148 Skull tumour & GB 21.4 skull tumors

Greenberg,Handbook of neurosurgery 21.4
Skull Tumors
Youmans,Neurological surgery Chapter 148
Skull Tumors
Osborn,Expert ddx in brain&spine
Skull tumors

Outline
 Osteoma
 Hemangioma
 Dermoid and
epidermoid tumors
 Chondroma
(osteochondroma)
 Meningioma
 Aneurysmal bone
cyst
Malignant tumors
Bone metastases to
the skull
Chondrosarcoma
Osteogenic sarcoma
Fibrosarcoma
Benign tumors
Non-neoplastic
skull lesion
 Paget's disease
of the skull
 Langerhans Cell
Histiocytosis
(Histiocytosis
X)
 Fibrous
dysplasia
 Hyperostosis
frontalis interna
 Sinus pericranii

Benign tumors
 Osteoma
 Hemangioma
 Dermoid and epidermoid tumors
 Chondroma(osteochondroma)
 Meningioma
 Aneurysmal bone cyst
Greenberg,Handbook of neurosurgery 21.4 Skull

Osteoma
 The most common primary bone tumor of the
calvaria
 Benign,slow-growing lesions
 Occur commonly in the cranial vault, mastoid and
paranasal
air sinuses, and the mandible
• Common in females, highest incidence is in 6th
decade
• Pathology : Consists of osteoid tissue within
osteoblastic tissue, surrounded by reactive bone

Osteoma
 Skull x-ray
 Round, sclerotic, well demarcated, homogeneous
dense projection.
 Usually arise from outer table of skull (inner table less
common).
 May be compact or spongy(spongy osteoma may be
radiolucent)
 Unlike meningiomas, diploe are preserved and vascular
channels are not increased.
 Treatment
 Asymptomatic lesions may simply be followed.
 Surgery may be considered for cosmetic reasons, or if
pressure on adjacent tissues produces discomfort.

 7% of skull tumors
 Two types: cavernous (most common) and
capillary(rare)
 X-ray
 Characteristically shows a circular lucency with
honeycomb or trabecular pattern (50% of cases)
or radial trabeculations producing a sunburst
pattern (11% of cases)
 Sclerotic margins are evident in only ... 33%.
 CT: hypodense lesion with sclerotic spaced
trabeculations. Nonenhancing
 Bone scan: typically hot.
Hemangioma

Hemangioma
 Treatment
 Accessible lesions may be cured by en bloc
excision or curettage.
 The gross appearances of a hard, blue-domed
mass beneath the pericranium.
 Radiation may be considered for inaccessible
tumors

 Rare
 Usually midline
 Arise within the diploe and expand both inner and
outer tables
 These benign lesions may involve underlying
dural venous structures or brain
Dermoid and epidermoid tumor

Dermoid and epidermoid tumor
 X-ray
 Osteolytic lesions have well-defined, sclerotic
margins
 CT : hypodense (keratin contains fats), non-
enhancing
 MRI : like CSF, they are low intensity on T1 WI and
high signal on T2WI, but unlike CSF they are high
signal on DWI MRI
 Treatment
 Surgical
 Search must be made for a tract leading to the
intracranial cavity which must be followed if found

Chondroma(Osteochondroma)
 Arise form ectopic hyaline cartilaginous rest
trapped within suture line
 Common : skull base,anterior to pon ,sphenoid
bone, boardering of foramen lacerum
 CT
 Well-demarcated, off midline, lobulated, contrast-
enhancing, dense mass contiginous with the
underlying bone and marked by popcorn
calcification
 MRI
 Thin cartilaginous cap overlying the dense osseous
core

Chondroma(Osteochondroma)
 Treatment
 Complete resection,including cartilaginous capsule

Meningioma
 Intraosseous(intradiploic) meningioma are
uncommon
 Primary intraosseous meningiomas do not involve
the inner or outer tables of the skull or the dura
 “ectopic meningiomas” or “primary extradural
meningiomas”
 Arise from rests of arachnoid cap cells trapped
within sutures at birth and during molding of
the head

Meningioma
 Plain radiograph and CT
 Hyperostosis(some are lytic or both lytic and
sclerotic)
 Expansion of the diploë, with the inner and outer
tables of the skull separated and thinned over the
biconvex mass
 MRI
 TW1 : hypointense, TW2 : hyperintense
 Homogeneous contrast enhancement on both CT
and MRI
 Treatment
 En bloc resection with marginYoumans,Neurological surgery Chapter 148

Aneurysmal bone cyst
 Rare
 Secondary to trauma to be caused by a
circulatory disturbance resulting in venous
hypertension and venous pooling within the bone
 A typical finding : local swelling and tenderness of
a few month duration
 Gross : vascular channel that give them spongy-
like appearance
 Histologic : communicating pools of venous blood
without endothelium in a thin matrix of fibro-
osseous strands along with frequent
multinucleate giant cells.

Aneurysmal bone cyst
 Imaging
 Lytic
 Loculated lesion with fluid-fluid levels caused by
layering of blood products within internal cavities
 The lesion typically begins in the diploë, and
expansion or “blowout” of the inner and outer
cortices
 Treatment
 Gross total resection

Malignant tumors
 Bone metastases to the skull
 1. prostate
 2. breast
 3. lung
 4. kidney
 5. thyroid
 6. lymphoma
 7. multiple myeloma/plasmacytoma
 Chondrosarcoma
 Osteogenic sarcoma
 Fibrosarcoma

Bone metastasis to skull
 Most common : breast, lung, and prostate
 Uncommon : renal and thyroid carcinoma
 Cranila vault more than skull base
 Radiographic
 Lytic lesion except prostate cause sclerotic lesion
 MRI : T1W : hypointense with variable T2W
characteristic and variable contrast enhancement
 Radionuclide bone scanning : sensitive method for
detecting skull metastasis

 CSF
 Helpful in suspected metastasis to the skull base
 If CSF is normal, metastasis is more likely than
infection to be the cause of multiple cranial
neuropathies
 A CSF study may also reveal the presence of
meningeal carcinomatosis

 Treatment
 Patients with skull metastases are frequently at an
advanced stage of their primary disease and often
asymptomatic : Surgery may not be required for
diagnostic or even therapeutic purposes
 Symptomatic or palpable skull mass may be the
first sign of the underlying cancer : surgical
resection may be helpful or FNA in multiple or too
indolent to need resection

Chondrosarcoma
 Rare,malignant neoplasm of cartilage
 Found away from midline with cranial
neuropathies(particularly abducen neuropathy)
 Skull : painless expanding mass
 Subtype
 Myxoid(conventional low grade) most common
 Dedifferentiated
 Mesenchymal
 Pathology
 hypercellular with hyperchromatic and
pleomorphic nuclei

Chondrosarcoma
 CT
 Calcifications and ossifications within the tumor
mass
 MRI
 Lobulated lesions that appear isointense to
hypointense on T1-weighted images and
hyperintense on T2-weighted images
 Show heterogeneous contrast enhancement
 Treatment
 Surgical resection with adjuvant therapy

Osteogenic sarcoma
 Most common malignanat tumor in bone but it is
relatively rare in skull
 Cranial vault than skull base
 Secondary to radiation
 Patients with tumors attaining large size or
involving the skull base may complain of
local tenderness, headaches, proptosis,
ophthalmoplegia, facial weakness,
decreasedhearing ability, or tinnitus
 The alkaline phosphatase level may be a useful
diagnostic test

Osteogenic sarcoma
 Pathology
 Sarcomatous spindle cell stroma with an associated
osseous component
 CT
 Bony destruction, cortical expansion, and a
“sunburst” periosteal reaction
 Demonstrate areas of irregular calcification, as
well as low-attenuation areas representing
necrosis
 MRI
 heterogeneous signal on both T1- and T2-weighted
 Treatment
 Gross total resectionYoumans,Neurological surgery Chapter 148

Fibrosarcoma
 Rare
 May arise from degeneration of a preexisting
lesion such as a fibroma from Paget’s disease
or it may occur after radiation treatment
 Typically indolent, asymptomatic masses
 Type : low grade (most differentiated), moderately
differentiated, and poorly differentiated
 Pathology
 interlaced bundles of spindle cells and collagen
fibers in a “herringbone” pattern

Fibrosarcoma
 Radiographic
 Lytic lesion with cortical destruction or expansion,
or both, and soft tissue extension
 Radiolucent
 Treatment
 En bloc resection

Non-neoplastic skull lesion
 Paget's disease of the skull
 Langerhans Cell Histiocytosis(Histiocytosis X)
 Fibrous dysplasia
 Hyperostosis frontalis interna
 Sinus pericranii

Paget’s disease of the skull
(Osteitis Deformans)
 Disorder characterized by the uncoupling of
bone formation and resorption, with resultant
bone thickening and weakening
 Clinical
 Headache, cranial neuropathy, EDH fron trauma
 CT
 Areas of sclerosis mix with the preexisting lytic
areas, and a mottled “cotton wool” appearance
develops
 MRI
 mixed intensity

 The most common types of sarcomatous
degeneration are to osteosarcoma (50% to 60%
of instances) and fibrosarcoma (20% to 25% of
instances)
 Treatment
 Medical : bisphosphonates or calcitonin is the first
line
Paget’s disease Fibrous dysplasia
Symmetrical Unilateral
Thickening of inner cortex Cortical destruction
Cotton wool Groud grass apperance
Older thane fibrous
dysplasia
Involve orbit,paranasal
sinus, sphenoid bone

 Osborn,Expert ddx in

Fibrous dysplasia
 Benign condition in which normal bone is
replaced by fibrous connective tissue
 Most common : rib or craniofacial bone esp
maxilla
 Pattern
 Monostotic: most common
 Polyostotic: 25% with this form have> 50% of the
skeleton involved with associated fractures and
skeletal deformities
 as part of McCune-Albright syndrome
 Autonomous endocrine hormone excess, such as
in precocious puberty
 Polyostotic fibrous dysplasiaGreenberg,Handbook of neurosurgery 21.4 Skull

Fibrous dysplasia
 Clinical
 incidental finding
 local pain and swelling or deformity
 may predispose to pathologic fractures when they
occur in long bones
 cranial nerve involvement
 seizures
 serum alkaline phosphatase is elevated in about
33%, calcium levels are normal
 darkened hair pigmentation overlying skull lesions
 spontaneous scalp hemorrhages
 rarely associated with Cushing's syndrome

Fibrous dysplasia
 Groud glass appearance on x-ray due to the thin
spicules of woven bone
 Treatment
 No cure for fibrous dysplasia
 Local procedures (mostly orthopedic) are used for
deformities or bone pain that is refractory to other
treatment
 Neurosurgical involvement may be required for skull
lesions producing refractory pain or neurologic
symptoms
 Calcitonin may be used for widespread lesions with
bone pain and/or high serum alkaline phosphataseGreenberg,Handbook of neurosurgery 21.4 Skull

Fibrous dysplasia

Langerhans cell Histiocytosis
(Histiocytosis X)
 Group of related disorders of abnormal
uncontrolled histiocyte proliferation:
 Eosinophilic granuloma
 Hand-Schüller-Christian disease
 Abt-Letterer-Siwe disease
 Hashimoto-Pritzker disease
 Predominantly occurs in children and
adolescents, with the mean age at incidence
being 12 years
 Clinical
 Solitary mass with localized pain

(Histiocytosis X)
 Pathology
 Clonal proliferation of S-100–positive histiocytic
cells in clusters mixed with inflammatory cells,
which are predominantly eosinophils.
 CD1a antigen
 EM : racquet-shaped Birbeck’s granules
 X-ray
 Classic punched-out lytic border without a sclerotic
boarder
 Bevel appearance : bony destruction outer table
than inner table
 Extraosseous contrast-enhancing soft tissue massYoumans,Neurological surgery Chapter 148

(Histiocytosis X)
 Treatment
 surgery
 radiation therapy
 chemotherapy
 immunotherapy

(Histiocytosis X)

Hyperostosis Frontalis Interna
 Benign irregular nodular thickening of the inner
table of the frontal bone that is almost always
bilateral
 More common in women
 Clinical
 Incidental finding, headache, cranial defect
 X-ray
 Thickening of the frontal bone with characteristic
sparing of the midline
 Treatment
 Removal of thickening bone

 Metabolic craniopathy
 Morgagni's syndrome (AKA Morgagni-Stewart-
Morel syndrome)
 headache, obesity, virilism and neuropsychiatric disorders
(including mental retardation)
 Endocrinologic abnormalities
 Acromegaly
 hyperprolactinemia
 Metabolic
 hyperphosphatemia
 obesity
 diffuse idiopathic skeletal hyperostosis (DISH
 Diffuse idiopathic skeletal hyperostosis (DISH)

Sinus pericranii
 Abnormal collection of veins adherent to the
outer table of the skull in communication with
the intracranial venous sinuses via dilated diploic
and emissary veins of the skull
 Cause : congenital, spontaneous, trauma
 Located in midline
 The lesion is nonpulsatile, expands with a
Valsalva maneuver or while the patient is supine,
and decreases with head elevation or direct
compression
 Histology : a tangle of nonmuscular venous
vessel

Sinus pericranii
 X-ray
 Radiolucent due to skull defect
 CT
 Extraosseous soft tissue mass, as well as an
emissary channel through the skull, with smooth
erosion of the bone’s outer table
 CT with contrast show venous filling
 MRI
 Hypointense on T1 and hyperintense on T2
 Treatment
 Progressive or symptomatic lesion
 Surgical resection

148 Skull tumour & GB 21.4 skull tumors

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148 Skull tumour & GB 21.4 skull tumors