Spinal cord tumours Luc van den Hauwe et al.

Transcription

1 overview spinal cord tumours L. van den Hauwe 1,2, D. Balériaux 3, J.W. Van Goethem 2, C. Venstermans 2, F. De Belder 2, P.M. Parizel 2 introduction imaging spinal tumour classification spinal cord tumours intradural extramedullary tumours epidural and primary spinal bone tumours differential diagnosis - tumor mimics conclusions 1 AZ KLINA Brasschaat, 2 Universitair Ziekenhuis Antwerpen-University of Antwerp, 3 Hôpital Erasme-Free University of Brussels, Belgium 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)

2 solitary vs multiple lesions multiple 1. spinal cord ependymoma (NF-2) hemangioblastoma (VHL syndrome) M + number of lesions? where is the lesion? solitary 2. intradural extramedullary neurofibroma (NF-1) schwannoma (NF-2) 3. epidural space/osseous spine 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 MRI imaging modalities 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

3 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 nonneoplastic lesions; none had imaging evidence of cord expansion Lee et al. Neurosurgery 1998 contrast enhancement almost all spinal cord tumours show some enhancement 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

4 MR imaging DTI 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 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)

5 Spinal cord tumours 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

6 schwannoma hypointense on T1-wi hyperintense on T2-wi astrocytomas 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

7 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 Takai et al Case courtesy: D. Balériaux, Brussels, B case courtesy: D. Balériaux, Brussels, B

8 10/09/2012 ganglioglioma rare tumours in adults (1 2% of all spinal cord tumours) 2 nd 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 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 Case courtesy: A. Rossi, Genova, Italy

9 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!!!

10 MS NMO ADEM tumefactive demyelinating lesions AJNR 2008; 29: neuromyelitis optica: NMO Devic disease

11 Spinal cord tumours Case courtesy: B. Goraj, Nijmegen, The Netherlands conclusion classify spinal tumours spinal cord tumours are rare DD with non-tumoural conditions tumour vs associated changes polar cysts oedema final diagnosis: microscope dural AV-fistula sarcoidosis