Morning case

CH. Nguyễn Xuân Phong

 Lâm sàng
• Bệnh nhân nữ 20 tuổi
• Lí do vào viện: tê bì, yếu 2 chân và khó đại tiểu tiện 2
tháng nay
• Khám:
o Tỉnh, không sốt
o Giảm phản xạ gân xương, giảm cảm giác từ D4
• Tiền sử: bình thường
Phẫu thuật – Mô bệnh học
Spinal Ependymoma
Anatomy Spinal Cord
Anatomy Spinal Cord
Anatomy Spinal Cord
Spinal Lesion
 Classification
SPINAL CORD TUMOR

Intra Dural Extra Dural

Metastatic
INTRAMEDULLARY EXTRAMEDULLARY Osteoid
Ependymoma Meningioma Nerve - osteoma
Astrocytoma sheath tumor Osteoblastoma
Hemangioblastoma Vascular tumor ABC
Oligodendroglioma Myxopapillary - Plasmacytoma / Myeloma
Ependymoma Chordoma Chondrosarcoma
Lipoma/Dermoid/E Osteosarcoma
pidermoid Ewing’s sarcoma
Cauda equina and
filum terminale Epidural hemangioma
Myxopapillary - Lipoma
ependymoma Extradural meningioma
Spinal nerve sheath Nerve sheath tumor
tumor Lymphoma
 Spinal Lesion

INTRAMEDULLARY IDEM EXTRADURAL

 Primary Spinal Tumor
Pediatric Adult

20% 20%
Intramedullary Intramedullary
40%
IDEM IDEM
50%
Extradural Extradural
60%
10%

Location
 Approach Spinal Tumor
• MRI is the modality of choice for the assessment of
lesions within the spinal canal
• CT Myelography is done for patients whom an MRI is
contraindicated
• CT: assess the osseous structures
• Angiography is useful for patients who have vascular
lesions: vascular malformations, vascular tumors
• Ultrasound except in the infant, does not have a role in
diagnosis
 Approach Spinal Tumor
• MRI protocol
o T1 sagittal and axial
o T2 sagittal and axial
o T1 C+ sagittal and axial, with one or both planes fat-
saturated (especially when there is foraminal extension or
when there is concern for an extradural process)
• Additional sequences
o CSF flow studies
o gradient echo sequences (for blood products/calcification)
o high resolution
o diffusion weighted imaging
o tractography
 What clinicians want to know
Size
signal intensity on T1 and T2 weighted images +/- appearance on other sequences
presence and pattern of contrast enhancement
location
intramedullary or intradural extramedullary
spinal cord segment(s) affected
for intramedullary tumors, location within the spinal cord (central vs eccentric)
exophytic component
single vs multiple lesions
presence of: hemorrhage, calcification, necrosis
associated cysts (tumoral, non-tumoral) or syringomyelia
surrounding abnormalities
peritumoural edema
prominent flow voids
leptomeningeal enhancement
bony changes (though these are relatively rare) - widening of the spinal canal,
scalloping of the posterior vertebral bodies, neural exit foraminal enlargement,
scoliosis
compression of the spinal cord or displacement of nerve roots
 Location
• An extramedullary mass
will push the cord away
from it
• Intramedullary lesion will
expand and thin the
cord around it
Claw sign
 Location
• Intradural extramedullary lesions may be related to
nerve roots and may extend into the foramen (e.g.
schwannomas and neurofibromas) - dumbbell lesions
• or they may have a broad dural attachment (e.g.
meningiomas) – dural tail sign
• Involve vertebral body, epidural – means extradural
tumor
 Signal characteristics
T1W T2W Contrast Blood products
enhancement
- Most: Isointense/ - Most: Hyperintense - Most: contrast - Signal depend on
hypointense - Hypointense: collagen enhancement age of blood products
- Hypointense: fiber, hemosiderin rim, - Hemorrhage:
hemorrhage, cyst calcification ependymoma,
formation, fibrous - Peritumoral edema: hemangioblastoma,
tissue and hyperintense surround paraganglioma or a
calcification - Vascular flow void: hemorrhagic
- Hyperintense: vascular tumor metastasis
hemorrhage, fat,
mucinous
Intramedullary tumor
2% to 4% of all the central nervous system neoplasms
20–25% of all intra spinal tumors.

4%

14%

55%
27%

ASTROCYTOMA EPENDYMOMA HEMANGIOBLASTOMA OTHERS

 Spinal Cord Ependymoma (SCE)
Epidemiology:
• SCE is a rare tumor that is most commonly low-grade,
Arises from ependymal cells of central canal
• In US, 1000 – 2000 adults spinal/ intracranial
ependymoma each year
• ~ 25% intramedullary spinal cord tumor, 2% primary CNS
malignancies
• 40-60% in adults, 30% in children
• Mean age: 30 – 40 years
• Gender: male/female: 1/1
• Slow growing
• Related NF2, loss of chromosome 22q
 Histological
WHO 2016
Grade I Grade II Grade III

Classic
Cellular
Myxopapillary
Papillary Anaplastic
Subependymoma
Clear cell
Tanycytic

• The grades differ in their most likely locations within the spinal cord,
ease of resection, and tendency to recur
• Most WHO grade II
• Tend to expand the cord symmetrically and focally (c.f astrocytomas
• more diffuse and eccentrically located)
• Rarely change growth characteristics and metastasize
 Grade II spinal ependymoma
• Commonly in cervical, thoracic, rarely lumbar cord
• Classic:
o Pseudorosettes 80%, True rosettes 10% - specific to ependymomas –
consist of cells arranged similarly around a central lumen
• Cellular:
o hypercellularity , high nuclear-to-cytoplasm ratio, few rosettes
o lacks the microvascular proliferation, cellular pleomorphism
• Clear cell:
o perinuclear halos, pseudorosettes
• Papillary:
o the arrangement of neoplastic cells around a fibrovascular core
• Tanycytic: least common grade II subtype
o similar to pilocytic astrocyte
Grade II spinal ependymoma

a,b,c: high cellularity,

pseudorosettes, true
rosettes
d: Ependymoma with
clear cells
e:Papillary
 Grade III – I spinal ependymoma
• Anaplastic ependymoma (Grade III):
o mitoses, endothelial proliferation, and nuclear pleomorphism
o tend to infiltrate surrounding tissue and thus less often allow gross
total resection (GTR)
• Grade I:
• Subependymoma: rarely in spinal cord
o tend towards peripheral location within the cord, other subtypes
are typically central
o microcystic spaces and grouped cells on a dense fibrillary
background
• Myxopapillary: ¼ SCE, around the conus medullaris, often
involving the cauda equina or filum terminale
o appearance of loosely structured cells with intervening pools of
mucin, with markedly hyalinized blood vessels
f, g, h: Myxopapillary, myxoid spaces and hyalinized
vessels
i, j: Subependymoma, microcystic, clusters of cells on a
fibrillary background
k, l: Anaplastic ependymoma, high cellularity and lack of
rosettes, cellular atypia, nuclear pleomorphism, and
mitoses
 Clinical presentation
• Present with nonspecific symptoms progressing over
years prior to diagnosis
• Common symptoms: back pain, spasticity in the
lower extremities, gait ataxia, sensory loss, and
paresthesias
• Cervical tumors can present upper or lower
extremity symptoms if they affect the corticospinal
tract or dorsal columns
• Lumbar tumors may cause incontinence, radicular
back and leg pain, and even asymmetric weakness
if the tumor causes significant mass effect in more
advanced disease
 Radiographic features
Plain radiograph
• scoliosis
• spinal canal
widening
• vertebral body
scalloping
• pedicle erosion
• laminar thinning

L2 ependymoma causing
focal posterior vertebral
body scalloping
 CT
• Non-specific canal widening
• provides little diagnostic utility except to identify
areas ofcalcification
• Iso to slightly hyper-attenuating compared with
normal spinal cord
• Intense enhancement with iodinated contrast
• Large lesions may cause scalloping of the posterior
vertebral bodies and neural exit foraminal
enlargement
 MRI
• the best modality to assess suspected spinal cord
neoplasms
• Perfusion MRI and MR spectroscopy are not
typically useful due to the small diameter of the
spinal canal and the movement of the spinal cord
with arterial blood flow
• PET ependymomas typically appear hypometabolic
due to their low cellular density and slow growth
 MRI
• Cord expansion is a key finding to identify intramedullary
tumors; when the cord is normal in size non-neoplastic
processes such as a demyelinating disease should also be
considered
• Centrally located, well circumscribed, and non - infiltrative
• syringohydromyelia 9 -50% (hydromyelia + syringomyelia)
• calcification is uncommon
• 4 – 5 vertebral body segments
• T1W: hypo - isointense/ T2W: hyperintense/ Enhance contrast
heterogenous signal: cystic change, hemorrhage, necrosis
• Usually enhance more homogeneously than astrocytomas
with sharply defined poles and are capped superiorly by a
cyst, inferior cyst is less common.
• hemosiderin cap sign: hypointense hemosiderin rim on T2, 20 –
33% ependymoma. The cap sign is suggestive
 MRI
Myxopapillary ependymomas
• conus medullaris
• heterogeneous lesion: isointense cellular +
hyperintense mucin or hemorrhage
Anaplastic ependymomas
• T1 isointense/ T2 iso- or hyperintense/ variable
contrast enhancement
• Infiltration into surrounding tissue
Imaging alone cannot reliably distinguish histologic
grade or exclude other diagnoses. Imaging is
therefore most useful for preoperative planning before
tissue diagnosis
 Male 45 years old,
Gradual upper limb
weakness

Ependymoma Grade II

A mass ( * ) located centrally within the cord (note displaced 'normal' cord around
the lesion (green arrows)) is of high T2 signal and demonstrates moderate contrast
enhancement. At either end multiple cystic regions are demonstrated (blue arrows)
most of which do not have any solid enhancing component and thus probably
represent tumor syrinx rather than intra-tumoral cysts. Best seen inferiorly is a region of
signal drop out (yellow arrow) which probably represents blood product (a poorly
formed hemosiderin cap)
Female 65 years, 18 months of neck pain radiating down to right arm,
Vividly enhancing intramedullary mass ( * ) is located centrally within
the cord (note the cord evenly splayed around mass on axial imaging
(blue arrows) ) with a prominent inferior hemosiderin cap (yellow arrow)
Ependymoma Grade II
 Intramedullary mass: T11 – L4/5
Isointense T1, hyperintense T2
Avid enhancement
Scalloping of the posterior
lumbar vertebral bodies L2-L4,
widening the lumbar neural
exit foramen bilaterally from
T12-L3
Male 25 years, New onset right sided
parasthesia in lumbosacral distribution. No evidence of hemorrhage
Reduced anal tone into or surrounding the tumor
Ependymoma grade II
Sagittal T1-weighted and T2-weighted MR images of the lumbar spine
demonstrate an enhancing intradural intramedullary mass extending from T10
to the conus medullaris. Sagittal T1 pre- and postcontrast MR images reveal
enhancement throughout the subarachnoid space of the lumbar and thoracic
spine extending superiorly to the level of the cervical spine (not shown) and
into the basilar cisterns - Anaplastic spinal cord ependymoma
 Treatment
Surgical
• The aim: gross total resection (GTR) and protect healthy tissue
• depends on tumor location, size, histology, and the presence
of a capsule or syrinx (providing a plane of resection)
• GTR rate is high 84-93% due to rarely infiltrate the spinal cord
• GTR is not achieved in most patients due to most
ependymomas being located in areas that, if resected, would
decrease neurological function
Radiotherapy
• recommend If full resection is not possible due to tumor
location or anatomy
Chemotherapy
• The role of chemotherapy for treatment of SCE is even less
clear than that of radiation
 Prognosis
• Location and genetic markers may more accurate predictors
of prognosis than histologic grade
• Progression-free survival (PFS) and overall survival (OS) for
grade I or grade II better than grade III
• grade II: PFS 80-90%/ 5-10 years
• The recurrence rate: Recurrence is rare following complete
excision, depend on the rate of GTR
Grade II < Grade I < Grade III
• Although metastatic spread is rare, the most common sites for
metastases include the retroperitoneum and lymph nodes
 Differential diagnosis
Spinal Astrocytoma
• Entire spinal cord (holocord presentation) common
in children (up to 60%) quite rare in adults
• Usually grey tumor, more infiltrative , often poor
plane
• Arise from the cord parenchyma not from the
central canal, are usually eccentric within the cord
• T1: isointense to hypointense
• T2: hyperintense
• T1 C+ (Gd) vast majority enhance, usually patchy
enhancement pattern
 Male 25 years, 2 weeks of
increasing lower limb
numbness and weakness

A mass with high T2 signal ( * ) is located eccentrically within the cord,

with 'normal' appearing cord displaced posteriorly and to the left (blue
arrows). The mass demonstrates heterogeneous contrast enhancement
(red arrows) - Spinal cord astrocytoma
 D/Dx
Spinal cavernous malformation
• are vascular malformations that occur within the
spinal cord.
• Site: ½ thoracic cord – second cervical cord
• Complete hemosiderin ring heterogeneous signal
intensity on T1 and T2 – popcorn sign
• Gradient echo (GE): hypointense hemosiderin
• T1 C+ (Gd): minimal enhancement
Female 65 years, Rapid
onset upper and lower
limb symptoms

Complete hemosiderin ring (red arrow) demonstrates blooming on

gradient echo sequences (yellow arrow). Intrinsic high T1 signal (blue
arrow) is visible on both pre and post contrast sequences and should
bot be mistaken for contrast enhancement
 D/Dx
Spinal hemangioblastoma
• 3rd intramedullary spinal neoplasm, benign vascular lesions
• thoracic cord (50%), cervical cord (40%), most solitary; Multiple -
Von Hippel-Lindau syndrome
• T1
• variable relative to the normal spinal cord
• hypo- to isointense most common, and difficult to identify
• hyperintense (25%)
• T2
• iso- to hyperintense
• focal flow voids especially in larger lesions
• surrounding edema and associated syrinx are usually seen
• hemosiderin capping may be present
• T1 C+ (Gd)
• the tumor nodule enhances vividly
Male 35 years,
Progressive leg
weakness and sensory
changes
An intramedullary nodule (yellow arrow) which demonstrates very bright
contrast enhancement and is associated with holocord syrinx ( * ) extending
above and bellow the lesion from the medulla (red arrow) to the tip of the
conus (green arrow)
Numerous enlarged flow voids are noted both within the cord (orange arrow)
and on the surface of the cord (blue arrows)
 Conclusion
• The approach of spinal lesion: intramedullary - intradural
extramedullary – extradural
• MRI is the modality of choice for the assessment of
lesions within the spinal canal
• Spinal ependymoma: intramedullary neoplasm most
commonly in adult, The WHO subtypes are classified into
three grades based on degree of malignancy, most
grade II
• Clinical symptoms’s nonspecific
• MRI features: cord expansion, central intramedullary,
well – circumscribed, non – infiltrative and hemosiderin
cap sign are suggestive
• Treatment outcome and Prognosis are well if gross total
resection (GTR)
Thank you for your attention