of radiation therapy. Never theless R.T. may provide some tumor control in some patients with low-grade ependymomas or astrocytomas. This response is neither uniform nor predictable R.T is given for benign but infiltrative S.C.T. Not recommended affer substantial removal of reasonably well circumscribed tumor. R.T is recommended for recurrence of tumor. R.T is recommended for malignant I.M astrocytomas.
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