Ependymoma of the spine

Transcription

1 Ependymoma of the spine Tenny Zhang, MS-3 Harvard Medical School 1

2 Case presentation: history and exam HPI: A 30-year-old man with no significant past medical history presents with one week of bilateral buttock and radiating leg pain worse with valsalva, one week of progressive low back pain, and one day of severe headache, neck stiffness, nausea, and vomiting. He reports having URI symptoms one week prior to onset of current symptoms. Per patient, back pain seemed to begin after going over a speed bump roughly while driving. He denies fever, photophobia, bladder or bowel incontinence, or changes in mental status. ROS: In addition to the above, notable for mild fatigue. Exam: Temp 99.4, HR 76, BP 150/71, SaO2 96%RA. General and neurologic exams were unremarkable other than marked nuchal rigidity and positive Kernig and Brudzinski signs. 2

3 Case presentation: initial work-up In the emergency department, initial labs were drawn, a lumbar puncture was performed, and a non-contrast head CT was obtained. Labs: Other than WBC 13.9, CBC, BMP, and UA were normal. Urine and serum toxicology screens were negative. HIV negative. Blood cultures and Lyme serology were sent. LP: Tube 1 returned TNC 623 and 78,482 RBC. Tube 4 returned TNC 107 and 45,102 RBC. Normal differential. No xanthochromia was noted. Total protein elevated to 280. Glucose normal at 46. PCR for HSV and enterovirus, gram stain, and CSF culture were sent. Imaging: NCHCT showed no evidence of acute intracranial process. 3

4 Case presentation: meningeal signs The patient was admitted to the neurology service and started on empiric vancomycin, ceftriaxone, and acyclovir with concern for meningitis given his marked meningeal signs. The differential largely centered around meningitis vs. subarachnoid hemorrhage. Bacterial meningitis Patients typically appear extremely sick Though this patient had severe headache, nuchal rigidity, nausea, and vomiting, he did not have fever or photophobia and appeared well overall Aseptic meningitis Evidence of meningeal inflammation with negative bacterial cultures Causes are numerous including viral (enterovirus, HSV, VZV, CMV, EBV, HIV), fungi, parasites, medications, and systemic disease **Subarachnoid hemorrhage presents with sudden-onset severe headache +/- signs of increased ICP and/or meningeal irritation 4

5 Radiologic work-up: menu of tests The patient received numerous radiologic studies. Again, a non-contrast head CT was first performed and found to be unremarkable. Study Indication Findings 1. NCHCT Evaluate for acute intracranial process No evidence of acute intracranial process 5

6 Radiologic work-up: menu of tests Head and neck CT angiography was next performed with concern for subarachnoid bleed. Study Indication Findings 1. NCHCT Evaluate for acute intracranial process No evidence of acute intracranial process 2. CTA head and neck Concern for SAH/aneurysm Unremarkable with no evidence of aneurysm 6

7 Radiologic work-up: CTA volume rendering Rendering technique is used to transform serially acquired axial CT image data into 3D images Here, we see the vessels of the circle of Willis and their principal intracranial branches appear normal without stenosis, occlusion, or aneurysm formation BIDMC PACS The carotid and vertebral arteries also appear NeuroEMS, normal 7

8 Radiologic work-up: menu of tests MR of the head was also normal. Finally, MR of the spine was performed because of the patient s persistent low back pain, ultimately revealing a spinal mass. Study Indication Findings 1. NCHCT Evaluate for acute intracranial process No evidence of acute intracranial process 2. CTA head and neck Concern for SAH/aneurysm Unremarkable with no evidence of aneurysm 3. MR head w/wo Evaluate for meningeal enhancement, hemorrhage, obstruction of CSF flow No evidence of meningeal enhancement, hemorrhage, or ventriculomegaly 4. MR full spine w/wo Worsening low back pain Intradural mass at T12-L1 8

9 Radiologic work-up: MR spine sagittal view T2 There is a 1.3 x 2.1 cm intradural mass at the level of T12-L1 demonstrating T2 hypointensity There is resultant impingement of the cauda equina nerve roots with effacement of the CSF space There is layering hemorrhage at the L5- S1 level within the thecal sac, possibly related to prior LP or bleeding mass BIDMC PACS 9

10 Radiologic work-up: MR spine sagittal view T2 b a There is a 1.3 x 2.1 cm intradural mass (a) at the level of T12-L1 demonstrating T2 hypointensity There is resultant impingement (b) of the cauda equina nerve roots with effacement of the CSF space c There is layering hemorrhage (c) at the L5-S1 level within the thecal sac, possibly related to prior LP or bleeding mass BIDMC PACS 10

11 Radiologic work-up: MR spine sagittal view T1 post-contrast The 1.3 x 2.1 cm intradural mass at the level of T12-L1 demonstrates T1 isointensity and enhancement around the periphery Radiologically, this mass was thought to resemble an ependymoma, meningioma, or schwannoma (continue for a discussion of key radiologic features of these and other spinal tumors) BIDMC PACS 11

12 Radiologic work-up: MR spine sagittal and axial views T2 T2 T1 post-contrast * * Is the mass intraduralextramedullary or intramedullary? BIDMC PACS BIDMC PACS PACS 12

13 Spinal cord tumors: overview and anatomy UpToDate Spinal cord tumors occur within or adjacent to the spinal cord, and can be primary or metastatic Primary spinal cord tumors account for just 2-4% of all CNS tumors Spinal cord tumors are classified according to location: Intramedullary tumors sit within the spinal cord itself and mostly arise from glial cells Intradural-extramedullary tumors arise within the dura but outside the spinal cord, mostly from Schwann cells covering nerve roots or meningeal cells covering the spinal cord Extradural masses most often are metastases arising in vertebral bodies 13

14 Spinal cord tumors: presentation and diagnostic imaging Presentation The most common symptom is pain that frequently causes nocturnal awakening and is exacerbated by maneuvers that increase intrathoracic or intraabdominal pressure Neurologic deficits may occur distal to the lesion, most commonly sensory dysesthesia, weakness, sensory loss, and sphincter dysfunction Imaging MRI is the diagnostic study of choice, providing superb visualization of the spinal cord and surrounding structures CT is useful for patients with contraindications to MRI, and is good for visualizing bony structures and evaluating spinal stability in preoperative planning Plain film may demonstrate some bony changes, particularly in children 14

15 Spinal cord tumors: subtypes and differential diagnosis Intramedullary Intradural-extramedullary Most common Ependymoma Astrocytoma Meningioma Schwannoma Less common Oligodendroglioma, ganglioma, hemangioma, PCNSL, mets, teratoma, neurenteric cyst, dermoid tumor, epidermoid tumor, lipoma, cavernous angioma Neurofibroma, paraganglioma, mets, neurenteric cyst, dermoid tumor, epidermoid tumor, lipoma, cavernous angioma Extradural Metastatic disease Chordoma, multiple myeloma, osteosarcoma, chondrosarcoma, Ewing sarcoma, sarcoma, plasmacytoma, giant cell tumor, osteoblastoma 15

16 Spinal cord tumors: intramedullary Ependymoma: Central Well-marginated Hemorrhage, cyst, syrinx Astrocytoma: Eccentrically located Poorly marginated T2 BIDMC PAS Sagittal FSE T1 post-contrast; ependymoma Wald, Imaging of Spine Neoplasm, Radiol Clin N Am (2012) Sagittal FSE T1 post-contrast; astrocytoma Wald, Imaging of Spine Neoplasm, Radiol Clin N Am (2012) 16

17 Spinal cord tumors: intradural-extramedullary Meningioma: Homogeneously enhancing Dural tail Calcifications Schwannoma: Ring enhancement Vertebral scalloping Widening of neural foramen Coronal FSE T1 post-contrast; meningioma Wald, Imaging of Spine Neoplasm, Radiol Clin N Am (2012) Axial CT; schwannoma Wald, Imaging of Spine Neoplasm, Radiol Clin N Am (2012) 17

18 Spinal cord tumors: extradural metastases Metastatic disease: Most commonly prostate, breast, lung T-spine > L-spine > C-spine Can cause cord compression when involving epidural area May be lytic or blastic Sagittal FSE T2; metastasis causing cord compression and pathologic fracture Wald, Imaging of Spine Neoplasm, Radiol Clin N Am (2012) Sagittal reformat FSE T2; lytic metastasis Wald, Imaging of Spine Neoplasm, Radiol Clin N Am (2012) 18

19 Case presentation: clinical course UCSF The patient s blood and CSF cultures, CSF gram stain, and Lyme, HSV, and enterovirus assays returned negative Neurosurgery was consulted following MR spine and patient underwent tumor resection at outside hospital without complications Pathology revealed grade II ependymoma with infarct-like necrosis, foci of prior hemorrhage, and cystic components On the left, we see normal cuboid ependymal cells, which line the ventricles of the brain and the spinal canal, producing CSF and helping to circulate it with cilia 19

20 Case presentation: diagnosis and reports in the literature This patient s presentation is consistent with subarachnoid hemorrhage (SAH) due to spinal tumor, a rare clinical scenario that accounts for less than 1% of all patients with SAH Ependymomas are the most common cause of SAH of spinal origin, accounting for 60% of cases A 2008 review found 17 similar cases of spinal ependymoma presenting as SAH reported in the literature since 1958 (Ulrich et al. 2008) Symptoms of SAH due to spinal ependymoma depend on the amount and rate of bleeding, and occur secondary to nerve root and meningeal irritation Acute-onset lower back pain, radicular pain, and severe headache are most common May also present with nausea, vomiting, photophobia, altered mental status and other meningeal signs, incontinence, cranial nerve deficits, and motor deficits Proposed mechanisms of SAH due to spinal tumors in the conus region are twofold (Ekuna et al. 2017): 1. Mechanical: the conus region is highly mobile, thus traction forces during sudden or sharp movements may disrupt vessels on the tumor surface (recall this patient noticed his symptoms after experiencing a jolting speed bump) 2. Histopathological: bleeding occurs due to the numerous small, thin-walled blood vessels and loss of connective tissue within certain tumor types 20

21 Summary 1. Spinal cord tumors may be primary or metastatic and are categorized by location: intramedullary, intradural-extramedullary, or extradural 2. Patients may present with a wide range of symptoms, but unrelenting local pain is the most common complaint 3. In rare cases, a hemorrhagic spinal mass may cause meningeal irritation, masking as meningitis or intracranial subarachnoid hemorrhage 4. MRI offers superior visualization of the specific location of the mass, and taken together with history, allows narrowing of specific tumor type 5. Final diagnosis requires pathologic investigation 21

22 References 1. Argyropoulou, P.I. et al. (2001). Myxopapillary ependymoma of the conus medullaris with subarachnoid haemorrhage: MRI in two cases. Neuroradiology 43: Ekuma, E.M. et al. (2017). A rare case of pediatric lumbar spinal ependymoma mimicking meningitis. World Neurosurg 100:710.e1-e5. 3. Ependymal cells. UCSF. l2.jpg. 4. Koeller, K.K. et al. (2000). Neoplasms of the spinal cord and filum terminale: radiologic-pathologic correlation. AFIP Archives 20: Sa adah, M. et al. (2004). Atypical presentations of conus medullaris and filum terminale myxopapillary ependymomas. Journal of Clinical Neuroscience 11(3): Ulrich, C.T. et al. (2008). Ependymoma of conus medullaris presenting as subarachnoid haemorrhage. Acta Neurochir 150: Wald, J.T. (2012). Imaging of spine neoplasm. Radiol Clin N Am 50: Welch, W.C. et al. (2016). Spinal cord tumors [image]. UpToDate. 22