clival pathology

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Page 1: Clival Pathology

بسم الله الرحمن الرحيمبسم الله الرحمن الرحيم

قالوا سبحانك ال علم لنا إال ما قالوا سبحانك ال علم لنا إال ما “ “ علمتناعلمتنا

“ “ إنك أنت العليم الحكيمإنك أنت العليم الحكيم

(( 3232البقرة البقرة ) )

صدق الله العظيمصدق الله العظيم

Page 2: Clival Pathology

BYBY

Dr. Mahmoud SaadDr. Mahmoud Saad

Clival PathologyClival Pathology

Page 3: Clival Pathology

Congenital:Congenital:

1-Proatlas segmentation failure1-Proatlas segmentation failure

2-Neuroenteric cysts2-Neuroenteric cysts

Acquired:Acquired:

1-Basilar Invagination1-Basilar Invagination

2-Basilar Impression2-Basilar Impression

3-Rickets Paget's disease3-Rickets Paget's disease

4-Osteogenesis imperfecta4-Osteogenesis imperfecta

5- Clival fractures5- Clival fractures

1ry Neoplastic:1ry Neoplastic:

1-Eosinophilic granuloma1-Eosinophilic granuloma

2-Fibrous dysplasia2-Fibrous dysplasia

3-Chordoma3-Chordoma

4-Chondroma4-Chondroma

5-Chondrosarcoma5-Chondrosarcoma

6-Plasmocytoma6-Plasmocytoma

2ry Neoplastic:2ry Neoplastic:

1-Metastatic1-Metastatic

2-Nasopharyngeal malignancy2-Nasopharyngeal malignancy

3-Ectopic pituitary adenoma3-Ectopic pituitary adenoma

4-Craniopharyngioma4-Craniopharyngioma

Clival PathologyClival Pathology

Page 4: Clival Pathology

Extra-Intra Dural:Extra-Intra Dural:

1-Meningioma1-Meningioma

2-Neurofibroma2-Neurofibroma

3-Chordoma3-Chordoma

4-Glomus tumour4-Glomus tumour

5-5-RhabdomyosarcoRhabdomyosarcomama

2ry Neoplastic:2ry Neoplastic:

1-Metastatic1-Metastatic

2-Nasopharyngeal 2-Nasopharyngeal malignancymalignancy

3-Ectopic pituitary 3-Ectopic pituitary adenomaadenoma

4-Craniopharyngioma4-Craniopharyngioma

Page 5: Clival Pathology

• Malformations of the most caudal of the occipital Malformations of the most caudal of the occipital sclerotomessclerotomes

• Surround the foramen magnum involve the posterior arches Surround the foramen magnum involve the posterior arches of C1.of C1.

• A hindbrain herniation is associated in 33%.A hindbrain herniation is associated in 33%.• The Proatlas component of the dens fail to separate from The Proatlas component of the dens fail to separate from

that portion that forms the basiocciput of the clivus that portion that forms the basiocciput of the clivus the the anterior arch of the atlas comes to rest above the axis body.anterior arch of the atlas comes to rest above the axis body.

• The proatlas abnormality is united with the clivus, distorting The proatlas abnormality is united with the clivus, distorting cervicomedullary junction ventrally.cervicomedullary junction ventrally.

Pro Atlas Segmentation FailuresPro Atlas Segmentation Failures

Page 6: Clival Pathology

Treatment:Treatment:• Relief of neurovascular Relief of neurovascular

compression.compression.• Prevention of recurrence by Prevention of recurrence by

stabilization.stabilization.

Page 7: Clival Pathology

• (AKA enterogenous cysts, endodermal cysts, archenteric cysts, (AKA enterogenous cysts, endodermal cysts, archenteric cysts, gastrocystomas, intestinomas, cystic teratomas, foregut cysts).gastrocystomas, intestinomas, cystic teratomas, foregut cysts).

• 0.7% of tumors and 16% of cysts in the CNS.0.7% of tumors and 16% of cysts in the CNS.• During normal development, the neuroenteric canal closes and During normal development, the neuroenteric canal closes and

the notochord separates from the primitive gut.the notochord separates from the primitive gut.• Transient adhesion occurs between the neural ectoderm and Transient adhesion occurs between the neural ectoderm and

endoderm.endoderm.• When it persists because of the incomplete separation at this  When it persists because of the incomplete separation at this 

adherence or remnant canal, the cyst forms.adherence or remnant canal, the cyst forms.• Intracranial neurenteric cysts are typically intradural, extra-Intracranial neurenteric cysts are typically intradural, extra-

axial posterior fossa masses.axial posterior fossa masses.

Neuroenteric cystNeuroenteric cyst

Page 8: Clival Pathology

Enteric cyst (H&E): Cyst lined by simple columnar Enteric cyst (H&E): Cyst lined by simple columnar and cuboidal epithelium.and cuboidal epithelium.

Page 9: Clival Pathology

D.D:D.D:• A Rathke cleft cyst is usually sellar or suprasellarA Rathke cleft cyst is usually sellar or suprasellar• Colloid cyst the anterior wall of the third ventricle adjacent to foramen Colloid cyst the anterior wall of the third ventricle adjacent to foramen

of Monro.of Monro.

Treatment:Treatment:• Surgical excision. Surgical excision. • Simple aspiration.Simple aspiration.• Cyst wall marsupialization.Cyst wall marsupialization.• Cysto-subarchnoid shunt may be employed when total removal is not Cysto-subarchnoid shunt may be employed when total removal is not

possible.possible.• No role for radiotherapy or chemotherapy.No role for radiotherapy or chemotherapy.

Page 10: Clival Pathology

Basilar Invagination:Basilar Invagination:• Rare condition.Rare condition.

Etiology:Etiology:• Anomalies of occiput, atlas, axis.Anomalies of occiput, atlas, axis.• Softened bone: rickets / osteomalacia, Paget'sSoftened bone: rickets / osteomalacia, Paget's• Fibrous dysplasiaFibrous dysplasia• Dysostoses: OI, achondroplasia, cleidocranial dysostosisDysostoses: OI, achondroplasia, cleidocranial dysostosis

Occurs when the top of the second vertebrae moves upward. Occurs when the top of the second vertebrae moves upward. It can cause the foramen magnum to close.It can cause the foramen magnum to close.

It also may press on the lower brainstem.It also may press on the lower brainstem.

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Symptoms:Symptoms:• If there is pressure on the lower brainstem, its first If there is pressure on the lower brainstem, its first

symptom may be death.symptom may be death.– Periods of confusionPeriods of confusion– Difficulty swallowing or (dysarthria)Difficulty swallowing or (dysarthria)– DizzinessDizziness– Loss of sensationLoss of sensation– Pain in the back of the headPain in the back of the head– Loss of the ability to know how joints are positionedLoss of the ability to know how joints are positioned

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Complications:Complications:• Hydrocephalus or syringomyelia because it blocks the flow of fluid around Hydrocephalus or syringomyelia because it blocks the flow of fluid around

the brain and spinal cord.the brain and spinal cord.

Diagnosis:Diagnosis:• X-rayX-ray• MRI provides the most information.MRI provides the most information.• CT Scan.CT Scan.

Treatment:Treatment:

Conservative approach:Conservative approach:• Drugs: aspirin, without steroids to relieve inflammation (tenderness and Drugs: aspirin, without steroids to relieve inflammation (tenderness and

swelling)swelling)• Neck traction (neck collar) thus relieving pressure on the spinal cord.Neck traction (neck collar) thus relieving pressure on the spinal cord.

If there is pressure on the spinal cord, surgery is recommended.If there is pressure on the spinal cord, surgery is recommended.

Page 13: Clival Pathology

• A rare mechanical deformity of the CVJA rare mechanical deformity of the CVJ• Migration of the upper cervical spine and the odontoid Migration of the upper cervical spine and the odontoid

peg into the base of the skull.peg into the base of the skull.• Brain stem and cerebellum compression.Brain stem and cerebellum compression.• Disturbance of cerebrospinal fluid circulation.Disturbance of cerebrospinal fluid circulation.• Mechanical stretching of cranial nerves.Mechanical stretching of cranial nerves.• Progressive lead to rapid neurological deterioration Progressive lead to rapid neurological deterioration

respiratory arrest or even sudden death.respiratory arrest or even sudden death.• Basilar impression is sometimes referred to as basilar Basilar impression is sometimes referred to as basilar

invagination, and these terms are used interchangeablyinvagination, and these terms are used interchangeably

Basilar impressionBasilar impression

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• Genetic disorder:Genetic disorder: mutation on a gene that mutation on a gene that affects the body’s production of the collagen affects the body’s production of the collagen found in bones, and other tissues.found in bones, and other tissues.

• Fragile bones that break easily “brittle bone Fragile bones that break easily “brittle bone disease.disease.

• OI:OI: muscle weakness, hearing loss, fatigue, muscle weakness, hearing loss, fatigue, joint laxity, curved bones, scoliosis, blue joint laxity, curved bones, scoliosis, blue sclerae, dentinogenesis imperfecta (brittle sclerae, dentinogenesis imperfecta (brittle teeth), and short stature.teeth), and short stature.

Osteogenesis Imperfecta (OI)Osteogenesis Imperfecta (OI)

Page 15: Clival Pathology

Treatment:Treatment:• Physical therapyPhysical therapy• Casts, splintsCasts, splints• Orthopedic surgery implanting rodsOrthopedic surgery implanting rods• Medications to strengthen bonesMedications to strengthen bones• Mobility aids walkers, or wheelchairsMobility aids walkers, or wheelchairs• MedicationsMedications• Bisphosphonates: ©Fosamax ©Reclast ©Forteo for adults onlyBisphosphonates: ©Fosamax ©Reclast ©Forteo for adults only• Growth HormoneGrowth Hormone• Increased vitamin D intakeIncreased vitamin D intake• Physical activityPhysical activity• Potential for gene therapyPotential for gene therapy• At this time, there is no cure.At this time, there is no cure.

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• 3 categories3 categories• Longitudinal injuries injuries to vertebrobasilar vessels.Longitudinal injuries injuries to vertebrobasilar vessels.• Dissection or occlusion: brain stem infarction.Dissection or occlusion: brain stem infarction.• Traumatic aneurysms.Traumatic aneurysms.• Transverse injuries to anterior circulation.Transverse injuries to anterior circulation.• Oblique.Oblique.

• 1-CN deficits: III, VI.1-CN deficits: III, VI.• 2- CSF leak.2- CSF leak.• 3- Diabetes Inspidus.3- Diabetes Inspidus.

Clival FracturesClival Fractures

Clival Fractures are LethalClival Fractures are Lethal

Page 17: Clival Pathology

• 3 clinical variants of Langerhans cell histiocytosis3 clinical variants of Langerhans cell histiocytosis

1- Letterer-Siwe disease1- Letterer-Siwe disease

2-Hand-Schüller-Christian disease2-Hand-Schüller-Christian disease

3- EG (formerly termed histiocytosis X).3- EG (formerly termed histiocytosis X).• EG single or multiple skeletal lesionsEG single or multiple skeletal lesions• Solitary lesions are more common than multiple lesions.Solitary lesions are more common than multiple lesions.• Sites:Sites: skull, mandible, spine, ribs, and long bones. skull, mandible, spine, ribs, and long bones.• Symptoms:Symptoms: localized pain, tenderness, swelling, fever, localized pain, tenderness, swelling, fever,

and leukocytosis.and leukocytosis.

Eosinophilic GranulomaEosinophilic Granuloma

Page 18: Clival Pathology

Pathology:Pathology:• Proliferation of foamy and vacuolated histiocytes is Proliferation of foamy and vacuolated histiocytes is

associated with a variable admixture of neutrophils, associated with a variable admixture of neutrophils, eosinophils, lymphocytes, and plasma cells.eosinophils, lymphocytes, and plasma cells.

• Spontaneous resolution by fibrosis occurring within 1-2 Spontaneous resolution by fibrosis occurring within 1-2 years.years.

• Curettage, excision, or local irradiation leads to cureCurettage, excision, or local irradiation leads to cure

Page 19: Clival Pathology

• Chronic disorder in which bone expands due to abnormal development of Chronic disorder in which bone expands due to abnormal development of fibrous tissuefibrous tissue

• Prognosis of fibrous dysplasia is generally goodPrognosis of fibrous dysplasia is generally good• Malignant degeneration and aggressive behaviour.Malignant degeneration and aggressive behaviour.• The incidence of malignant transformation is highest for monostotic The incidence of malignant transformation is highest for monostotic

craniofacial lesioncraniofacial lesion

There are two types:There are two types:• Monostotic FDMonostotic FD• Polyostotic FDPolyostotic FD• McCune-Albright syndrome: TriadMcCune-Albright syndrome: Triad

1-Polyostotic FD1-Polyostotic FD

2-Skin hyperpigmentation2-Skin hyperpigmentation

3-Endocrine dysfunction (hyperthyroid, precocious 3-Endocrine dysfunction (hyperthyroid, precocious

puberty, Cushing syndrome, acromegaly etc).puberty, Cushing syndrome, acromegaly etc).

Fibrous DysplasiaFibrous Dysplasia

Page 20: Clival Pathology

Clinical:Clinical:• AsymptomaticAsymptomatic• Headache in severe cases.Headache in severe cases.• Cranial nerve involvement.Cranial nerve involvement.

Diagnosis:Diagnosis:• X-rayX-ray• 3 patterns of FD: Cyst-like, Sclerotic and Mixed “Pagetoid”.3 patterns of FD: Cyst-like, Sclerotic and Mixed “Pagetoid”.

Treatment:Treatment:

1-Mainly conservative 1-Mainly conservative

2-Surgical treatment: 2-Surgical treatment: – Cranial nerve compromiseCranial nerve compromise– Severe headacheSevere headache– Development of a malignancy .Development of a malignancy .

Page 21: Clival Pathology

• Solitary mass of neoplastic monoclonal plasma cells in either Solitary mass of neoplastic monoclonal plasma cells in either bone or soft tissue (extramedullary).bone or soft tissue (extramedullary).

Types:Types:• Soft-tissue or nonosseous extramedullary plasmacytoma (EMP)Soft-tissue or nonosseous extramedullary plasmacytoma (EMP)• Solitary bone plasmacytoma (SBP)Solitary bone plasmacytoma (SBP)• Multifocal form of multiple myeloma.Multifocal form of multiple myeloma.• Multiple myelomaMultiple myeloma• Plasmablastic sarcomaPlasmablastic sarcoma

Solitary plasmacytomas: Solitary plasmacytomas: • Plasmacytoma of the skeletal system (SBP)Plasmacytoma of the skeletal system (SBP)• Extramedullary plasmacytoma (EMP)Extramedullary plasmacytoma (EMP)

PlasmacytomaPlasmacytoma

Page 22: Clival Pathology

• 0.2% of all intracranial tumors.0.2% of all intracranial tumors.• 1ry malignant tumour of spine 55% and clivus 35%1ry malignant tumour of spine 55% and clivus 35%• Benign tumors; critical location, local invasion, recurrence, Benign tumors; critical location, local invasion, recurrence,

occasional metastatic spread.occasional metastatic spread.• Characteristic physaliphorous cells (intracellular mucin).Characteristic physaliphorous cells (intracellular mucin).• Slowly growing & radioresitantSlowly growing & radioresitant• Treatment of choice: wide en bloc resection, proton beam radiationTreatment of choice: wide en bloc resection, proton beam radiation• Tumors originating from embryonic remnants of the primitive Tumors originating from embryonic remnants of the primitive

notochord (differentiate to the nucleus pulposus of IVD).notochord (differentiate to the nucleus pulposus of IVD).• Chordomas lie in bone: extradural and induce bone destruction. Chordomas lie in bone: extradural and induce bone destruction.

Chordomas in adults and rarely in younger than 30 years.Chordomas in adults and rarely in younger than 30 years.

Chordoma of the ClivusChordoma of the Clivus

Page 23: Clival Pathology

Presentation:Presentation:• Compression and invasion of important neighboring Compression and invasion of important neighboring

structures.structures.• Headache and cranial nerve deficits (CN VI abducens).Headache and cranial nerve deficits (CN VI abducens).• Dysphagia, facial pain, facial paresis, visual loss, hearing Dysphagia, facial pain, facial paresis, visual loss, hearing

loss, and ataxia.loss, and ataxia.

Examination:Examination:• MRI MRI • CT scanning (assess the degree of bone involvement or CT scanning (assess the degree of bone involvement or

destruction)destruction)

Page 24: Clival Pathology
Page 25: Clival Pathology

CraniopharyngiomaCraniopharyngioma

• 1-3% of intracranial tumors and 13% of suprasellar tumors.1-3% of intracranial tumors and 13% of suprasellar tumors.• In children, 5-10% of all tumors and 56% of sellar and suprasellar In children, 5-10% of all tumors and 56% of sellar and suprasellar

tumors.tumors.• Slow-growing, extra-axial, epithelial-squamous, calcified cystic tumourSlow-growing, extra-axial, epithelial-squamous, calcified cystic tumour• Proteinaceous material Proteinaceous material brownish-yellow color (high content of brownish-yellow color (high content of

floating cholesterol crystals) floating cholesterol crystals) machinery oil fluid. machinery oil fluid.• Arising from remnants of the craniopharyngeal duct and/or Rathke Arising from remnants of the craniopharyngeal duct and/or Rathke

cleftcleft• Occupying the (supra) sellar regionOccupying the (supra) sellar region• It arises in the pituitary stalk and projects into the hypothalamus.It arises in the pituitary stalk and projects into the hypothalamus.• Rare locations:Rare locations: nasopharyngeal. Pure posterior fossa extending down nasopharyngeal. Pure posterior fossa extending down

the cervical spine.the cervical spine.

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1- Embryogenetic theory1- Embryogenetic theory• Development of the adenohypophysis and transformation of the Development of the adenohypophysis and transformation of the

remnant ectoblastic cells of the craniopharyngeal duct and the remnant ectoblastic cells of the craniopharyngeal duct and the involuted Rathke pouch. involuted Rathke pouch.

2-Metaplastic theory2-Metaplastic theory• Metaplasia residual squamous epithelium (derived from stomodeum)Metaplasia residual squamous epithelium (derived from stomodeum)

3-Dual theory3-Dual theory• Adamantinous type (childhood) to embryonic remnantsAdamantinous type (childhood) to embryonic remnants• Adult type (squamous papillary) to metaplastic foci derived from Adult type (squamous papillary) to metaplastic foci derived from

mature cells of the anterior hypophysis mature cells of the anterior hypophysis

3 Theory 3 Theory

Page 27: Clival Pathology

Clinical:Clinical:• Headache (55-86%).Headache (55-86%).• Endocrine dysfunction (66-90%).Endocrine dysfunction (66-90%).• Visual disturbances (37-68%).Visual disturbances (37-68%).• Three clinical syndromes.Three clinical syndromes.

• Prechiasmal: optic atrophy. Prechiasmal: optic atrophy. • Retrochiasmal: hydrocephalus with signs of Retrochiasmal: hydrocephalus with signs of ICT. ICT.• Intrasellar: headache and endocrinopathy.Intrasellar: headache and endocrinopathy.

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• Uncommon benign neoplasmsUncommon benign neoplasms• Modified smooth muscle cells called Modified smooth muscle cells called

glomus cells.glomus cells.• Two variants exist:Two variants exist:

– Solitary glomus tumorsSolitary glomus tumors– Multiple glomus tumors, known as Multiple glomus tumors, known as

glomangiomas or glomulovenous glomangiomas or glomulovenous malformations.malformations.

Glomus TumorsGlomus Tumors

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PathophysiologyPathophysiology

Glomus tumors arise from:Glomus tumors arise from:• Arterial portion of the glomus body.Arterial portion of the glomus body.• Sucquet-Hoyer canal, AV shunt in the dermis that Sucquet-Hoyer canal, AV shunt in the dermis that

contributes to temperature regulation.contributes to temperature regulation.

Causes:Causes:• Proliferation of glomus cells (portion of the glomus body).Proliferation of glomus cells (portion of the glomus body).• Multiple glomus tumors, autosomal dominant pattern.Multiple glomus tumors, autosomal dominant pattern.

Page 30: Clival Pathology

• Benign tumors of peripheral nerves.Benign tumors of peripheral nerves.• Arise from Schwann cells, fibroblasts, and perineural cells.Arise from Schwann cells, fibroblasts, and perineural cells.• Most commonly is the vestibulocochlear nerve.Most commonly is the vestibulocochlear nerve.• Neurofibromas can be single or multiple.Neurofibromas can be single or multiple.• When multiple, associated with NF type I When multiple, associated with NF type I a genetic disorder AKA a genetic disorder AKA

Von Recklinghausen disease.Von Recklinghausen disease.

Symptoms:Symptoms:• Painless, slow-growing mass.Painless, slow-growing mass.• Tinel sign: an electric-like shock when light pressure is applied to Tinel sign: an electric-like shock when light pressure is applied to

the tumor. the tumor. • Affection of motor or sensory nerve function.Affection of motor or sensory nerve function.

NeurofibromasNeurofibromas

Page 31: Clival Pathology

Diagnosis:Diagnosis:• Characteristic appearance on MRI. Characteristic appearance on MRI. • A biopsy of the tumor enables positive diagnosis.A biopsy of the tumor enables positive diagnosis.

Treatment:Treatment:• Surgery is the treatment of choice Surgery is the treatment of choice • Neurofibromas of the skull base Neurofibromas of the skull base expanded expanded

endonasal approach (EEA).endonasal approach (EEA).

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• Posterior fossa meningioma 10% of total intracranial Posterior fossa meningioma 10% of total intracranial meningiomameningioma

• 50% involve the petroclival region.50% involve the petroclival region.• Skull base meningioma has a predilection for female Skull base meningioma has a predilection for female

sex.sex.• Petroclival lesions usually present in 5th or 6th decade.Petroclival lesions usually present in 5th or 6th decade.• Meningioma the commonest lesion in the petroclival Meningioma the commonest lesion in the petroclival

region followed by chordoma.region followed by chordoma.• Chondrosarcoma and neurinoams are rare lesions of Chondrosarcoma and neurinoams are rare lesions of

the petroclival region.the petroclival region.

Clival MeningiomaClival Meningioma

Page 33: Clival Pathology

Castellano Castellano andand Ruggiero Ruggiero classify posterior classify posterior fossa meningiomas (site of dural implant)fossa meningiomas (site of dural implant)

1- Cerebellar convexity.1- Cerebellar convexity.

2- Tentorium.2- Tentorium.

3- Posterior surface of the petrous bone.3- Posterior surface of the petrous bone.

4- Clivus.4- Clivus.

5- Foramen magnum.5- Foramen magnum.

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Desgeorges et al.Desgeorges et al. divided the posterior divided the posterior surface of petrous bone meningiomas surface of petrous bone meningiomas according to the exact site of implant in according to the exact site of implant in relation to the internal auditory canal (IAC) relation to the internal auditory canal (IAC) and labyrinth into:and labyrinth into:

1-Anterior group1-Anterior group

2- Median group (centered on the IAC),2- Median group (centered on the IAC),

3- Posterior group.3- Posterior group.

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Sekhar et al.Sekhar et al. had classified clival meningiomas had classified clival meningiomas based on volume and tumor size into medium, based on volume and tumor size into medium, large and giant subtypes.large and giant subtypes.

Giant tumors were those having maximum Giant tumors were those having maximum diameter more than 4.5 cm.diameter more than 4.5 cm.

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Yasargil et al.Yasargil et al. classify basal posterior fossa classify basal posterior fossa meningiomas into five subgroups: meningiomas into five subgroups:

1.1. Clival. Clival.

2.2. Petroclival. Petroclival.

3.3. Sphenopetroclival. Sphenopetroclival.

4.4. Foramen magnum.Foramen magnum.

5.5. CPA meningiomas.CPA meningiomas.

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• One surgical group, which we call One surgical group, which we call "petroclival Meningiomas"petroclival Meningiomas

1-The clival (medial origin).1-The clival (medial origin).

2-Petroclival (origin at the petrous tip medial 2-Petroclival (origin at the petrous tip medial to the V nerve).to the V nerve).

3-Sphenopetroclival (origin at Meckel's Cave) 3-Sphenopetroclival (origin at Meckel's Cave) meningiomas.meningiomas.

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Investigations:Investigations:• Computerized tomography (CT) scansComputerized tomography (CT) scans• Magnetic resonance imaging (MRI) with contrast Magnetic resonance imaging (MRI) with contrast

enhancement.enhancement.• Cerebral angiography visualization of carotid and Cerebral angiography visualization of carotid and

vertebral circulation.vertebral circulation.• MR angiography and venography.MR angiography and venography.

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• The angiographic relationship of the basilar artery:The angiographic relationship of the basilar artery:

1-The basilar artery is usually pushed posteriorly and to one 1-The basilar artery is usually pushed posteriorly and to one side.side.

2-The posterior and superior cerebellar arteries usually are 2-The posterior and superior cerebellar arteries usually are frequently elevated on the same side of the tumor.frequently elevated on the same side of the tumor.

• The venous phase of the angiogram demonstrating:The venous phase of the angiogram demonstrating:

1-Presence and contribution of two transverse sigmoid sinuses.1-Presence and contribution of two transverse sigmoid sinuses.

2-Delineating the course and dominance of the vein of Labbé.2-Delineating the course and dominance of the vein of Labbé.

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• Most common soft tissue sarcoma in children.Most common soft tissue sarcoma in children.• Greek words rhabdo = rod shape, myo = muscle.Greek words rhabdo = rod shape, myo = muscle.• Tumor arise from a primitive muscle cells Tumor arise from a primitive muscle cells

rhabdomyoblast. rhabdomyoblast. • Histologic groups.Histologic groups.• Embryonal rhabdomyosarcoma (ERMS).Embryonal rhabdomyosarcoma (ERMS).• Alveolar rhabdomyosarcoma (ARMS).Alveolar rhabdomyosarcoma (ARMS).• Undifferentiated sarcoma (UDS).Undifferentiated sarcoma (UDS).

RhabdomyosarcomaRhabdomyosarcoma

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Clinical:Clinical:• Expanding mass. Expanding mass. • Pain. Pain. • Metastatic disease presentation. Metastatic disease presentation.

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Thank YouThank You