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Grand Rounds

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Title: Fellows Rounds Author: OR Last modified by: Mark Corrigan Created Date: 11/4/2006 5:49:34 PM Document presentation format: On-screen Show (4:3) – PowerPoint PPT presentation

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Title: Grand Rounds


1
Grand Rounds
  • Carotid Body Tumours

2
Intro
  • Tumours
  • Derived from neural crest cells
  • Called nonchromaffin paragangliomas
  • Slow growing tumours

3
Historical Background
  • Swiss anatomist Von Haller in 1743
  • Alfred Kohn coined term paraganglion
  • Renamed vascular glomus in early 20th century
  • 1941, Guild described glomic tissue

4
  • Glenner and Grimley distingushed adrenal and
    extra-adrenal paraganglionma
  • CURRENTLY, PARAGANGLIOMA BASED ON ANATOMICAL
    LOCATION IS PROPER TERMINOLOGY (e.g. carotid
    paraganglioma, jugulotympanic paraganglioma)

5
Site
  • 90 pheochromocytoma
  • 10 extra-adrenal
  • 85 abdomen
  • 12 thorax
  • 3 head and neck
  • Carotid body most common
  • Jugulotympanic
  • Vagal
  • Other

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8
Shamblin Classification
  • Shamblin 1971
  • Type I
  • Localized easily resected
  • Type II
  • Adherent partially surrounding vessels
  • Type III
  • Completely encased carotids
  • 70 are type II or III

9
Imaging
  • MRI / MRA / Angio
  • Vascular insight
  • Occult tumors-0.8 cm
  • T1, T1 post gad, T2, axial FLAIR, FSE T2
  • Skull base to thoracic inlet

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15
All well
16
Patient goes home
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18
Is it cancer ?
Local recurrence Metastases seen to lungs, lymph
nodes, and bones Malignant nature cannot be
predicted by microscopic characteristics, such as
nuclear atypia and mitotic rate. Determined by
the presence of local invasion or distant
metastasis, Mets can be late Zellballen
19
We dont know
20
How quickly will it grow ?
No solid data Reality is imaging will tell us
21
Imaging ?
  • CT
  • MRI
  • MRI angiogram
  • Angiography
  • U/S
  • Functional scans

22
Paragangliomas have somatostatin receptors
  • Stage disease
  • Guide therapy
  • Monitor response
  • May provide long term palliation

Otolaryngol Head Neck Surg 2000122358-62.
23
Bomanji JB et al. Middlesex
  • MIBI I (123 / 131)
  • MIBI similar to NA..taken into chromaffrin
    cells.into storage granules
  • But PGL are non chromaffrin ?
  • They also express somatostatin receptors
  • This can be confirmed histologically
  • Pentetreotide / octreotide 111 and subsequent
    labeling with Y 90

24
How did I get it ?
Genetics
Bad luck
Bad luck
Bad luck
Vs
25
Hypoxia (10 times higher in high altitude
African countries)
Sporadic vs Hereditary
Should we screen patients with sporadic head and
neck paragangliomas for hereditary syndromes
? M.D. Anderson Jimenez C et al. 2006 The
Journal Of Clinical Endocrinology and Metabolism
Vol 91 (8) 2851 - 2858
26
7 genes known to cause hereditary paragangliomas
or phaeochromocytomas NF I VHL RET MEN
II SDHD SDHC SDHB
27
Specific to head and neck paragangliomas PGL
genes
Germline mutations in SDHB, SDHC and SDHD cause
hereditary paragangliomas
Some families have hereditary paragangliomas
SDHD (PGL 1) - Chromosome 11q23 SDHB (PGL 4) -
Chromosome 1p36 SDHC (PGL 3) - Chromosome 1q21
(single German family ?? ) PGL (PGL 2) locus
mapped to 11q13 in an extended Dutch family
These encode 3 subunits of MC II Succinate
dehydrogenase
28
A
Mitochondria complex dysfunction
Plasma membrane
Mitochondrion
Mimics chronic hypoxia at a microscopic level

B
Functional hyperplasia
?? Loss of oncosuppression
29
What does the carotid body do ?
30
What will happen when its gone ?
Unilateral nothing Bilateral Something ?
Anecdotal reports
31
Timmers HJ
  • Rare but known to occur in neck after RT for NPC
  • Dutch woman
  • Bilateral carotid body tumour (at same time)
  • Immediate onset of hypertension
  • Reported in Ned Tijdschr 2001

32
Baroreflex sensitivity was significantly decreased
  • 3 had orthostatic hypotension
  • When valsalva done vagal tone was down
  • Normocapnic ventilatory response to hypoxia was
    absent in all

33
Conclusion
  • In bilateral tumour resection we should tell
    patients about the risks of labile blood pressure

Timmers
34
Do I need pre operative embolisation ?
35
Embolisation
Transfemoral under angiographic control
36
Complications are rare.
  • Cerebrovascular accident due to rupture of an
    atheroma with the catheter. The rate is
    approximately 1 in most centres.
  • Prior to using platinum coils for embolisation,
    polyvinyl alcohol particles were used and this
    was associated with stroke due to reflux into the
    internal carotid system during introduction and
    removal of the catheter.
  • Facial pain, trisimus and tonsillar ulceration
    have all been reported.

37
  • Type 1 NO
  • Types 2 and 3 - Yes

38
Bakoyiannis KC et al. Int Angiol. 2006 Mar 25
(1) 40 - 5
  • Athens
  • Pre operative embolisation not necessary
  • 10 year retrospective study
  • 11 patients 12 tumours
  • None embolised
  • Perioperative mortality zero.
  • Out of 9 grade III tumour, 1 had ICA injured and
    vein grafted per op

39
Radiotherapy
40
Valdagni et al. Am J Clin Oncol.1990 Feb 13
(1) 45-8 (Italy)
  • Questioned this theory
  • 13 Carotid body tumours had Rt (mean 52 Gy)
  • 10 - only
  • - In addition
  • Local control (Subjective and objective ) in all
  • 3/13 complete response
  • 7/13 Some response
  • 3/13 No change

41
Mayer et al Strahlenther Onkol 2000
Aug 176 (8) 356 -60 Good as post op in
malignant cases postpone spreading Eradicating
existing nodal disease Prolong local control
42
Vascular surgery and complications ?
43
Smith JJ / Netterville JL et al Ann Vasc Surg.
2006 Jul 20 (4) 435 - 9
  • Retrospective analysis 1990 2005
  • Compared all CBR and CBR vasc.
  • 71 tumours 16 needed vasc. Recon. (23 )
  • Type I usually CBR
  • Type II / III where vasc. Recon needed
  • Cranial nerve damage was higher in recon.(x 2)

44
Thank you
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