Vascular Spinal Rarity

1. A Spinal Rarity
Rohit Sinha
Dr Rajendra Prasad

2. The case of Mr IK
A 35 yr old Bangladeshi stock-
broker and M.Phil student
presented with a 6/12 Hx of:
Excruciating, constant lower back
pain; gradually increasing in
severity.
Urinary and faecal constipation.
Poor sleep

3. Previous Pathologies
Diagnosed with Diabetes Mellitus in
November 2005
Previously underwent spinal surgery
for a what was presumed to be a
ependymoma arising from the filum
terminale L1- L3.(18/09/1995)

4. Preoperative MRI from 1995

5. A T1 weighted
saggital MR image
of the spine
showing a well
circumscribed
tumour from L1 to
L3 with multiple
flow voids at the
superior pole of
the tumour

7. Other history features
Mr IK takes Gabapentin to relieve his
‘shock like’ neuropathic pain.
Nil relevant Family or Social Hx
Subjectively, Mr IK is most concerned
about:
1.) His anticipation of walking problems
2.) His current constipation
3.) His disturbed sleep patterns due to
worsening pain

8. Salient examination findings
Felt unstable on heel-toe walking
Normal joint position sense
Normal power, tone, reflexes and co-
ordination (heel-shin drag)
Experiences pain on dorsiflexion
Has recently noticed that he can no
longer stand on tip toes.

9. Investigations – MR Spine Imaging
MRI lumbosacral spine
report:
‘An elongated, moderately
enhancing, lobulated,
intradural, altered signal
intensity mass lesion is
seen from mid L4 to S3
level. Remaining cauda
equina nerve roots L1 to L4
level appear thickened,
distorted and clumped.’

10. The T1 post contrast
image (right) shows a
lobulated tumour
extending from L4 – S3,
possibly with two
components, a lumbral
part and a sacral part.

11. Further imaging investigation
The previous MR images showing multiple flow
voids prior to the initial operation are suggestive of a
highly vascular tumour
Cystic/highly vascular components are common at
the superior pole of such tumours (Aggarwal et al,
1993)
Selective spinal angiography has been
recommended by some authors for the assessment
of vascularity; even combined with preoperative
embolization to minimise intraoperative bleeding
(Aghakhani et al, 1999)

12. Pre-Embolisation image

13. Post-Embolisation image

14. The Operative Intervention
A Laminectomy and tumour decompression was
carried out at levels L4-S3
Intra-operatively, the tumour was completely
confined within the dura
Many nerve roots were incorporated by the tumour
The tumour was soft, friable and highly vascular
despite embolisation
There was no local infiltration by the tumour into
adjacent structures
Biopsy was taken for histopathological analysis

15. Histopathological biopsy slide 1
‘…Cells have
moderate to
abundant
granular ,
eosinophilic
cytoplasm…
mildly
pleomorphic
nuclei…
occasional
mitotic figure is
seen’

16. Histopathological biopsy slide 2
‘A layer of spindle
shaped
Sustentacular cells
is seen… cells are
positive for
synaptophysin …
histology is that of
a paraganglioma.
Note: in view of the
presence of foci of
necrosis, the
tumour may behave
aggressively…’

17. A brief literature review
Incidence: Extremely rare.
First case reported by Miller & Torrack in 1970,
since then only 155 cases have been added to the
literature until 2003.
Age of patients: Range 12 yrs to 71 yrs
Sex: No gender preponderance
Common sites:
90% of paragangliomas occur in the carotid body
and glomus jugulare Other CNS sites include the
sella turcica, pineal gland, and CP angle.
Spinal paraganglioma are most commonly located
at the lumbar spine followed by the cauda equina
& filum terminale

18. Aetiology & Pathophysiology
No concrete aetiology but current research
implicates mutations in the SDHD gene and a
familial autosomal dominant trait (Masuoka et
al, 2001)
Associated with MEN syndrome (Kleiwer &
Cochran, 1989)
Unclear whether these are true neoplasia,
hyperplasia or hamartomas or even if they
actually derive from ectoderm rather than
‘misplaced endoderm’ (Gaffney et al, 1986)

19. Clinical Features
The main symptom is low back pain (in 90% of
cases)
Sciatica accompanies this in 72% of cases
Mechanical compression is the pathophysiological
factor, but episodes of blood leakage from the
tumour are also implicated (Horoupian et al, 1974)
Sensory and motor deficits are found in 35% of
cases; 20% show symptom progression
Sphincter dysfunction occurs in 14% of cases and
erectile dysfunction has also been reported.
Systemic manifestations are seldom seen.

20. Diagnosis and Investigations
No particular investigations to document its
preoperative diagnosis, but MRI is the investigation of
choice (Miliaras et al, 2003)
More common considerations for the differential
diagnosis include ependymoma and schwannoma. No
Hx or imaging clues to differentiate (systemic amine
syndromes are unusual).
MRI features: remarkable, heterogenous enhancement
with a vascular/cystic component.
T2 hypointense margins of intradural, well
encapsulated tumours attributed to haemosiderin or
ferritin from previous haemorrhages (Taira et al, 2000)

21. Images from the Miliaras et al, 2003

22. Other investigations
Selective spinal angiography has been
recommended by some authors for the assessment
of vascularity; even combined with preoperative
embolization to minimise intraoperative bleeding
(Aghakhani et al, 1999)
CSF protein levels are elevated as with all lower
spinal tumours as noticed by Cushing in 1923.
Intraoperatively, 80% of lesions are encapsulated;
only 15% are locally invasive.

23. Treatment
Total excision is the Gold standard for tumours of
such protracted course; since these are usually
encapsulated this goal is more realistic.
An important consideration for Mr IK is his previous
surgery which may mean the tumour is not
completely encapsulated making total excision more
difficult/invasiveness more likely.
Such tumours are highly vascular, friable and
haemorrhagic. 85% are attached to the filum
terminale, or also to nerve rootlets; parts of which
may have to be sacrificed with variable
postoperative sequelae (Miliaras et al, 2003)

24. Post operative considerations
After SUBtotal removal 12% show regrowth; latest
recorded progression being 30 years postoperatively
(Boker et al, 1983)
4 cases are reported in which CSF dissemination has
occurred; all had been preceded by an operation.
Postoperative radiotherapy is advocated by some
authors for incompletely excised or non-encapsulated
tumours; although prevention of tumour recurrence as
such has never been demonstrated (Sonneland et al,
1986).
‘Reliable histological criteria to distinguish benign
from malignant variant do not exist (Miliaras et al,
2003)
Spinal paragangliomas are classified as grade 1
tumours according to the WHO; less than 1% are

25. Key References
J Neurooncol. 2003; 65(2):177-90 (ISSN: 0167-594X)
Miliaras GC; Kyritsis AP; Polyzoidis KS
Neurosurgical Institute, University of Ioannina,
Department of Neurosurgery, Medical School, Ioannina, Greece.
Aggarwal S, Deck JH, Kucharczyk W: Neuroendocrine
tumor (Paraganglioma) of the cauda equina:MRand pathologic
findings. AJNR Am J Neuroradiol 14: 1003–1007,
1993
Aghakhani N, George B, Parker F: Paraganglioma of the
cauda equina region – Report of two cases and review of
the literature. Acta Neurochir (Wien) 141: 81–87, 1999
B¨oker DK, Wassmann H, Solymosi L: Paragangliomas of
the spinal canal. Surg Neurol 19: 461–468, 1983
Gaffney EF, Doorly T, Dinn JJ: Aggressive oncocytic
neuroendocrine tumour (‘ocncocytic paraganglioma’) of
the cauda equina. Histopathology 10: 311–319, 1986