clivus region pathology

1. BY
Dr. Mahmoud Saad
Clival Pathology

2. Congenital:
1-Proatlas segmentation failure
2-Neuroenteric cysts
Acquired:
1-Basilar Invagination
2-Basilar Impression
3-Rickets Paget's disease
4-Osteogenesis imperfecta
5- Clival fractures
1ry Neoplastic:
1-Eosinophilic granuloma
2-Fibrous dysplasia
3-Chordoma
4-Chondroma
5-Chondrosarcoma
6-Plasmocytoma
2ry Neoplastic:
1-Metastatic
2-Nasopharyngeal malignancy
3-Ectopic pituitary adenoma
4-Craniopharyngioma
Clival Pathology

3. Extra-Intra Dural:
1-Meningioma
2-Neurofibroma
3-Chordoma
4-Glomus tumour
5-Rhabdomyosarcoma
2ry Neoplastic:
1-Metastatic
2-Nasopharyngeal malignancy
3-Ectopic pituitary adenoma
4-Craniopharyngioma

4. • Malformations of the most caudal of the occipital
sclerotomes
• Surround the foramen magnum involve the
posterior arches of C1.
• A hindbrain herniation is associated in 33%.
• The Proatlas component of the dens fail to separate
from that portion that forms the basiocciput of the
clivus  the anterior arch of the atlas comes to rest
above the axis body.
• The proatlas abnormality is united with the clivus,
distorting cervicomedullary junction ventrally.
Pro Atlas Segmentation Failures

5. Treatment:
• Relief of neurovascular compression.
• Prevention of recurrence by
stabilization.

6. • (AKA enterogenous cysts, endodermal cysts,
archenteric cysts, gastrocystomas, intestinomas,
cystic teratomas, foregut cysts).
• 0.7% of tumors and 16% of cysts in the CNS.
• During normal development, the neuroenteric
canal closes and the notochord separates from
the primitive gut.
• Transient adhesion occurs between the neural
ectoderm and endoderm.
• When it persists because of the incomplete
separation at this adherence or remnant canal,
the cyst forms.
• Intracranial neurenteric cysts are typically
intradural, extra-axial posterior fossa masses.
Neuroenteric cyst

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

8. D.D:
• A Rathke cleft cyst is usually sellar or suprasellar
• Colloid cyst the anterior wall of the third ventricle
adjacent to foramen of Monro.
Treatment:
• Surgical excision.
• Simple aspiration.
• Cyst wall marsupialization.
• Cysto-subarchnoid shunt may be employed when
total removal is not possible.
• No role for radiotherapy or chemotherapy.

9. Basilar Invagination:
• Rare condition.
Etiology:
• Anomalies of occiput, atlas, axis.
• Softened bone: rickets / osteomalacia, Paget's
• Fibrous dysplasia
• Dysostoses: OI, achondroplasia, cleidocranial
dysostosis
Occurs when the top of the second vertebrae
moves upward. It can cause the foramen magnum
to close.
It also may press on the lower brainstem.

10. Symptoms:
• If there is pressure on the lower brainstem, its
first symptom may be death.
– Periods of confusion
– Difficulty swallowing or (dysarthria)
– Dizziness
– Loss of sensation
– Pain in the back of the head
– Loss of the ability to know how joints are
positioned

11. Complications:
• Hydrocephalus or syringomyelia because it blocks
the flow of fluid around the brain and spinal cord.
Diagnosis:
• X-ray
• MRI provides the most information.
• CT Scan.
Treatment:
Conservative approach:
• Drugs: aspirin, without steroids to relieve
inflammation (tenderness and swelling)
• Neck traction (neck collar) thus relieving pressure
on the spinal cord.
If there is pressure on the spinal cord, surgery is
recommended.

12. • A rare mechanical deformity of the CVJ
• Migration of the upper cervical spine and the
odontoid peg into the base of the skull.
• Brain stem and cerebellum compression.
• Disturbance of cerebrospinal fluid circulation.
• Mechanical stretching of cranial nerves.
• Progressive lead to rapid neurological deterioration
respiratory arrest or even sudden death.
• Basilar impression is sometimes referred to as
basilar invagination, and these terms are used
interchangeably
Basilar impression

13. • Genetic disorder: mutation on a gene that affects
the body’s production of the collagen found in
bones, and other tissues.
• Fragile bones that break easily “brittle bone
disease.
• OI: muscle weakness, hearing loss, fatigue,
joint laxity, curved bones, scoliosis, blue sclerae,
dentinogenesis imperfecta (brittle teeth), and
short stature.
Osteogenesis Imperfecta (OI)

14. Treatment:
• Physical therapy
• Casts, splints
• Orthopedic surgery implanting rods
• Medications to strengthen bones
• Mobility aids walkers, or wheelchairs
• Medications
• Bisphosphonates: ©Fosamax ©Reclast ©Forteo for
adults only
• Growth Hormone
• Increased vitamin D intake
• Physical activity
• Potential for gene therapy
• At this time, there is no cure.

15. • 3 categories
• Longitudinal injuries injuries to vertebrobasilar
vessels.
• Dissection or occlusion: brain stem infarction.
• Traumatic aneurysms.
• Transverse injuries to anterior circulation.
• Oblique.
• 1-CN deficits: III, VI.
• 2- CSF leak.
• 3- Diabetes Inspidus.
Clival Fractures
Clival Fractures are Lethal

16. • 3 clinical variants of Langerhans cell histiocytosis
1- Letterer-Siwe disease
2-Hand-Schüller-Christian disease
3- EG (formerly termed histiocytosis X).
• EG single or multiple skeletal lesions
• Solitary lesions are more common than multiple
lesions.
• Sites: skull, mandible, spine, ribs, and long
bones.
• Symptoms: localized pain, tenderness, swelling,
fever, and leukocytosis.
Eosinophilic Granuloma

17. Pathology:
• Proliferation of foamy and vacuolated histiocytes
is associated with a variable admixture of
neutrophils, eosinophils, lymphocytes, and
plasma cells.
• Spontaneous resolution by fibrosis occurring
within 1-2 years.
• Curettage, excision, or local irradiation leads to
cure

18. • Chronic disorder in which bone expands due to
abnormal development of fibrous tissue
• Prognosis of fibrous dysplasia is generally good
• Malignant degeneration and aggressive behaviour.
• The incidence of malignant transformation is
highest for monostotic craniofacial lesion
There are two types:
• Monostotic FD
• Polyostotic FD
• McCune-Albright syndrome: Triad
1-Polyostotic FD
2-Skin hyperpigmentation
3-Endocrine dysfunction (hyperthyroid, precocious
puberty, Cushing syndrome, acromegaly etc).
Fibrous Dysplasia

19. Clinical:
• Asymptomatic
• Headache in severe cases.
• Cranial nerve involvement.
Diagnosis:
• X-ray
• 3 patterns of FD: Cyst-like, Sclerotic and Mixed
“Pagetoid”.
Treatment:
1-Mainly conservative
2-Surgical treatment:
– Cranial nerve compromise
– Severe headache
– Development of a malignancy .

20. • Solitary mass of neoplastic monoclonal plasma
cells in either bone or soft tissue (extramedullary).
Types:
• Soft-tissue or nonosseous extramedullary
plasmacytoma (EMP)
• Solitary bone plasmacytoma (SBP)
• Multifocal form of multiple myeloma.
• Multiple myeloma
• Plasmablastic sarcoma
Solitary plasmacytomas:
• Plasmacytoma of the skeletal system (SBP)
• Extramedullary plasmacytoma (EMP)
Plasmacytoma

21. • 0.2% of all intracranial tumors.
• 1ry malignant tumour of spine 55% and clivus 35%
• Benign tumors; critical location, local invasion,
recurrence, occasional metastatic spread.
• Characteristic physaliphorous cells (intracellular mucin).
• Slowly growing & radioresitant
• Treatment of choice: wide en bloc resection, proton
beam radiation
• Tumors originating from embryonic remnants of the
primitive notochord (differentiate to the nucleus
pulposus of IVD).
• Chordomas lie in bone: extradural and induce bone
destruction.
Chordomas in adults and rarely in younger than 30
years.
Chordoma of the Clivus

22. Presentation:
• Compression and invasion of important
neighboring structures.
• Headache and cranial nerve deficits (CN VI
abducens).
• Dysphagia, facial pain, facial paresis, visual
loss, hearing loss, and ataxia.
Examination:
• MRI
• CT scanning (assess the degree of bone
involvement or destruction)

24. Craniopharyngioma
• 1-3% of intracranial tumors and 13% of suprasellar tumors.
• In children, 5-10% of all tumors and 56% of sellar and
suprasellar tumors.
• Slow-growing, extra-axial, epithelial-squamous, calcified
cystic tumour
• Proteinaceous material  brownish-yellow color (high
content of floating cholesterol crystals)  machinery oil
fluid.
• Arising from remnants of the craniopharyngeal duct and/or
Rathke cleft
• Occupying the (supra) sellar region
• It arises in the pituitary stalk and projects into the
hypothalamus.
• Rare locations: nasopharyngeal. Pure posterior fossa
extending down the cervical spine.

25. 1- Embryogenetic theory
• Development of the adenohypophysis and
transformation of the remnant ectoblastic cells of the
craniopharyngeal duct and the involuted Rathke pouch.
2-Metaplastic theory
• Metaplasia residual squamous epithelium (derived from
stomodeum)
3-Dual theory
• Adamantinous type (childhood) to embryonic remnants
• Adult type (squamous papillary) to metaplastic foci
derived from mature cells of the anterior hypophysis
3 Theory

26. Clinical:
• Headache (55-86%).
• Endocrine dysfunction (66-90%).
• Visual disturbances (37-68%).
• Three clinical syndromes.
• Prechiasmal: optic atrophy.
• Retrochiasmal: hydrocephalus with signs of  ICT.
• Intrasellar: headache and endocrinopathy.

27. • Uncommon benign neoplasms
• Modified smooth muscle cells called glomus
cells.
• Two variants exist:
– Solitary glomus tumors
– Multiple glomus tumors, known as glomangiomas or
glomulovenous malformations.
Glomus Tumors

28. Pathophysiology
Glomus tumors arise from:
• Arterial portion of the glomus body.
• Sucquet-Hoyer canal, AV shunt in the dermis that
contributes to temperature regulation.
Causes:
• Proliferation of glomus cells (portion of the
glomus body).
• Multiple glomus tumors, autosomal dominant
pattern.

29. • Benign tumors of peripheral nerves.
• Arise from Schwann cells, fibroblasts, and perineural
cells.
• Most commonly is the vestibulocochlear nerve.
• Neurofibromas can be single or multiple.
• When multiple, associated with NF type I  a genetic
disorder AKA Von Recklinghausen disease.
Symptoms:
• Painless, slow-growing mass.
• Tinel sign: an electric-like shock when light pressure
is applied to the tumor.
• Affection of motor or sensory nerve function.
Neurofibromas

30. Diagnosis:
• Characteristic appearance on MRI.
• A biopsy of the tumor enables positive diagnosis.
Treatment:
• Surgery is the treatment of choice
• Neurofibromas of the skull base  expanded
endonasal approach (EEA).

31. • Posterior fossa meningioma 10% of total
intracranial meningioma
• 50% involve the petroclival region.
• Skull base meningioma has a predilection for
female sex.
• Petroclival lesions usually present in 5th or 6th
decade.
• Meningioma the commonest lesion in the
petroclival region followed by chordoma.
• Chondrosarcoma and neurinoams are rare lesions
of the petroclival region.
Clival Meningioma

32. Castellano and Ruggiero classify posterior fossa
meningiomas (site of dural implant)
1- Cerebellar convexity.
2- Tentorium.
3- Posterior surface of the petrous bone.
4- Clivus.
5- Foramen magnum.

33. Desgeorges et al. divided the posterior surface
of petrous bone meningiomas according to the
exact site of implant in relation to the internal
auditory canal (IAC) and labyrinth into:
1-Anterior group
2- Median group (centered on the IAC),
3- Posterior group.

35. Sekhar et al. had classified clival meningiomas
based on volume and tumor size into medium,
large and giant subtypes.
Giant tumors were those having maximum
diameter more than 4.5 cm.

37. Yasargil et al. classify basal posterior fossa
meningiomas into five subgroups:
1. Clival.
2. Petroclival.
3. Sphenopetroclival.
4. Foramen magnum.
5. CPA meningiomas.

38. • One surgical group, which we call "petroclival
Meningiomas
1-The clival (medial origin).
2-Petroclival (origin at the petrous tip medial to the
V nerve).
3-Sphenopetroclival (origin at Meckel's Cave)
meningiomas.

40. Investigations:
• Computerized tomography (CT) scans
• Magnetic resonance imaging (MRI) with contrast
enhancement.
• Cerebral angiography visualization of carotid
and vertebral circulation.
• MR angiography and venography.

42. • The angiographic relationship of the basilar artery:
1-The basilar artery is usually pushed posteriorly and
to one side.
2-The posterior and superior cerebellar arteries usually
are frequently elevated on the same side of the
tumor.
• The venous phase of the angiogram demonstrating:
1-Presence and contribution of two transverse sigmoid
sinuses.
2-Delineating the course and dominance of the vein of
Labbé.

44. • Most common soft tissue sarcoma in children.
• Greek words rhabdo = rod shape, myo = muscle.
• Tumor arise from a primitive muscle cells
rhabdomyoblast.
• Histologic groups.
• Embryonal rhabdomyosarcoma (ERMS).
• Alveolar rhabdomyosarcoma (ARMS).
• Undifferentiated sarcoma (UDS).
Rhabdomyosarcoma

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

46. Thank You