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Carotid Body Tumors:
Review of 56 cases
Amr Gad,MD
Prof. of Vascular Surgery
Cairo University
Carotid Body Tumors
 Arise from paraganglionic cells derived
from the neural crest
 >50% of neck paragangliomas (PGLs)
 Incidence less than 1 in 30 000
Disastrous History…
1743 Von
Haller
first described the carotid body
1880 Reigner attempted excision, but the patient died.
1886 Maydl attempted excision but the patient
developed a stroke.
1889 Albert first successful CBT excision
1891 Marchand first description of the histologic
appearance
1903 Scudder first successful resection in USA
1903 Kohn first introduced the term paraganglioma
(PGL)
Till 1950s mortality and morbidity remained very high
Patients
54
Patients
( 25 y )
2
Bilateral
56
CBTs
56 CBTs
39 (69.64%)
In males
17 (30.36%)
In females
Age 42 y
range( 32 : 47)
Patients
54
Patients
(25 y )
2
Bilateral
56
CBTs
GROUP A
35(62.5%)
CORRECT
DIAGNOSIS
56 CBTs
GROUP B
21(37.5%)
MISS
DIAGNOSIS
(Prior trial of
excision)
Radiologic
Imaging
Shamblin’s Classification
(1971)
I=27 (48.21%) II=22 (39.29%) III=7 (12.5%)
Suggested Modification by Luna-Ortiz et al
Eur Arch Otorhinolaryngol (2006) 263: 171–175
Dissection Technique
(Periadventitial Plane)
Vascular Reconstruction was
Inevitable in 10 cases(17.85%)
54
Patients
2 bilateral
56
CBTs
56 CBTs
46 Resection
10
Resection +VR
Vein interposition graft 7
End to end anastomosis 1
lateral sutures 2
Vascular Reconstruction was Inevitable
in 17.85% of our cases
 This emphasizes the need for vascular experience among
the operating team.
 A multicenter review of CBTs reported that 80% of cases
were operated on by vascular surgeons.
Sajid et al, Eur J Vasc Endovasc Surg 34, 127-130 (2007)
 It was obvious that vascular reconstruction is
significantly higher among patients with prior removal
attempts (9/21 vs. 1/35, p<0.05), reflecting the
difficulties that may be encountered when operating on a
CBT that was not correctly identified from the beginning.
n=10
Vascular Reconstruction was
Inevitable in 17.85% of our cases
Vascular Reconstruction was
Inevitable in 17.85% of our cases
Complication Number (%)
Cerebrovascular stroke (transient
hemiparesis) 2 (3.57%)
TIA 2 ( 3.57%)
Hypoglossal nerve injury 6 (10.71%)
Superior laryngeal nerve injury 1 (1.78%)
Hematoma 1 (1.78%)
Total 12 (21.43%)
Postoperative Complications
Permanent 3.57%
Study name & year Number of
operated CBTs
CNI %* Vascular
reconstruction %
CVA%
Total Permanent
Studies between 1980-2000
Rosen et al (1981)15 29 28 NA NA NA
Lees et al (1981)16 41 NA 18 NA NA
Dickinson et al (1986)17 37 19 NA NA 4
Gaylis et al (1987)18 52 7 NA 13 2
Hallett et al (1988)13 139 30-46† NA 33 3-23†
Williams et al (1992)19 33 13 3 NA 27‡
Netterville et al (1995)20 46 13 NA 26 0
Muhm et al (1997)21 28 32 NA 36 NA
Rodriguez-Cuevas et al (1998)22 80 20 NA NA 4
Westerband et al (1998)23 31 13 NA 25 6
Wang et al (2000)24 36 41 24 NA 0
Studies between 2000-2010
Makeieff et al (2008)10 57 42 14 28 14‡
Sajid et al (2007)8 95 19 1 NA 1
Qin et al (2009)25 33 53 NA 30 NA
Plukker et al (2001)26 45 11 7 13 10
Papaspyro et al (2009)27 40 NA§ NA§ 25 0
van der Bogt et al (2008)12 111 21-42† 7-26† 2-4† 0
Patetsios et al (2002)28 34 46 17 28 0
Luna-Ortiz et al (2005)1 69 49 38 6 4
Dardik et al (2002)29 27 33 NA 41 4
Ma et al (2009)2 55 27 6 11 4
Kakkos et al (2009)14 41 32 10 18 7
Koskas et al (2009)30 39 NA§ 15 22 3
Ünlü et al (2009)31 28 3 NA 29 0
Paris et al (2006)32 29 NA§ NA§ 14 0
Preoperative Embolization
Attractive Option Prior to Surgery to Reduce Bleeding
and Tumor Bulk.
 Against:
o Associated with an inflammatory response that makes precise
periadventitial dissection more difficult .
Netterville et al Laryngoscope 1995;105(2):115-26
o A risk of intracranial embolization
Makeieff et al, Annals of Surgical Oncology 2008 ;15(8):2180-2186
o It did not affect the probability of need for vascular repair
Smith et al Ann Vasc Surg 2006;20:435-9
o Can be quite tedious and hazardous.
Currently, recommended only for:
o Tumors that are large (5cm in size)
o Shamblin’s class III
o Extend significantly cranially
Kakkos et al, J Vasc Surg 2009;49:1365-73
Preoperative Embolization
Covered Stent Placement of
ECA Scanlon et al, J Vasc Surg 2008;48:1322-4
Radiotherapy
 Radiosensitive but “total resolution” of the tumors
is rare.
 Local control means stability (or regression)
Possible indications:
 Minimal residual tumors
 Tumor destaging.
 Non-resectable tumors
Rodrı´guez-Cuevas et al, Head Neck,(1998) 20: 374–378
Luna-Ortiz et al, Oral Oncology (2005) 41, 56–61
 Our patients’ showed characteristics similar to Latin
American series as:
● Lower incidence of malignancy (4%vs.>10%)
● Lower incidence of bilaterality (9%vs.>10%)
● Lower incidence of familial cases (0%)
 This could reflect a potential genetic pattern which needs
to be further investigated.
Conclusion
 CBTs should be resected by surgeons with
experience in carotid reconstruction.
 Correct preoperative diagnosis and planning
for treatment are essential to avoid
complicated surgical procedures.
 CNI continue to appear in all studies in spite
of the advances in the management and
techniques, but they present minor morbidity.
Cairo University
School of Medicine
Thank You

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Carotid body tumors review of 56 cases

  • 1. Carotid Body Tumors: Review of 56 cases Amr Gad,MD Prof. of Vascular Surgery Cairo University
  • 2. Carotid Body Tumors  Arise from paraganglionic cells derived from the neural crest  >50% of neck paragangliomas (PGLs)  Incidence less than 1 in 30 000
  • 3. Disastrous History… 1743 Von Haller first described the carotid body 1880 Reigner attempted excision, but the patient died. 1886 Maydl attempted excision but the patient developed a stroke. 1889 Albert first successful CBT excision 1891 Marchand first description of the histologic appearance 1903 Scudder first successful resection in USA 1903 Kohn first introduced the term paraganglioma (PGL) Till 1950s mortality and morbidity remained very high
  • 4. Patients 54 Patients ( 25 y ) 2 Bilateral 56 CBTs 56 CBTs 39 (69.64%) In males 17 (30.36%) In females Age 42 y range( 32 : 47)
  • 5. Patients 54 Patients (25 y ) 2 Bilateral 56 CBTs GROUP A 35(62.5%) CORRECT DIAGNOSIS 56 CBTs GROUP B 21(37.5%) MISS DIAGNOSIS (Prior trial of excision)
  • 7.
  • 8.
  • 10. Suggested Modification by Luna-Ortiz et al Eur Arch Otorhinolaryngol (2006) 263: 171–175
  • 11.
  • 13. Vascular Reconstruction was Inevitable in 10 cases(17.85%) 54 Patients 2 bilateral 56 CBTs 56 CBTs 46 Resection 10 Resection +VR Vein interposition graft 7 End to end anastomosis 1 lateral sutures 2
  • 14. Vascular Reconstruction was Inevitable in 17.85% of our cases  This emphasizes the need for vascular experience among the operating team.  A multicenter review of CBTs reported that 80% of cases were operated on by vascular surgeons. Sajid et al, Eur J Vasc Endovasc Surg 34, 127-130 (2007)  It was obvious that vascular reconstruction is significantly higher among patients with prior removal attempts (9/21 vs. 1/35, p<0.05), reflecting the difficulties that may be encountered when operating on a CBT that was not correctly identified from the beginning.
  • 16. Vascular Reconstruction was Inevitable in 17.85% of our cases
  • 17. Complication Number (%) Cerebrovascular stroke (transient hemiparesis) 2 (3.57%) TIA 2 ( 3.57%) Hypoglossal nerve injury 6 (10.71%) Superior laryngeal nerve injury 1 (1.78%) Hematoma 1 (1.78%) Total 12 (21.43%) Postoperative Complications Permanent 3.57%
  • 18. Study name & year Number of operated CBTs CNI %* Vascular reconstruction % CVA% Total Permanent Studies between 1980-2000 Rosen et al (1981)15 29 28 NA NA NA Lees et al (1981)16 41 NA 18 NA NA Dickinson et al (1986)17 37 19 NA NA 4 Gaylis et al (1987)18 52 7 NA 13 2 Hallett et al (1988)13 139 30-46† NA 33 3-23† Williams et al (1992)19 33 13 3 NA 27‡ Netterville et al (1995)20 46 13 NA 26 0 Muhm et al (1997)21 28 32 NA 36 NA Rodriguez-Cuevas et al (1998)22 80 20 NA NA 4 Westerband et al (1998)23 31 13 NA 25 6 Wang et al (2000)24 36 41 24 NA 0 Studies between 2000-2010 Makeieff et al (2008)10 57 42 14 28 14‡ Sajid et al (2007)8 95 19 1 NA 1 Qin et al (2009)25 33 53 NA 30 NA Plukker et al (2001)26 45 11 7 13 10 Papaspyro et al (2009)27 40 NA§ NA§ 25 0 van der Bogt et al (2008)12 111 21-42† 7-26† 2-4† 0 Patetsios et al (2002)28 34 46 17 28 0 Luna-Ortiz et al (2005)1 69 49 38 6 4 Dardik et al (2002)29 27 33 NA 41 4 Ma et al (2009)2 55 27 6 11 4 Kakkos et al (2009)14 41 32 10 18 7 Koskas et al (2009)30 39 NA§ 15 22 3 Ünlü et al (2009)31 28 3 NA 29 0 Paris et al (2006)32 29 NA§ NA§ 14 0
  • 19. Preoperative Embolization Attractive Option Prior to Surgery to Reduce Bleeding and Tumor Bulk.  Against: o Associated with an inflammatory response that makes precise periadventitial dissection more difficult . Netterville et al Laryngoscope 1995;105(2):115-26 o A risk of intracranial embolization Makeieff et al, Annals of Surgical Oncology 2008 ;15(8):2180-2186 o It did not affect the probability of need for vascular repair Smith et al Ann Vasc Surg 2006;20:435-9 o Can be quite tedious and hazardous.
  • 20. Currently, recommended only for: o Tumors that are large (5cm in size) o Shamblin’s class III o Extend significantly cranially Kakkos et al, J Vasc Surg 2009;49:1365-73 Preoperative Embolization
  • 21. Covered Stent Placement of ECA Scanlon et al, J Vasc Surg 2008;48:1322-4
  • 22. Radiotherapy  Radiosensitive but “total resolution” of the tumors is rare.  Local control means stability (or regression) Possible indications:  Minimal residual tumors  Tumor destaging.  Non-resectable tumors
  • 23. Rodrı´guez-Cuevas et al, Head Neck,(1998) 20: 374–378 Luna-Ortiz et al, Oral Oncology (2005) 41, 56–61  Our patients’ showed characteristics similar to Latin American series as: ● Lower incidence of malignancy (4%vs.>10%) ● Lower incidence of bilaterality (9%vs.>10%) ● Lower incidence of familial cases (0%)  This could reflect a potential genetic pattern which needs to be further investigated.
  • 24. Conclusion  CBTs should be resected by surgeons with experience in carotid reconstruction.  Correct preoperative diagnosis and planning for treatment are essential to avoid complicated surgical procedures.  CNI continue to appear in all studies in spite of the advances in the management and techniques, but they present minor morbidity.
  • 25. Cairo University School of Medicine Thank You

Editor's Notes

  1. The carotid body was first anatomically described by Albrecht Von Haller in 1743 [1,2]. Reports of attempted resection of CBT first appeared in the literature in the 1880s. The early cases were disastrous, ending in death from intraoperative hemorrhage or profound neurologic complications [1,3]. Riegner performed the first excision of a carotid body tumor in 1888. The patient did not survive. In 1886, Maydl removed a carotid body tumor; his patient survived with hemiplegia and aphasia. In 1889, Albert was the first to excise a carotid body tumor successfully without ligating the carotid vessels. 1891 Marchand first description of the histologic appearance Scudder reported the first successful removal of a carotid body tumor in the United States in 1903. 1903 Kohn first introduced the term paraganglioma (PGL) Till 1950s mortality and morbidity remained very high
  2. Cutting through the tumour is not recommended due to the possibility of massive bleeding10. However, we were compelled to do this in certain Shamblin’s III tumours, where the surgeon decided that there is a possibility for tumour resection without ICA ligation. This approach was successful in 3 out of the 7 Shamblin’s III tumours, demonstrating that Shamblin’s III tumours do not necessarily require vascular resection. A modified Shamblin’s classification was proposed by Luna-Ortiz et al in 2006 to improve the prediction of difficulties and complications11They proposed a class IIIa which represents the old class III, and a class IIIb which includes tumours of any class (I, II, or III) where there is infiltration of the vessel wall and not just circumferential encasement. The distinction between vascular encasement and vascular infiltration should be clear when interpreting the outcomes of resection of Shamblin III tumors.
  3. Two recent studies adopted a dissection technique starting from the periphery of the tumour and ending at the bifurcation10,12. They considered the posterior surface of the bifurcation the most difficult part of dissection and finished dissection at this point. Their results showed that this technique achieved less blood loss and less persistent CNI. We believe, however, that the most problematic point is the upper part of the tumour which is usually in close contact with ICA and the last four cranial nerves before they diverge in different directions. Most CNI were encountered at this point.
  4. It is assumed that the carotid body is located in the adventitia of the carotid bifurcation3. However, the authors and others2,4,9 believe that it lies in the periadventitial plane. Thus, it is possible to resect the tumour through the existing space between the vessels and the tumour. This anatomic distinction is critical for safe surgical procedures, since dissection in the deeper plane may lead to carotid injury (and repair or resection). The lack of appreciation of this anatomic detail may account for complications4.
  5. We reviewed the publications with more than 25 CBTs and found wide variations in the results, particularly the rate of CNI (Table4). Moreover, we compared the studies published between 1980 and 2000 with those published between 2000 and 2010. We found that the CNI problem continues to exist, since the rates of CNI were more or less the same high figures. The low CNI rates reported in some publications may be due to the fact that they report permanent CNI only, rather than total (permanent and temporary) CNI. The permanent CNI rate is more important because most minor postoperative neuropathies are transient, and patients who suffer them frequently benefit from rehabilitative services3. Other contributing factors to variations in CNI rates include the differences in tumour sizes, Shamblin’s classes, surgical techniques and operators experience among the different studies.
  6. The paragangliomas are radiosensitive but “total resolution” of the tumours is rare. Thus, local control usually means stability (or regression) of tumours size and no progression (or improvement) of neurologic symptoms. Knight et al, 2008 Farr used radiotherapy for minimal residual tumours after incomplete resection. Sajid et al showed that it was used only in one case for tumour destaging. Qin et al and Makeieff et al demonstrated that it is not very effective and should be limited to unresectable tumours to prevent disease progression rather than cure.