spinal cord tumours luc van den hauwe et al. · spinal cord tumours luc van den hauwe et al. spinal...

Spinal cord tumours Luc van den Hauwe et al.

spinal cord tumours

L. van den Hauwe1,2,

J.W. Van Goethem2, C. Venstermans2, F. De Belder2, P.M. Parizel2

1AZ KLINA Brasschaat, 2Universitair Ziekenhuis Antwerpen-University of Antwerp, 3Hôpital Erasme-Free University of Brussels, Belgium

D. Balériaux3,

overview

•  introduction •  imaging •  spinal tumour classification

–  spinal cord tumours –  intradural extramedullary tumours –  epidural and primary spinal bone tumours

•  differential diagnosis - tumor mimics •  conclusions

overview

•  introduction •  imaging •  spinal cord tumours

–  astrocytoma –  ependymoma –  hemangioblastoma –  ganglioglioma –  other

•  differential diagnosis - tumour mimics •  conclusions

introduction •  classification of spinal tumours

–  spinal cord tumours –  intradural extramedullary tumours –  epidural and primary spinal bone tumours

•  tumours of the spinal cord are (very) rare •  may cause significant and longstanding morbidity •  clinical findings are most often non-specific

–  pain !!! –  motor and sensory deficits –  sphincteric disturbances, impotence

•  age

some statistics

•  intramedullary spinal cord tumours: 5% – within the spinal cord

•  intradural extramedullary tumours: 40% –  arise from leptomeninges and nerve roots

•  extradural tumours: 55% –  outside the thecal sac, in vertebral bodies and

epidural tissues

more statistics intramedullary spinal cord tumours •  90% are gliomas

–  ependymomas 60% –  astrocytomas 30%

•  in children –  astrocytomas

•  90% when younger than 10 •  60% in adolescents 2/3

–  gangliogliomas –  ependymomas are very (very) rare (NF2)


solitary vs multiple lesions number of lesions ?

solitary multiple where is the lesion ?

3. epidural space/osseous spine

2. intradural extramedullary

1. spinal cord ependymoma (NF-2) hemangioblastoma (VHL syndrome) M+

neurofibroma (NF-1) schwannoma (NF-2) meningioma (NF-2) M+ M+

lymphoma multiple myeloma hemangioma

more statistics

•  myxopapillary ependymomas compose 90% of filum terminale tumours

•  DD paraganglioma, giant schwannoma

introduction

•  role of imaging –  detection –  differentiating these tumours from other non-tumoural

pathology (e.g. demyelination) is the most important task for the neuroradiologist

–  determining the relationship of the tumour with the spinal cord and the extent of the tumour along with associated spinal cord changes such as oedema or syringomyelia is essential in therapy planning and monitoring

–  attempting to diagnose the type of tumor according to imaging criteria may be difficult in some cases but is less critical in patient management

imaging modalities

• MRI •  CT •  CR •  Myelography and myelo-CT •  DSA •  PET

Tomura et al. AJNR 2012

CR and CT

•  failed to reveal the true extent of intramedullary spinal neoplasms until gross expansion of the spinal canal had occurred

•  straightening of the spine •  progressive scoliosis •  widening of the spinal canal

myelography – myelo-CT

•  complete or partial block in the flow of intrathecal contrast material

•  cannot define the character of the spinal cord lesion •  in patients in whom MR imaging is contraindicated

Abul-Kasim et al. Neuroradiology 2008 Garcia-Morales et al. Neuroradiology Case of the Week, Case 34


MR imaging

•  the whole spinal cord must be visualized •  imaging protocol

–  sagittal T2, FS-T2 (STIR), T1 -/+ Gd –  axial T2, T1 + Gd –  additional sequences: GRE T2*, FLAIR, PRD, DTI, …

•  3 tenets: –  spinal cord expansion –  contrast enhancement –  associated cysts and pseudocysts

spinal cord expansion •  signal changes without cord expansion ?

–  consider other, nontumoural lesion •  demyelinating •  sarcoid •  vascular •  dAVF

•  in a series of 212 patients suspected of having intramedullary disease, nine (4%) had non-neoplastic lesions; none had imaging evidence of cord expansion

Lee et al. Neurosurgery 1998

contrast enhancement

•  almost all spinal cord tumours show some enhance-ment

–  the absence of enhancement does not exclude an intramedullary neoplasm in the presence of cord expansion

–  not all expansive, enhancing lesions are tumours!

contrast enhancement

Gd-enhanced MR imaging: •  accurate delineation of the solid enhancing portion

of the tumour –  tumour vs normal cord on T1-wi –  tumour vs oedema on T2-wi

Parizel et al. AJR 1989

contrast enhancement •  ependymoma:

–  intense, homogeneous, and sharply marginated focal enhancement

–  occupy the whole width of the spinal cord in the affected segment, centrifugal expansion

–  intratumoural cysts

•  astrocytoma: –  more patchy, irregular enhancement –  even low-grade lesions did enhance –  eccentrical location

Parizel et al. AJR 1989

overlap

associated cysts •  tumoural cysts

–  enhancing wall –  part of the tumour – must be resected – more frequent in astrocytomas

•  non-tumoural cysts –  polar or sattelite cysts, rostral or

caudal from the tumour –  non-enhancing wall –  reactive dilatation of the central

canal: syringomyelia


MR imaging •  although MR imaging is a powerful tool, it is not

perfect

•  in a series of 171 patients, MR imaging could correctly suggest the histologic diagnosis in only 70% of cases

•  in particular, the differentiation of ependymoma from astrocytoma was the most difficult

Brotchi et al. Neurosurgery 1991

DTI

Vargas et al. Neuroradiology 2008

ependymomas •  originate from cells bordering the central

ependymal canal: more centromedullary located •  young patients (mean age is 42), slight male

preponderance •  typically found in the C-spine (50%) •  associated (large) satellite cysts (60%) •  intense, mostly homogeneous enhancement •  well-defined borders •  ‘cap-sign’ (27%)

–  low SI areas at both sides of the tumor limits –  hemosiderin deposits (chronic hemorrhage)


myxopapillary ependymomas •  typical tumor < conus medullaris and filum terminale •  relatively common •  in children & young adults •  WHO, grade I •  slow growing tumours

–  long standing history of non specific low back pain exacerbated at night

–  may become very large before diagnosis is made •  hemorrhage may occur; sudden worsening of

clinical symptoms

LBP after trauma


schwannoma

astrocytomas •  hypointense on T1-wi •  hyperintense on T2-wi

•  pilocytic astrocytoma •  high-grade and GBM show patchy enhancement, and

are more heterogeneous, with necrotic/cystic areas and hemorrhage (T2*-wi)

•  ill-defined borders (infiltrative nature); cleavage plane is rarely found

hemangioblastomas

•  benign, usually richly vascularized tumours •  located superficially, in the sub-pial region,

postero-lateral •  small nodule with extensive edema and

cystic components •  more commonly in adults, with a peak

incidence in the fourth decade

Baker et al. AJR 2000 Lee et al. 2003


hemangioblastomas

•  can be solitary (80%) or multiple (20%), when associated with von Hippel-Lindau syndrome (VHLs)

•  DD with metastases of renal cell carcinoma

Wanebo et al. 2002 Takai et al. 2010

Case courtesy: D. Balériaux, Brussels, B

case courtesy: D. Balériaux, Brussels, B


10/09/2012 ganglioglioma •  rare tumours in adults (1–2% of all spinal cord

tumours) •  2nd most common intramedullary tumour in the

pediatric age group (15% of cases); mostly affect children between 1 and 5 years of age, as do PA

•  composed of a combination of neoplastic ganglion cells and glial elements

•  typically low-grade tumours (WHO grade 1-2); have a significant propensity for local recurrence, and the glial element may progress to higher grade tumours

Rossi et al. Neuroimag Clin N Am 2007 Smith et al. AJR 2012

ganglioglioma •  cervical and upper thoracic cord •  may extend over more than 8 vertebral segments •  typically eccentrically located •  imaging,

–  scoliosis and remodeling –  calcification may be seen on CT, is probably the single most

suggestive feature of gangliogliomas –  in the absence of gross calcification, the MR imaging is

nonspecific and does not allow differentiation from astrocytomas

–  young patient age, long tumour length, absence of edema, mixed signal intensity on T1-wi, patchy tumor enhancement

Rossi et al. Neuroimag Clin N Am 2007 Case courtesy: A. Rossi, Genova, Italy

Case courtesy: A. Rossi, Genova, Italy

spinal cord metastases

•  considered rare, accounting for only 5% of all intramedullary lesions

•  number is growing fast due to the longer survival of many cancer patients (improved chemotherapy, etc…).

•  less common than leptomeningeal metastases •  primary tumour: lung and breast carcinoma •  high sensitivity of MR imaging


M+ of breast carcinoma solitary M+ of renal cell carcinoma

spinal cord tumour mimics –  in one series of 212 patients undergoing surgery

for intramedullary spinal cord “tumours”, 4% of non-neoplastic lesions were found

–  a variety of lesions may mimic a spinal cord tumour

•  cavernous malformations (cavernomas) •  tumefactive demyelinating lesions in multiple

sclerosis (MS), neuromyelitis optica (NMO) and acute disseminating encephalomyelitis (ADEM)

•  acute transverse myelitis (ATM) •  spinal cord contusion and spinal cord infarction •  sarcoidosis •  vasculitis •  … MR imaging of the brain, follow-up examination

spinal cord tumour mimics

•  may enlarge the cord mildly

•  are less extensive

•  may or may not enhance!

•  DD: –  cavernous malformation (‘cavernoma’) –  inflammatory/demyelinating diseases –  infectious disease –  vascular/ischemic lesions

MR imaging of the brain, follow-up examination

cavernous malformation

•  aka cavernous angioma, or cavernoma •  represent 7%-10% of all intramedullary tumours •  most commonly involve the thoracic spine •  clinical:

–  may remain clinically silent for a long period of time –  acute and rapidly progressive neurological deficit may

occur due to bleeding

•  typical “black and white” or “popcorn” appearance due to areas of mixed signal intensity on both T1- and T2- or T2*-wi

•  look also in the brain!!! use SWI!!!


AJNR 2008; 29:154-158

tumefactive demyelinating lesions

•  MS •  NMO •  ADEM

neuromyelitis optica: NMO Devic disease


sarcoidosis Case courtesy: B. Goraj, Nijmegen, The Netherlands

dural AV-fistula

conclusion

•  classify spinal tumours •  spinal cord tumours are rare •  DD with non-tumoural conditions •  tumour vs associated changes

–  polar cysts –  oedema

•  final diagnosis: microscope

spinal cord tumours luc van den hauwe et al. · spinal cord tumours luc van den hauwe et al. spinal...

Documents