Pediatric Spine and Spinal Cord Tumors: Disclosure A Neglected Area? • I have nothing to disclose • No relevant financial relations interfering with ECPNR 2020 my presentation Rome • Several cases shared by Thierry Huisman, MD and Aylin Tekes, MD Bruno P Soares, MD Associate Professor of Radiology Division Chief of Neuroradiology University of Vermont Medical Center Burlington, VT Outline Epidemiology • Epidemiology • Pediatric spinal cord neoplasms • Clinical presentation • 4-10% primary CNS tumors • Compartmental approach to diagnosis • 25% of all spinal tumors • Most prevalent between 1 and 5 years • Imaging findings • Differential diagnosis Epidemiology Approach to Diagnosis: Location • Intramedullary • Intramedullary Tumors • Glial: Astrocytoma, Ependymoma • Astrocytoma (pilocytic > diffuse): 60% • Neuroglial or Non-glial: Ganglioglioma, Hemangioblastoma • Ependymoma (assoc. with NF2): 15-30% • Intradural extramedullary • Ganglioglioma: up to 15% • Nerve sheath tumors • Meningioma • Paraganglioma • Mixopapillary ependymoma • Embryonal tumors •ATRT • Leptomeningeal metastasis Nonspecific Clinical Symptoms Clinical Presentation • Progressive motor weakness • Progressive scoliosis • Gait disturbance / falls • Paraspinal muscle spasmp • 25-30% presentp withh back pain!p • Up to 15% present with raised ICP Incorrect radiological work-up: Delay between onset of clinical symptoms and diagnosis Clinical Presentation: Back Pain MRI of Spinal Cord Tumors 1.. Cord expansion is a • Spinal pain (70%) major feature of T2-FSE • Dull and aching pain, localized to bone segments intramedullary adjacent to tumor neoplasms • Distension of thecal sac by enlarged cord • Root pain • Nerve root compression If absent or minimal, • Tract pain consider non-neoplastic • Vague, burning pain with paresthesias etiologies: MS, ADEM, • Infiltration of spinothalamic tracts sarcoidosis, ischemia... Astrocytoma ADEM Epstein F, Epstein N. Pediatric neurosurgery 1982: 529-540 MRI of Spinal Cord Tumors MRI of Spinal Cord Tumors 2. Vast majority of tumors showw enhancement 3.3. Cysts are frequently seen in spinal tumors Nontumoral cysts: • At poles of solid tumor • Reactive dilatation of central canal • Fluid produced by tumor • No enhancement Tumoral cysts: • Peripheral enhancement Nontumoral cyst Tumoral cyst 12Y, M, Low grade glioma Spinal Astrocytoma Spinal Astrocytoma • Most common cord tumor in children: 60% Eccentric location • 50% are cervicothoracic Asymmetric expansion • Small number of segments • Most are low grade • High grade: (?) diffuse midline glioma H3K27M • MRI differentiation of high and low grade cord tumors is often difficult Pilocyticc Astrocytoma Pilocyticc Astrocytoma T2-FSE T1-SE T1-SE T1-SE +C Astrocytoma: Exophytic Growth Astrocytomama: Loww Grade Excellent postoperative recovery Astrocytomama: H3K27M (?) Astrocytomaa in NF1 High-grade spinal cord tumors may invade the medulla Spinal Ependymoma Ependymoma • Most frequently in cervical cord • Centrally located • Compression of adjacent spinal cord tissue rather than infiltration • Tends to bleed • Polar cysts common, tumor cysts less common Central location, • Strong enhancement peripheral white matter compressed Ependymoma with tumor cyst and intratumoral hemorrhage Ependymomaa: Cap Sign DTI for lesion characterization • Astrocytoma • “Cap sign“ in 20%: rim of • Eccentric location, hemosiderin at tumor infiltrating in between of fibers, low ADC values poles due to hemorrhage • Ependymoma • Central, grow outwards displacing fibers, low ADC values •ADEM • Fibers intact, increased ADC values Source: radiopaedia.org Thurnher M, Law M. Magn Reson Imaging Clin N Am 2009 Myxopapillaryy Ependymoma Myxopapillaryy Ependymoma • 13% of spinal ependymomas • More common in male children T2-FSE T1-SE +C • Tip of conus and filum terminale • Arise from ependymal glia of filum • Mucin-producing, lobulated tumor • Lower back pain, leg weakness and sphincter dysfunction Myxopapillaryy Ependymoma Childrenn Are Notot Small Adults • Higher ratio of malignant astrocytomas • Up to 50% of pediatric cord glial neoplasms • Lower ratio of ependymoma • 15-30% of spinal cord neoplasms • Nearly half of intramedullary tumors in adults are ependymoma • Mixopapillary ependymoma (WHO I) proportionally less common in children Ganglioglioma Ganglioglioma: Calcification ¾Up 15% of pediatric cord tumors ¾1 to 5 years of age ¾Local recurrence ¾Cysts are common ¾Ca++: uncommon but characteristic Rossi A. et al, Neuroimag Clin N Am 2007 Diffuse Leptomeningeal Hemangioblastoma Glioneuronal Tumor • 1-7% of all spinal cord neoplasms • Intramedullary, with extension to intra- and extradural space • Slow growing • Mostly solitary • Multiple in von Hippel-Lindau Courtesy Felice D‘Arco, London Hemangioblastoma Hemangioblastoma • Highly vascular tumor T2-FSE • Prominent dilated feeding arteries and draining pial veins • May present with subarachnoid hemorrhagemoorrrhaagee or hematomyelia • Associated syrinx is common • Nodular, intense enhancement • Edema and cap sign may be seen Hemangioblastoma Metastasis T2-FSE T1-SET1 SE T1-SET1 SE +C • Intramedullary metastases are very rare • Hematogenous spread • Direct extension from leptomeninges • Extramedullary, intradural more frequent • CSF seeding by intracranial neoplasms • Medulloblastoma, ependymoma, high grade glioma, germ cell tumor, choroid plexus carcinoma, pineoblastoma Pearl: Vasogenic edema and low-grade tumors do not extend to the medulla Metastasis Drop Metastases: Nodular Pattern T1-SE T1-SE +C • Intramedullary metastasis • Poor prognosis • Extensive edema • CSF seeding ("drop mets") • Nodular and irregular thickening of thecal sac • Coating of surface of spinal cord • Nerve root thickening Drop Metastases: Sugar Coating Drop Metastases: SSFP T1-SE +C T2 SSFP T1 +C T2 T1 +C Soares BP et al, Neurographics 2014 Approach to Diagnosis: Location Sympathetic Tumors • Extradural • Ganglioneuroma • Nerve sheath tumors • Neuroblastoma / ganglioneuroma • Ganglioneuroblastoma • Osseous • Neuroblastoma • Osteoid osteoma / osteoblastoma • Aneurysmal bone cyst / giant cell tumor • Ewing sarcoma • Osteosarcoma • Langerhans cell histiocytosis • Metastatic disease, lymphoma / leukemia Lumbar Ganglioneuroma Cervicothoracic Ganglioneuroma Aneurysmal Bone Cyst Aneurysmal Bone Cyst Differential Diagnosis: Langerhans Cell Histiocytosis Non-neoplastic Disorders • Spinal Cord • Multiple sclerosis •ADEM • Neuromyelitis Optica • Osseous •LCH • Gorham-Stout disease • Discitis/osteomyelitis •CRMO • Paraspinal • Extramedullary hematopoiesis ADEM NMO T2 T1 T1 +C T2 T2 Gorham-Stout Disease Gorham-Stout Disease T2 FS T1 +C FS Summary Summary • Pediatric intramedullary tumors are not common in A neglected area of study? children • Yes... • Lower ratio of spinal to intracranial neoplasms • Uncommon entities • 90% glial: astrocytoma and ependymoma • Overlapping imaging features • 10% various nonglial tumors • Technically challenging to image • Nonspecific clinical symptoms • Persistent back pain often major complaint ¾...but there is hope! 9Development of genetic biomarkers 9Advanced Imaging (DTI, ASL, MRS) Thank you! 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