1. Remy R. Lobo, MD Stephen F. Kralik, MD Isaac C. Wu, MD
eEdE#: eEdE-201
Control#: 851
Pediatric Extradural Spinal Masses: Spectrum of Common and Uncommon Pathology Affecting the Young Spine
Control #: 851 Title: Pediatric Extradural Spinal Masses: Spectrum of Common and Uncommon Pathology Affecting the Young Spine eEdE#: eEdE-201 (Shared Display)
Remy R. Lobo, MD
Stephen F. Kralik, MD
Isaac C. Wu, MD
Required format: Purpose, Approach/Methods,
Findings/Discussion, Summary/Conclusion.

2. No financial disclosures

3. Purpose/Background
CNS tumors are second only to hematopoeitic malignancies in the pediatric population
Spinal tumors make up less than 10% of these
This exhibit presents a spectrum of extradural spinal masses and proposes a framework to characterize these tumors in practice

4. “Back” ground
Most articles focus on the intramedullary space lesions (red)
~60% of spinal lesions
Or the intradural, extramedullary space lesions (blue)
~25% of spinal lesions
Illustration A. Micheau – MD e-anatomy4, copyright IMAIOS 2015
Illustration A. Micheau – MD
e-anatomy4, ©IMAIOS 2015

5. This exhibit will focus on everything else Bone (purple) Soft tissue
Nerves
Fat
Muscles
Lesions in both spaces (bone & soft tissues)
Illustration A. Micheau – MD e-anatomy4, copyright IMAIOS 2015
Illustration A. Micheau – MD
e-anatomy4, ©IMAIOS 2015
#EverythingBUTtheCORD!

6. With a few sports analogies thrown in for effect!
So, with that settled
Let’s play ball!
With a few sports analogies thrown in for effect!
Great Games by Williamson Publishing

7. Spectrum of normal
First, we will cover the normal findings as children age
i.e. what’s fair?

8. Normal (continued) 3 month old Note the ossification center at C1CT
3 month old
Note the ossification center at C1
Hematopoietic marrow
Hypointense on T1
T2 slightly hyperintense T1 T2
STIR to the side

9. Normal (continued) 2 year old spine: Developing bone
T1 hypointense (hematopoeitic)
Slightly T2 hyperintense

10. Normal (continued) 6 year old spine
Bone continues to develop on CT
Increasing T1 signal
Some decrease in T2 (bone is still active)
Lumbosacral spine shows similar T1 and T2 characteristics
Normal hematog marrow to 10yo, t1 hypo-iso to disc w/o enhancement, calvarium, spine, ribs and pelvis are last to switch to fatty, and first to convert back to red

11. Normal (continued)
By years of age, bone marrow can approximate (CT) mineralization and signal intensity (on T1 and T2) seen in adults CT T1 T2

12. Normal (continued) Old enough to drive:
Mineralization is basically equivalent to an adult
T1 hyperintense and predominantly T2 hypointense fatty marrow

13. A structured approach:
Know the rules
History often points to a diagnosis
Is the process
Bone?
Soft tissue?
Both?
Signal characteristics

14. “Frozen” bones 9 year old with an obvious bone finding Osteopetrosis
Dense and sclerotic bones

15. She was playing outside and fell
Diffusely abnormal T1 and T2 bone marrow signal
Extradural T1 isointense, T2 hypointense lesion with mass effect on the cord. Note the associated cord edema

16. T1 isointense extradural lesion with mass effect at the cord
T2 hypointense, note cord the associated edema
(descending corticospinal tract)

17. “Classic” case
No enhancement on axial T1+C or sagittal T1+C with FS confirms initial suspicion
Epidural hematoma
She had decompression surgery performed given the acute cord edema
#HistoryALWAYShelps!

18. Edge finding:
A 2.5 year old with fever and abdominal pain got a CT abdomen study
Small hypoattenuating lesions in the spleen (possible abscesses?)
Notice the subtle finding at the spine?
Better demonstrated on the coronal

19. Fever and back pain (continued)
T1 isointense and T2 hyperintense extradural lesion,
Exits neural foramen into the soft tissues
Axial and coronal T1+C with FS show the peripherally enhancing extradural lesion matching the clinical picture of suspected infection

20. Fever and Back Pain
Team approach with a good history helps guide management
Multiple bony abscesses on sagittal T1+C with FS
Emergent laminectomy with cultures performed
Specimen grew S. aureus
#It’salwaysStaph

21. Lesions outside the lines
16 year old girl with chronic anemia and multiple CVAs
MRA MIPs images (Rt and Lt)
She had spine imaging because of numbness, back pain, and difficulty walking
Low intensity extradural lesions on both T1 and T2
Moyamoya disease
You need a hawk-eye to see it!
9yo

22. Note abnormal hypointense marrow
Hypointense tissue on T1 and T2
No significant enhancement on T1+C with FS, axial and sagittal
Sickle cell patient, treated with repeated pheresis exchanges
Extramedullary hematopoeisis
Ax and sag t1 post con, and a post sag t1 w/ FS
#malariaresistant?

23. End of the first set History is very helpful
Abscess favored with fever
Hematopoeisis in the setting of chronic anemia
Hematoma in cases of recent trauma/dyscrasias
The next set will focus on bone lesions

24. Easy as 1, 2, 3 14 year old boy with back pain
Subtle left L4 pedicle lucency
Expansile lesion, smooth margins
3 phase bone scan, photopenic

25. At the plate Signal mimics fluid on T1 (left) and T2 (right) MRI
Coronal T2 shows the expansion seen on radiograph

26. Out of the park?
Mild peripheral enhancement on axial, sagittal T1+C with FS
Aneurysmal bone cyst (ABC)
Treated percutaneously
Often associated with fluid-fluid levels (on T2) though not seen here
#ABC_easyas123

27. One more at the wall: 10 year old girl with neck pain
Lucent C3 lesion on radiograph
CT shows the expansile lesion, arising from bone
Left sided asymmetric collapse of the vertebral body, and right sided expansion

28. Magnified T2 shows the septations and a fluid fluid level
Isointense axial and sagittal T1, with mixed signal intensities on T2 weighted sequences
Diffuse bony enhancement without expansile soft tissue component, possible septations?
Magnified T2 shows the septations and a fluid fluid level
Aneurysmal Bone Cyst
#cantALLreadtheBOOK!

29. Some things start small
14 year old girl with neck pain, often worse at night
T2 MRI shows edema at C7
Focal radiotracer accumulation on the left at this same level

30. Neck pain (cont’d) Centrally sclerotic nidus at the left C7 lamina
CT (axial)
Neck pain (cont’d)
Centrally sclerotic nidus at the left C7 lamina
Surrounding lucency
Osteoid osteoma
Typical history and age helps cinch the diagnosis
Note, nidus is <1.5cm in size
Some sources cite 2 cm
CT (sag)
#TeenagePAINintheNECK

31. Need an aspirin yet? 6 year old girl with scoliosis
Expansile lesion on T1, T2
Note mixed signal on axial T2
Had taken NSAIDs before the scoliosis worsened
Diffuse enhancement on T1+C
Osteoblastoma at excision
#GIANTosteoidosteoma

32. From a ‘cord’ of sorts
7 year old male with a history of limping for several months
Axial T1 iso-intense and T2 hyper-intense left sacral mass
Enhancement on sagittal T1+C
Tc MDP bone scan shows mild radiotracer accumulation
(atypical in this case)

33. Can occur anywhere along the neural axis
CT portal and delayed phase show the soft tissue enhancement, bony destruction, and minimal calcification (note absence of fat)
Notochord remnant, most commonly seen at the sacrum, though rare at pediatric patients
Chordoma
Ecchordosis Physaliphora doesn’t enhance
Can occur anywhere along the neural axis
#dorsumsella2coccyx

34. Bony lesions summary Fluid-fluid levels favor ABC (1o or 2o origin)
Teenager with pain and small sclerotic focus is likely an osteoid osteoma
Osteoblastoma when the nidus is > 1.5cm
Can dedifferentiate to osteoblastic osteosarcoma
Chordoma commonly occurs at the sacrum, T2 hyperintense and enhancing
Additional lesions could include Giant Cell Tumor, Chondrosarcoma and metastases

35. Halftime, want an orange?
Those were bone lesions
Next will be lesions arising in the soft tissues
Followed by lesions from soft tissue and bone
If you get tired… Here’s a good excuse:
Also, playing a World Cup winning German side

36. Hit the court 7 week old with stridor
Paraspinal soft tissue
CT abdomen shows the mass isodense to muscle
Coronal STIR shows the multilevel involvement, contiguous with multiple neural foramina on sagittal

37. Isointense to muscle on T1, spanning multiple levels as seen on CT
Diffusely hyperintense on T2, distinct from the right adrenal gland (not shown)
Most likely a nerve sheath tumor, favor sympathetic chain origin (especially given age and location)

38. Diffusely enhancing T1+C with FS Neuroblastoma at excision
Good prognosis early, especially if there are skin lesions (stage 4S)
Adrenal gland > sympathetic chain > thoracic > other sites
Can check urine catecholamines
Commonly metastasizes, can involve any location
#PEDSboardreview_flashback!

39. Neural tumor spectrum 7 year old boy who presented with cough
Heterogeneously enhancing posterior mediastinal mass
Spans multiple levels, neuroforaminal invasion
As seen on MRI
T1 and T2

40. Findings suggest a tumor of neural origin Ganglioneuroblastoma (GNB)
Sagittal T1, T2, STIR and coronal T2 redemonstrate the multilevel paraspinal mass
Post contrast T1 images show its extradural location and diffuse enhancement
Findings suggest a tumor of neural origin
Ganglioneuroblastoma (GNB)
May be hard to distinguish from NB
#cantALWAYStell?!

41. Ringing a different “bell”
6 year old female with gait abnormalities and urinary incontinence receives an MRI
Expansile hypo-intense T1 and a hyperintense T2 lesion exiting the spinal canal
The cord is displaced laterally
coronal

42. Sagittal T1 and T2 show the extradural origin
Diffuse enhancement on axial and sagittal T1+C
Note the expanded neural foramen (*)
Again, suspect nerve tissue origin, though from a peripheral nerve in this case
Schwannoma
#dumbbell_(0-0)_shape *

43. Balancing things out 10 year old girl with CMTC
Cutis Marmorata Telangiectatica Congenita
Presented with scoliosis, hemihypertrophy, and developmental delay
Expansile T1 isointense tissue, T2 hyperintense
Exits neural foramina
Mass effect at nerve
Though not a member of the Condensed Matter Theory Center

44. Diffusely enhancing on T1+C DDx includes Schwannoma, and hemangioma
Bonus T2 image shows a syrinx, and an additional hemangioma
Diffusely enhancing on T1+C
DDx includes Schwannoma, and hemangioma
Look for additional clues (right rib lesion)
Isointense on T1, hyperintense on T2
Diffusely enhancing
Hemangioma
#WHEREStheCORDUROY?

45. In the trenches 13 year old female with difficulty walking
Hypointense without suppression on STIR
Left (T1) and Right (T2) show similar findings
Extensive component into the soft tissues, out the foramina T1 T2
STIR

46. Breaking through the gaps
Diffuse enhancement on axial and sagittal T1+C with FS
Excision showed epidural lymphoma
Diffuse Large B-Cell Lymphoma
Treated with chemotherapy
Common pediatric malignancies
Leukemia, lymphoma
Intracranial neoplasms
#onEVERYradiologyDDx!

47. Soft Tissue summary Often arise from neural tissue
Think neuroblastoma (evaluate adrenals)
Ganglioneuroblastoma
Schwannoma (peripheral nerve sheath tumor)
Don’t forget about hemangioma as a mimic
Robust enhancement may favor lymphoma
What is the most common extradural soft tissue tumor?
Metastasis
We’ll finish with tumors of both soft tissue and bone
#WHENinDOUBT

48. “Ultimate” case? 6 year old boy with back pain Similar findings on MRI
Flattened vertebral body
Vertebra plana
Similar findings on MRI
Expansile soft tissue
Retropulsion
Replaces vertebra T1 T2 T1 T2

49. But not a “Disc” problem
Enhances profusely on axial and sagittal T1+C FS
Muscle density on CT
Can obtain tissue for diagnosis
Have needle, will stick!
Eosinophilic granuloma
Langerhans cell histiocytosis
Prone
#EVERYpediatricDDX!

50. Hidden lesions 10 year old girl with neck pain
Unremarkable CT on bone window
Mass seen on soft tissue window
Paraspinal and extradural
Posterior extent on coronal
Similarly on sagittal

51. Sagittal images show a T1 isointense, T2 hypointense mass without suppression on STIR, suggestion of osseous involvement (tumor on both sides of bone).
Coronal and sagittal T1+C show the necrotic center, and diffuse peripheral enhancement. Lesion remains in the extradural space, permeating the bone.

52. Ewing sarcoma (ES) Enhancement on T1+C is typical
ES is a small round blue cell tumor
PNET, leukemia, lymphoma and other SmRBCT all may appear similar to ES
Second decade is most common, male predominance (2:1)
Complications (due to spinal location) and recurrence (due to incomplete resection)
#SmRBCT=BAD!

53. Bone and Soft Tissue Summary
LCH (EG) often presents with vertebra plana
ES has an extensive soft tissue component, but will look like other SmRBCTs
Other tumors include rhabdomyosarcoma, aggressive hemangioma, metastasis
Infection can be a confounding feature

54. Conclusion
History and demographics are crucial in evaluating pediatric epidural masses
Discerning the tissue of origin is useful in developing an appropriate differential diagnosis
Intrinsic signal characteristics, enhancement, and secondary findings are helpful tools for narrowing potential etiologies of pathology
Keep differentials broad, as many patients fail to read the book!
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55. References Thank you again for browsing through this presentation.
Micheau, A. Illustration on slides 4-5. e-anatomy4, ©IMAIOS 2015
Additional images are credited by website in the notes section of the slide in which they appear
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Thank you again for browsing through this presentation.