1. NEUROSURGICAL CASE PRESENTATION
EXTRADURAL CERVICAL MYXOMATOUS CHORDOMA WITH EXTRA SPINAL EXTENSION AND ENCASEMENT OF THE VERTEBRAL ARTERY
WALID S. MAANI
PROFESSOR AND CHAIRMAN OF NEUROSURGERY
JORDAN UNIVERSITY HOSPITAL
AND MEDICAL SCHOOL
7/29/2018
FIRST SPECIALIST'S PROGRAM

2. CLINICAL PRESENTATION
A 15 years old girl who was admitted to JUH
complaining of:
Neck pain of 3 months’ duration
Numbness and parasthesias of the right upper
limb of three months’ duration
Right sided weakness of two weeks’ duration
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FIRST SPECIALIST'S PROGRAM

3. CLINICAL PRESENTATION
Physical examination revealed:
Weakness of the right upper limb predominant in
the shoulder abduction, wrist extension & elbow
flexion (1/5), with lost biceps and supinator reflexes
There was weakness of the right lower limb (4/5),
with exaggerated reflexes and a Babiniski sign .
The left lower limb was normal in power but the
reflexes were exaggerated and the planter was up.
The rest of the examination was normal
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4. FIRST SPECIALIST'S PROGRAM
NEURO-IMAGING
PLAIN RADIOGRAPHS
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5. FIRST SPECIALIST'S PROGRAM
NEURO-IMAGING
PLAIN RADIOGRAPHS
RIGHT LATERAL
LEFT LATERAL
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6. FIRST SPECIALIST'S PROGRAM
NEURO-IMAGING
COMPUTERIZED TOMOGRAPHY
TRANSVERSE CUTS WITH WIDEND RIGHT FORAMEN TRANSVERSARIUM
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7. FIRST SPECIALIST'S PROGRAM
NEURO-IMAGING
COMPUTERIZED TOMOGRAPHY
3D CT RECONSTRUCTION
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8. CT RECONSTRUCTION SHOWING WIDENING OF THE VERTEBRAL CANAL AND FORAMINA
NEURO-IMAGING
COMPUTERIZED TOMOGRAPHY
CT RECONSTRUCTION SHOWING WIDENING OF THE
VERTEBRAL CANAL AND FORAMINA
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FIRST SPECIALIST'S PROGRAM

9. T2 WEIGHTED IMAGES SHOWING EXTENSION THROUGH 2 INTERVERTEBRAL FORAMINA
NEURO-IMAGING
MAGNETIC RESONANCE (MRI)
T2 WEIGHTED IMAGES SHOWING EXTENSION THROUGH
2 INTERVERTEBRAL FORAMINA
C3/4 AND C4/5
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10. FIRST SPECIALIST'S PROGRAM
NEURO-IMAGING
MAGNETIC RESONANCE (MRI)
T2 WIEGHTED SHOWING ENCASEMENT ON RIGHT
T1 WIEGHTED WITH CONTRAST
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11. FIRST SPECIALIST'S PROGRAM
NEURO-IMAGING
MAGNETIC RESONANCE (MRI)
LEFT
T1 WIEGHTED WITH CONTRAST
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12. FIRST SPECIALIST'S PROGRAM
MAGNETIC RESONANCE (MRI)
TI WIEGHTED SAGITTAL WITH CONTRAST
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13. FIRST SPECIALIST'S PROGRAM
NEURO-IMAGING
VERTEBRAL ANGIOGRAPHY
RIGHT VERTEBRAL ARTERY ENCASEMENT
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14. FIRST SPECIALIST'S PROGRAM
NEURO-IMAGING
VERTEBRAL ANGIOGRAPHY
RIGHT VERTEBRAL ARTERY ENCASEMENT
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15. FIRST SPECIALIST'S PROGRAM
SURGICAL MANAGEMENT
FIRST STAGE OPERATION
The patient underwent a C3 –C5 laminectomy
in the sitting position on the 1st of July.
The intra-spinal component of the tumor was
excised completely.
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16. FIRST SPECIALIST'S PROGRAM
SURGICAL MANAGEMENT
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17. FIRST SPECIALIST'S PROGRAM
SURGICAL MANAGEMENT
The tumour was grey blue in colour
It was soft in nature
Was removed completely by suction
It passed through the intervertebral foramina on the right around the roots and was removed as far laterally as possible
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18. FIRST SPECIALIST'S PROGRAM
HISTOPATHOLOGY
The histopathology examination showed features of
Myxomatous Chordoma.
CHORDOMA
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19. PREPARATION FOR THE SECOND STAGE
The patient had an occlusion test of the right
vertebral artery two weeks later which proved
that the artery could be ligated if needed
without consequences.
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20. FIRST SPECIALIST'S PROGRAM
SURGICAL MANAGEMENT
SECOND STAGE OPERATION
The Head and Neck surgery team went on the 28th of
July and through a Mcaffey’s incision
excised the extra-spinal part without sacrificing the
vertebral artery, the foramina transversaria of C4-C6
were opened and the artery dissected and freed and
the tumor was removed macroscopically
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21. FIRST SPECIALIST'S PROGRAM
SURGICAL MANAGEMENT
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22. FIRST SPECIALIST'S PROGRAM
SURGICAL MANAGEMENT
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23. FIRST SPECIALIST'S PROGRAM
SURGICAL MANAGEMENT
THE CERVICAL COMPONENT
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24. FIRST SPECIALIST'S PROGRAM
HISTOPATHOLOGY
CHORDOMA
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25. FIRST SPECIALIST'S PROGRAM
HISTOPATHOLOGY
A light microscopic view of a hematoxylin and eosin (H&E) stained section of a chordoma showing the characteristic physaliphorous cells and mucinous matrix.
A higher magnification light microscopic view of a hematoxylin and eosin (H&E) stained section of a chordoma showing physaliphorous cells
7/29/2018
FIRST SPECIALIST'S PROGRAM

26. POST-OPERATIVE COMPLICATIONS
The patient developed
a partial right Horner’s
syndrome and some
weakness of right
shoulder abduction
following this surgery.
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27. FIRST SPECIALIST'S PROGRAM
ADJUVENT THERAPY
The patient was discussed in the inter
departmental meetings and agreement was
reached on giving a dose of radio-therapy to
this radio-resistant tumor.
Radiotherapy was delivered at the King
Hussein Cancer Center on Oct 5th.
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28. FIRST SPECIALIST'S PROGRAM
50.4 Gy
28 FRACTIONS
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29. POST OPERATIVE INSTABILITY
ONE YEAR AFTER SURGERY PLAIN X-RAYS SHOWING SWAN
NECK DEFORMITY FOLLOWING 3 LEVEL LAMINECTOMY
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30. POST OPERATIVE INSTABILITY
FELXION & EXTENSION VIEWS OF THE INSTABILITY
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31. POST OPERATIVE INSTABILITY
CT RECONSTRUCTION SHOWING SWAN NECK DEFORMITY
(EXTENT OF LAMINECTOMY IS SEEN)
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32. POST OPERATIVE INSTABILITY
MRI (T1 WEIGHTED) SHOWING SWAN NECK DEFORMITY
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33. POST OPERATIVE INSTABILITY
MRI (T2 WEIGHTED) SHOWING SWAN NECK DEFORMITY
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34. FIRST SPECIALIST'S PROGRAM
SURGICAL MANAGEMENT
POST OPERATIVE FELXION AND EXTENSION VIEWS
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35. FIRST SPECIALIST'S PROGRAM
SURGICAL MANAGEMENT
ANTERIOR CERVICAL FUSION WITH 2 CAGES , PLATE AND 6 SCREWS
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36. FIRST SPECIALIST'S PROGRAM
FOLLOW-UP
CLINICAL
ALL FUNCTIONS RETURNED TO NORMAL
THE PATIENT RETURNED TO SCHOOL
SHE IS ATTENDING REGULAR OPD CLINICS
RADIOLOGICAL
MRI: NO INTRASPINAL COMPONENTS
MRI: SMALL EXTRASPINAL NODULE
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37. FIRST SPECIALIST'S PROGRAM
CHORDOMAS
Rare tumors (histologically benign) forming <1% of CNS tumors, but around 3% of bone tumors.
First described by Virchow in 1857 and called them
ecchondrosis physaliphora, believing they were
cartilaginous in origin
In 1895, Ribbert pierced a nucleus pulposus and
found similar tumors. From this bit of evidence, he
correctly surmised the notochordal origin of
chordomas
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38. FIRST SPECIALIST'S PROGRAM
CHORDOMAS
Arise from remnants of the notochord.
Occur anywhere from sphenoid to coccyx.
Common at basi-occiput (30%)and sacrococcygeal
regions (50%), spinal axis tumors are very rare.
Vertebral chordomas form 15% and occur in lumbar, cervical and thoracic regions in that order.
They invade and destroy bone (locally invasive and
rarely metastasize).
Occur at any age but mainly 4th. decade.
Most patients die within 10 years of presentation
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FIRST SPECIALIST'S PROGRAM

39. FIRST SPECIALIST'S PROGRAM
CHORDOMAS
A light microscopic view of a hematoxylin and eosin (H&E) stained section of a chordoma showing the characteristic physaliphorous cells and mucinous matrix.
A higher magnification light microscopic view of a hematoxylin and eosin (H&E) stained section of a chordoma showing physaliphorous cells
7/29/2018
FIRST SPECIALIST'S PROGRAM

40. FIRST SPECIALIST'S PROGRAM
CHORDOMAS
Diagnosis is by:
Imaging
Exclusion
FNA of tumor
Treatment:
Surgical
Radiation
Medical: Clinical trials are underway to study the effectiveness
of imatinib mesylate in the treatment of chordoma. Imatinib
mesylate is a tyrosine kinase inhibitor targeting several
enzymes including platelet-derived growth factor receptor--b
(PDGFRB), which can be expressed in chordomas. This drug
has been shown to have antitumor activity in chordomas
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FIRST SPECIALIST'S PROGRAM

41. FIRST SPECIALIST'S PROGRAM
CHORDOMAS
Frequent follow-up is required because of the high rate of
recurrence of these tumors.
Tumor recurrence identified early is easier to treat.
The average interval to recurrence is 3.8 years for radically
resected tumors, 2.1 years for subtotal resection followed by
radiation therapy, and 8 months for subtotal excision without
adjuvant therapy.
The interval of follow-up, including repeat MRI or CT scans,
depends on the completeness of the resection
Because residual tumor drastically shortens the recurrence time,
patients with known or suspected residual tumor need to be
evaluated more frequently.
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42. FIRST SPECIALIST'S PROGRAM
CHORDOMAS
The 5-year survival rate is estimated to be
51%, and the 10-year survival is estimated
to be 35%. Factors that may improve
prognosis are young age, complete resection,
and the addition of radiation therapy in
incompletely resected tumors
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43. FIRST SPECIALIST'S PROGRAM
THANK YOU
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