Pediatric Spine Tumors (and other masses)

Pediatric Spine Tumors (and other masses) Francisco A Perez, MD, PhD Assistant Professor Neuroradiology and Pediatric Radiology Seattle Children s Hospital University of Washington, Seattle

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Pediatric spine tumors the challenges Pediatric spine tumors can be challenging to clinicians because they often present with varied, nonspecific, and delayed symptoms. Pediatric spinal tumors can be challenging for pediatric radiologists because they are uncommon, heterogeneous, and imaging features can be nonspecific. Other spinal masses can mimic spine tumors on imaging.

Objectives To generate a differential diagnosis of spinal tumors based on location (intramedullary, intraduralextramedullary, extradural, and extradural/osseous) To describe imaging features of pediatric spinal tumors To review spinal masses that can mimic spine tumors

Pediatric spine masses Key to differential diagnosis is to identify the location 35% 15% 20% I. Intramedullary II. Intradural-Extramedullary 30% III. Extradural IV. Extradural/Osseous

Pediatric spine masses Key to differential diagnosis is to identify the location 35% 15% 20% I. Intramedullary II. Intradural-Extramedullary 30% III. Extradural IV. Extradural/Osseous

IA. Intramedullary pediatric spine tumors Astrocytoma Ependymoma Ganglioglioma Hemangioblastoma Incidence 60% 30% 5% <5% Age 10 years 14 years (NF2) 12 years (NF2) Location Eccentric in cord Central in cord Eccentric in cord Multiple levels 14 years (VHL) Eccentric in cord Imaging Infiltrative Most enhance T2 hyperintense Cysts WHO grade WHO I to IV (Pilocytic, fibrillary, anaplastic and GBM) Treatment Surgery with extent determined by tumor plane +/- Radiation, chemotherapy Well circumscribed Most enhance T2 hyperintense Hemorrhage Metastasis Variable Cysts T1 heterogeneous Calcification Lack of peritumoral edema WHO II to IV WHO 1 typically WHO I Surgery +/- Radiation Well circumscribed Robust Flow voids, hemorrhage Surgery Surgery Radiosurgery

IA. Intramedullary pediatric spine tumors Astrocytoma Ependymoma Ganglioglioma Hemangioblastoma Incidence 60% 30% 5% <5% Age 10 years 14 years (NF2) 12 years (NF2) Location Eccentric in cord Central in cord Eccentric in cord Multiple levels 14 years (VHL) Eccentric in cord Imaging Infiltrative Most enhance T2 hyperintense Cysts WHO grade WHO I to IV (Pilocytic, fibrillary, anaplastic and GBM) Treatment Surgery with extent determined by tumor plane +/- Radiation, chemotherapy Well circumscribed Most enhance T2 hyperintense Hemorrhage Metastasis Variable Cysts T1 heterogeneous Calcification Lack of peritumoral edema WHO II to IV WHO 1 typically WHO I Surgery +/- Radiation Well circumscribed Robust Flow voids, hemorrhage Surgery Surgery Radiosurgery

Pilocytic astrocytoma (WHO I) Case IA.1: Two-year-old girl with one month of abdominal pain and new torticollis. T2 T1+ ADC

Piloytic astrocytoma more examples Case IA.2 12 yo with syrinx T2 T2 T1 T1+ Case IA.3 15 yo with neck pain and abnormal sensation Case IA.4 2 yo with rapidly progressing scoliosis

Fibrillary astrocytoma (WHO II) Case IA.5: Thirteen-year-old boy with chronic neck and back pain. T2 T2 T1+

Glioblastoma (WHO IV) Case IA.6: Eight-year-old girl with one month of neck pain, torticollis and right hand numbness/weakness. T2 T1+ T1+

Glioblastoma more examples Case IA.7 2 yo with back pain and abnormal gait T2 T2 T1 T1+ Case IA.8 16 yo with back pain Case IA.9 15 yo with neck pain

IA. Intramedullary pediatric spine tumors Astrocytoma Ependymoma Ganglioglioma Hemangioblastoma Incidence 60% 30% 5% <5% Age 10 years 14 years (NF2) 12 years (NF2) Location Eccentric in cord Central in cord Eccentric in cord Multiple levels 14 years (VHL) Eccentric in cord Imaging Infiltrative Most enhance T2 hyperintense Cysts WHO grade WHO I to IV (Pilocytic, fibrillary, anaplastic and GBM) Treatment Surgery with extent determined by tumor plane +/- Radiation, chemotherapy Well circumscribed Most enhance T2 hyperintense Hemorrhage Metastasis Variable Cysts T1 heterogeneous Calcification Lack of peritumoral edema WHO II to IV WHO 1 typically WHO I Surgery +/- Radiation Well circumscribed Robust Flow voids, hemorrhage Surgery Surgery Radiosurgery

Ependymoma Case IA.10: Sixteen-year-old girl with back pain. T2 T1 T1+

Ependymoma more examples Case IA.11 19 yo with back pain T2 T1 T1+ T1+ Case IA.12 10 yo with neck pain Case IA.13 20 yo with NF2

IA. Intramedullary pediatric spine tumors Astrocytoma Ependymoma Ganglioglioma Hemangioblastoma Incidence 60% 30% 5% <5% Age 10 years 14 years (NF2) 12 years (NF2) Location Eccentric in cord Central in cord Eccentric in cord Multiple levels 14 years (VHL) Eccentric in cord Imaging Infiltrative Most enhance T2 hyperintense Cysts WHO grade WHO I to IV (Pilocytic, fibrillary, anaplastic and GBM) Treatment Surgery with extent determined by tumor plane +/- Radiation, chemotherapy Well circumscribed Most enhance T2 hyperintense Hemorrhage Metastasis Variable Cysts T1 heterogeneous Calcification Lack of peritumoral edema WHO II to IV WHO 1 typically WHO I Surgery +/- Radiation Well circumscribed Robust Flow voids, hemorrhage Surgery Surgery Radiosurgery

Ganglioglioma Case 1A.14: Seven-year-old girl with head tilt. T2 T1 T1+

Ganglioglioma follow-up Case 1A.14: Six years later 2006 2012

IA. Intramedullary pediatric spine tumors Astrocytoma Ependymoma Ganglioglioma Hemangioblastoma Incidence 60% 30% 5% <5% Age 10 years 14 years (NF2) 12 years (NF2) Location Eccentric in cord Central in cord Eccentric in cord Multiple levels 14 years (VHL) Eccentric in cord Imaging Infiltrative Most enhance T2 hyperintense Cysts WHO grade WHO I to IV (Pilocytic, fibrillary, anaplastic and GBM) Treatment Surgery with extent determined by tumor plane +/- Radiation, chemotherapy Well circumscribed Most enhance T2 hyperintense Hemorrhage Metastasis Variable Cysts T1 heterogeneous Calcification Lack of peritumoral edema WHO II to IV WHO 1 typically WHO I Surgery +/- Radiation Well circumscribed Robust Flow voids, hemorrhage Surgery Surgery Radiosurgery

Hemangioblastoma Case 1A.15: 11-year-old girl with nausea, vomiting, and abnormal head CT. T2 T2 T1+

IA. Intramedullary pediatric spine tumors Astrocytoma Ependymoma Ganglioglioma Hemangioblastoma Incidence 60% 30% 5% <5% Age 10 years 14 years (NF2) 12 years (NF2) Location Eccentric in cord Central in cord Eccentric in cord Multiple levels 14 years (VHL) Eccentric in cord Imaging Infiltrative Most enhance T2 hyperintense Cysts WHO grade WHO I to IV (Pilocytic, fibrillary, anaplastic and GBM) Treatment Surgery with extent determined by tumor plane +/- Radiation, chemotherapy Well circumscribed Most enhance T2 hyperintense Hemorrhage Metastasis Variable Cysts T1 heterogeneous Calcification Lack of peritumoral edema WHO II to IV WHO 1 typically WHO I Surgery +/- Radiation Well circumscribed Robust Flow voids, hemorrhage Surgery Surgery Radiosurgery

IB. Intramedullary tumor mimics Case IB.1 Case IB.2 Case IB.3 T2 T2 Case IB.4 T2 T2

Intramedullary tumor mimics Case 1B.1: Six-year-old girl with neck and arm pain/weakness after trampoline injury. T2 Sag T1+ TWIST Intramedullary glomus arteriovenous malformation

Intramedullary tumor mimics Case 1B.2: Seventeen-year-old boy with remote history of craniospinal radiation for pineoblastoma. T2 T1 T1+ Radiation therapy-related cavernous malformation with acute hemorrhage

Intramedullary tumor mimics Case 1B.3: Twelve-year-old boy with rapid onset tetraplegia. T2 +5 days T2 +5 days T2 Spinal cord infarct (fibrocartilaginous embolism)

Intramedullary tumor mimics Case 1B.4: Five-year-old girl with acute left sided weakness. T2 +3 days T2 +3 days T1+ Longitudinally extensive transverse myelitis

IB. Intramedullary tumor mimics Case IB.1 AVM Case IB.2 Cavernous malformation Case IB.3 Spinal cord infarct T2 T2 Case IB.4 Transverse myelitis T2 T2

Pediatric spine masses Key to differential diagnosis is to identify the location 35% 15% 20% I. Intramedullary II. Intradural-Extramedullary 30% III. Extradural IV. Extradural/Osseous

IIA. Intradural-extramedullary pediatric spine tumors Leptomeningeal metastasis Myxopapillary ependymoma Nerve sheath tumor Incidence Common 15% Uncommon Rare Demographic Various Adolescent Neurofibroma NF1 Schwannoma NF2 Imaging Nerve roots Nodular Enhancement (Can be intramedullary) Pathology Various Medulloblastoma Filum terminale T1 heterogeneous T2 hyperintense Avid WHO I Ependymoma subtype Highly vascular Neuroforamen T1 isointense T2 hyperintense Avid Neurofibroma - dark T2 center Schwannoma - cystic or fatty degeneration Most benign Neurofibroma NF1 Schwannoma NF2 Can be malignant Meningioma NF2 Prior radiation T1 and T2 isointense Avid Dural tail Cystic Clear-cell type more agressive Treatment Chemotherapy Radiation Surgery Recurrence and CSF dissemination Radiation Surgery Surgery

IIA. Intradural-extramedullary pediatric spine tumors Leptomeningeal metastasis Myxopapillary ependymoma Nerve sheath tumor Incidence Common 15% Uncommon Rare Demographic Various Adolescent Neurofibroma NF1 Schwannoma NF2 Imaging Nerve roots Nodular Enhancement (Can be intramedullary) Pathology Various Medulloblastoma Filum terminale T1 heterogeneous T2 hyperintense Avid WHO I Ependymoma subtype Highly vascular Neuroforamen T1 isointense T2 hyperintense Avid Neurofibroma - dark T2 center Schwannoma - cystic or fatty degeneration Most benign Neurofibroma NF1 Schwannoma NF2 Can be malignant Meningioma NF2 Prior radiation T1 and T2 isointense Avid Dural tail Cystic Clear-cell type more agressive Treatment Chemotherapy Radiation Surgery Recurrence and CSF dissemination Radiation Surgery Surgery

Leptomeningeal metastasis Case IIA.1: Five-year-old girl with lifelong history of vomiting. ADC T1+ T1+

Leptomeningeal metastasis - intramedullary Case IIA.2: Five-year-old girl with medulloblastoma. T1+ T1+ T1+

Leptomeningeal metastasis another example? Case IIA.3: Eight-year-old girl with developmental delay, worsening tremor, and abnormal gait. ADC T1+ T1+ NF2

IIA. Intradural-extramedullary pediatric spine tumors Leptomeningeal metastasis Myxopapillary ependymoma Nerve sheath tumor Incidence Common 15% Uncommon Rare Demographic Various Adolescent Neurofibroma NF1 Schwannoma NF2 Imaging Nerve roots Nodular Enhancement (Can be intramedullary) Pathology Various Medulloblastoma Filum terminale T1 heterogeneous T2 hyperintense Avid WHO I Ependymoma subtype Highly vascular Neuroforamen T1 isointense T2 hyperintense Avid Neurofibroma - dark T2 center Schwannoma - cystic or fatty degeneration Most benign Neurofibroma NF1 Schwannoma NF2 Can be malignant Meningioma NF2 Prior radiation T1 and T2 isointense Avid Dural tail Cystic Clear-cell type more agressive Treatment Chemotherapy Radiation Surgery Recurrence and CSF dissemination Radiation Surgery Surgery

Myxopapillary ependymoma Case IIA.4: Fourteen-year-old boy with back pain. T2 T2 T1+

Myxopapillary ependymoma more examples Case IIA.5 15 yo with chronic back pain T2 T2 T1 T1+ Case IIA.6 15 yo with back pain and urinary retention after trauma Case IIA.7 17 yo with Chiari II and new twitching

IIA. Intradural-extramedullary pediatric spine tumors Leptomeningeal metastasis Myxopapillary ependymoma Nerve sheath tumor Incidence Common 15% Uncommon Rare Demographic Various Adolescent Neurofibroma NF1 Schwannoma NF2 Imaging Nerve roots Nodular Enhancement (Can be intramedullary) Pathology Various Medulloblastoma Filum terminale T1 heterogeneous T2 hyperintense Avid WHO I Ependymoma subtype Highly vascular Neuroforamen T1 isointense T2 hyperintense Avid Neurofibroma - dark T2 center Schwannoma - cystic or fatty degeneration Most benign Neurofibroma NF1 Schwannoma NF2 Can be malignant Meningioma NF2 Prior radiation T1 and T2 isointense Avid Dural tail Cystic Clear-cell type more agressive Treatment Chemotherapy Radiation Surgery Recurrence and CSF dissemination Radiation Surgery Surgery

Nerve sheath tumor schwannoma Case IIA.8: Thirteen-year-old girl with stumbling. T2 T2 T1+

Nerve sheath tumor more examples Case IIA.9 7 yo with back and hip pain after fall from horse T2 T2 T1 T1+ Case IIA.10 21 yo with metastatic renal rhabdoid tumor with back pain Case IIA.11 20 yo with NF2

IIA. Intradural-extramedullary pediatric spine tumors Leptomeningeal metastasis Myxopapillary ependymoma Nerve sheath tumor Incidence Common 15% Uncommon Rare Demographic Various Adolescent Neurofibroma NF1 Schwannoma NF2 Imaging Nerve roots Nodular Enhancement (Can be intramedullary) Pathology Various Medulloblastoma Filum terminale T1 heterogeneous T2 hyperintense Avid WHO I Ependymoma subtype Highly vascular Neuroforamen T1 isointense T2 hyperintense Avid Neurofibroma - dark T2 center Schwannoma - cystic or fatty degeneration Most benign Neurofibroma NF1 Schwannoma NF2 Can be malignant Meningioma NF2 Prior radiation T1 and T2 isointense Avid Dural tail Cystic Clear-cell type more agressive Treatment Chemotherapy Radiation Surgery Recurrence and CSF dissemination Radiation Surgery Surgery

Meningioma Case IIA.12: 20-year-old with NF2. T2 T1+ T1+

IIA. Intradural-extramedullary pediatric spine tumors Leptomeningeal metastasis Myxopapillary ependymoma Nerve sheath tumor Incidence Common 15% Uncommon Rare Demographic Various Adolescent Neurofibroma NF1 Schwannoma NF2 Imaging Nerve roots Nodular Enhancement (Can be intramedullary) Pathology Various Medulloblastoma Filum terminale T1 heterogeneous T2 hyperintense Avid WHO I Ependymoma subtype Highly vascular Neuroforamen T1 isointense T2 hyperintense Avid Neurofibroma - dark T2 center Schwannoma - cystic or fatty degeneration Most benign Neurofibroma NF1 Schwannoma NF2 Can be malignant Meningioma NF2 Prior radiation T1 and T2 isointense Avid Dural tail Cystic Clear-cell type more agressive Treatment Chemotherapy Radiation Surgery Recurrence and CSF dissemination Radiation Surgery Surgery

IIB. Intradural-extramedullary tumor mimics Case IIB.1 Case IIB.2 Case IIB.3 T1+ T1+ Case IIB.4 T1+ T2

IIB. Intradural-extramedullary tumor mimics Case IIB.1: 25-year-old male with new diagnosis medulloblastoma status post resection. CT T1 T1+ Post-operative subdural not metastasis

IIB. Intradural-extramedullary tumor mimics Case IIB.2: Four-year-old girl with abnormal gait for two weeks. T1 T1+ T1+ Chronic inflammatory demyelinating polyneuropathy not leptomeningeal metastasis

IIB. Intradural-extramedullary tumor mimics Case IIB.3: Seventeen-year-old boy with posterior fossa ependymoma and aspergillus infection. T2 T1+ T1+ Leptomeningeal aspergillosis fungal infection not dropped metastasis

IIB. Intradural-extramedullary tumor mimics Case IIB.4: Thirteen-year-old girl with leg weakness and hyperreflexia. T2 DWI T1+ Ventral neurenteric cyst

IIB. Intradural-extramedullary tumor mimics Case IIB.1 Post op subdural Case IIB.2 CIDP Case IIB.3 Fungal infection T1+ T1+ Case IIB.4 Neurenteric cyst T1+ T2

Pediatric spine masses Key to differential diagnosis is to identify the location 35% 15% 20% I. Intramedullary II. Intradural-Extramedullary 30% III. Extradural IV. Extradural/Osseous

Pediatric spine masses Key to differential diagnosis is to identify the location 35% 15% 20% I. Intramedullary II. Intradural-Extramedullary 30% III. Extradural IV. Extradural/Osseous

IIIA. Extradural pediatric spine tumors Neuroblastoma Incidence Relatively common Up to 15% develop spinal cord compression Myeloid sarcoma ( chloroma ) Relatively rare Demographic 1 to 5-years-old Patients with AML Imaging Large paraspinal soft tissue mass with intraspinal extension Mildly T2 hyperintense T2 hypointense areas may represent calcification Variable T1 isointense with homogeneous T2 hypointensity Pathology Neural crest cell origin Solid tumor of myelogenous stem cells Typically AML Treatment Chemotherapy Radiation Surgery Chemotherapy Radiation Surgery not usually necessary

IIIA. Extradural pediatric spine tumors Neuroblastoma Incidence Relatively common Up to 15% develop spinal cord compression Myeloid sarcoma ( chloroma ) Relatively rare Demographic 1 to 5-years-old Patients with AML Imaging Large paraspinal soft tissue mass with intraspinal extension Mildly T2 hyperintense T2 hypointense areas may represent calcification Variable T1 isointense with homogeneous T2 hypointensity Pathology Neural crest cell origin Solid tumor of myelogenous stem cells Typically AML Treatment Chemotherapy Radiation Surgery Chemotherapy Radiation Surgery not usually necessary

Neuroblastoma Case IIIA.1: Two-year-old girl unable to bear weight. T2 T2 T1+

IIIA. Extradural pediatric spine tumors Neuroblastoma Incidence Relatively common Up to 15% develop spinal cord compression Myeloid sarcoma ( chloroma ) Relatively rare Demographic 1 to 5-years-old Patients with AML Imaging Large paraspinal soft tissue mass with intraspinal extension Mildly T2 hyperintense T2 hypointense areas may represent calcification Variable T1 isointense with homogeneous T2 hypointensity Pathology Neural crest cell origin Solid tumor of myelogenous stem cells Typically AML Treatment Chemotherapy Radiation Surgery Chemotherapy Radiation Surgery not usually necessary

Meyloid sarcoma Case IIIA.2: Three-year-old boy with AML and worsening upper back pain. T2 T2 T1+

IIIA. Extradural pediatric spine tumors Neuroblastoma Incidence Relatively common Up to 15% develop spinal cord compression Myeloid sarcoma ( chloroma ) Relatively rare Demographic 1 to 5-years-old Patients with AML Imaging Large paraspinal soft tissue mass with intraspinal extension Mildly T2 hyperintense T2 hypointense areas may represent calcification Variable T1 isointense with homogeneous T2 hypointensity Pathology Neural crest cell origin Solid tumor of myelogenous stem cells Typically AML Treatment Chemotherapy Radiation Surgery Chemotherapy Radiation Surgery not usually necessary

IIIB. Extradural tumor mimics Case IIIB.1 Case IIIB.2 T2 T1+ T2

IIIB. Extradural tumor mimics Case IIIB.1: Two-year-old, follow up cystic lesion seen during evaluation for possible tethered cord. T2 T2 T1+ Epidural arachnoid cyst

IIIB. Extradural tumor mimics Case IIIB.2: Eleven-year-old girl with intermittent back pain awakening her at night with sudden worsening. T2 T1+ T1+ Venous vascular malformation with epidural hemorrhage

IIIB. Extradural tumor mimics Case IIIB.1 Arachnoid cyst Case IIIB.2 Venous vascular malformation T2 T1+ T2

Pediatric spine masses Key to differential diagnosis is to identify the location 35% 15% 20% I. Intramedullary II. Intradural-Extramedullary 30% III. Extradural IV. Extradural/Osseous

IVA. Osseous pediatric spine tumors Ewing Sarcoma Langerhans cell histiocytosis (LCH) Incidence Relatively common Relatively uncommon Chordoma Rare Osteoblastoma (and osteoid osteoma) Relatively uncommon Demographic 5 to 13-years-old 1 to 3-years-old 10-years-old Male adolescent Imaging Osseous and/or extraosseous Permeative, lytic osseous lesion Soft tissue mass T1 and T2 iso to hypointense Variable Lytic lesion Vertebra plana Soft tissue component Heterogeneous Lytic, destructive lesion T2 hyperintense with septations Variable Soft tissue mass Focal sclerotic nidus Posterior spinal elements Marked edema / May have soft tissue component Pathology Neuroectodermal small round-cell sarcoma Dysregulated growth and activity of Langerhans cells Neoplastic? Arises from notochordal remants Highly vascular nidus of osteoid bone with surrounding reactive osteosclerosis Treatment Chemotherapy Radiation Surgery Conservative Chemotherapy Surgery Radiation Surgery Radiation Surgery NSAID RFA

IVA. Osseous pediatric spine tumors Ewing Sarcoma Langerhans cell histiocytosis (LCH) Incidence Relatively common Relatively uncommon Chordoma Rare Osteoblastoma (and osteoid osteoma) Relatively uncommon Demographic 5 to 13-years-old 1 to 3-years-old 10-years-old Male adolescent Imaging Osseous and/or extraosseous Permeative, lytic osseous lesion Soft tissue mass T1 and T2 iso to hypointense Variable Lytic lesion Vertebra plana Soft tissue component Heterogeneous Lytic, destructive lesion T2 hyperintense with septations Variable Soft tissue mass Focal sclerotic nidus Posterior spinal elements Marked edema / May have soft tissue component Pathology Neuroectodermal small round-cell sarcoma Dysregulated growth and activity of Langerhans cells Neoplastic? Arises from notochordal remants Highly vascular nidus of osteoid bone with surrounding reactive osteosclerosis Treatment Chemotherapy Radiation Surgery Conservative Chemotherapy Surgery Radiation Surgery Radiation Surgery NSAID RFA

Ewing sarcoma Case IVA.1: Thirteen-year-old boy with back pain after skateboarding injury with new numbness and weakness. T2 T1+ CT

IVA. Osseous pediatric spine tumors Ewing Sarcoma Langerhans cell histiocytosis (LCH) Incidence Relatively common Relatively uncommon Chordoma Rare Osteoblastoma (and osteoid osteoma) Relatively uncommon Demographic 5 to 13-years-old 1 to 3-years-old 10-years-old Male adolescent Imaging Osseous and/or extraosseous Permeative, lytic osseous lesion Soft tissue mass T1 and T2 iso to hypointense Variable Lytic lesion Vertebra plana Soft tissue component Heterogeneous Lytic, destructive lesion T2 hyperintense with septations Variable Soft tissue mass Focal sclerotic nidus Posterior spinal elements Marked edema / May have soft tissue component Pathology Neuroectodermal small round-cell sarcoma Dysregulated growth and activity of Langerhans cells Neoplastic? Arises from notochordal remants Highly vascular nidus of osteoid bone with surrounding reactive osteosclerosis Treatment Chemotherapy Radiation Surgery Conservative Chemotherapy Surgery Radiation Surgery Radiation Surgery NSAID RFA

Langerhans cell histiocytosis Case IVA.2: Two-year-old boy with torticollis. DX FLAIR T1+

IVA. Osseous pediatric spine tumors Ewing Sarcoma Langerhans cell histiocytosis (LCH) Incidence Relatively common Relatively uncommon Chordoma Rare Osteoblastoma (and osteoid osteoma) Relatively uncommon Demographic 5 to 13-years-old 1 to 3-years-old 10-years-old Male adolescent Imaging Osseous and/or extraosseous Permeative, lytic osseous lesion Soft tissue mass T1 and T2 iso to hypointense Variable Lytic lesion Vertebra plana Soft tissue component Heterogeneous Lytic, destructive lesion T2 hyperintense with septations Variable Soft tissue mass Focal sclerotic nidus Posterior spinal elements Marked edema / May have soft tissue component Pathology Neuroectodermal small round-cell sarcoma Dysregulated growth and activity of Langerhans cells Neoplastic? Arises from notochordal remants Highly vascular nidus of osteoid bone with surrounding reactive osteosclerosis Treatment Chemotherapy Radiation Surgery Conservative Chemotherapy Surgery Radiation Surgery Radiation Surgery NSAID RFA

Chordoma Case IVA.3: Eleven-year-old girl with right lower quadrant pain and spine abnormality on abdomen CT. CT STIR T1+

IVA. Osseous pediatric spine tumors Ewing Sarcoma Langerhans cell histiocytosis (LCH) Incidence Relatively common Relatively uncommon Chordoma Rare Osteoblastoma (and osteoid osteoma) Relatively uncommon Demographic 5 to 13-years-old 1 to 3-years-old 10-years-old Male adolescent Imaging Osseous and/or extraosseous Permeative, lytic osseous lesion Soft tissue mass T1 and T2 iso to hypointense Variable Lytic lesion Vertebra plana Soft tissue component Heterogeneous Lytic, destructive lesion T2 hyperintense with septations Variable Soft tissue mass Focal sclerotic nidus Posterior spinal elements Marked edema / May have soft tissue component Pathology Neuroectodermal small round-cell sarcoma Dysregulated growth and activity of Langerhans cells Neoplastic? Arises from notochordal remants Highly vascular nidus of osteoid bone with surrounding reactive osteosclerosis Treatment Chemotherapy Radiation Surgery Conservative Chemotherapy Surgery Radiation Surgery Radiation Surgery NSAID RFA

Osteoblastoma Case IVA.4: Twelve-year-old boy with back pain. CT STIR +2 years CT

Osteoblastoma more examples Case IVA.5 11 yo with back pain CT CT STIR T1+ Case IVA.6 9 yo with neck pain Case IVA.7 5 yo with neck pain

IVA. Osseous pediatric spine tumors Ewing Sarcoma Langerhans cell histiocytosis (LCH) Incidence Relatively common Relatively uncommon Chordoma Rare Osteoblastoma (and osteoid osteoma) Relatively uncommon Demographic 5 to 13-years-old 1 to 3-years-old 10-years-old Male adolescent Imaging Osseous and/or extraosseous Permeative, lytic osseous lesion Soft tissue mass T1 and T2 iso to hypointense Variable Lytic lesion Vertebra plana Soft tissue component Heterogeneous Lytic, destructive lesion T2 hyperintense with septations Variable Soft tissue mass Focal sclerotic nidus Posterior spinal elements Marked edema / May have soft tissue component Pathology Neuroectodermal small round-cell sarcoma Dysregulated growth and activity of Langerhans cells Neoplastic? Arises from notochordal remants Highly vascular nidus of osteoid bone with surrounding reactive osteosclerosis Treatment Chemotherapy Radiation Surgery Conservative Chemotherapy Surgery Radiation Surgery Radiation Surgery NSAID RFA

IVB. Osseous tumor mimics Case IVB.1 Case IVB.2 Case IVB.3 T1+ T1+ Case IVB.4 T2 CT

IVB. Osseous tumor mimics Case IVB.1: 11-year-old boy with history of back pain and new leg weakness. CT T2 T1+ Intraosseous low flow/venous malformation

IVB. Osseous tumor mimics Case IVB.2: Fourteen-year-old boy with history of tuberculosis. T2 T1+ CT Tuberculous discitis

IVB. Osseous tumor mimics Case IVB.3: Nine-year-old boy with history of choroid plexus carcinoma and neck pain. CT STIR T1+ Aneurysmal bone cyst

IVB. Osseous tumor mimics Case IVB.4: Seventeen-year-old girl with low back pain. STIR T1 CT Myositis ossificans

IVB. Osseous tumor mimics Case IVB.1 Intraosseous hemangioma / vascular malformation Case IVB.2 Tuberculous discitis Case IVB.3 Aneurysmal bone cyst T1+ T1+ Case IVB.4 Myositis ossificans T2 CT

Pediatric spine masses Key to differential diagnosis is to identify the location 35% 15% 20% I. Intramedullary II. Intradural-Extramedullary 30% III. Extradural IV. Extradural/Osseous

Takeaways Identify the location and extent of a spinal mass to determine the differential diagnosis Consider non-neoplastic causes of spinal masses