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ESBS 2005: Skull Base Surgery: An Interdisciplinary Challenge
7th Congress of the European Skull Base Society held in association with
the 13th Congress of the German Society of Skull Base Surgery

18. - 21.05.2005, Fulda, Germany

Paragangliomas of the skull base – a South African experience: 120 cases 1982–2004

Meeting Contribution

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  • B. Le Roux - Dept. of Otorhinolaryngology, Pretoria University Medical School, Lyttelton, South Africa

ESBS 2005: Skull Base Surgery: An Interdisciplinary Challenge. 7th Congress of the European Skull Base Society held in association with the 13th Congress of the German Society of Skull Base Surgery. Fulda, 18.-21.05.2005. Düsseldorf: German Medical Science GMS Publishing House; 2009. Doc05esbs37

DOI: 10.3205/05esbs37, URN: urn:nbn:de:0183-05esbs371

Published: January 27, 2009

© 2009 Le Roux.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by-nc-nd/3.0/deed.en). You are free: to Share – to copy, distribute and transmit the work, provided the original author and source are credited.


Outline

Text

Glomus tumours: a South African experience

This report deals with 120 cases of paragangliomas seen and treated in a private practice with a small University appointment in Pretoria from 1982–2004. All the cases were seen, operated and followed up by the same surgeon. Although rare, these cases occur regularly in South Africa. Pretoria lies in the North-Eastern part of the country at an altitude of 1370 metres above sea level. Most of the cases come from areas 1200–1800 metres above sea level.

90% of the cases were treated surgically and 72% were female. 8% of the cases had a family history and in 9% we found vaso active amine secreting tumours. The familial cases all came from the Afrikaans speaking community who have strong Dutch, German and French ties. 24% were from the indigenous black community and 3% were of Indian descent.

The duration fo symptoms varied between 2 months–20 years with an average of 4 years. Previous surgery were performed in 25% of cases this included biopsios, myringotomies, mastoidectomies and limited skull base surgery and neurosurgical interventions.

Previous radiotherapy were given in 11% of cases. In all these cases further tumour growth took place and surgery was much more difficult.

The age group distribution was from 20 years to 75 years with a mean of 47 years.

All cases were classified according to the Fisch Classification and operated on using techniques described by Prof U. Fisch [1], [2], [3], M.E Glassock and Dr C. Jackson [4] and Dr D. Brackmann [5].

Classification [Fisch]: operated cases: 108 [90%]

Class A–13% [14]; Class B–22% [24]; Class C & D 65% [70]; Most cases fell in the 2C Group; Class D1 and D2–cases represented 12% of cases [9]; Class D1 and D2–12 cases 11%.

Presenting symptoms: 120 cases

Hearing loss was the most prominent presenting symptom. Pulsatile tinnitus were present in 88% of cases and the other presenting symptoms were more or less in line with previously published series.

Physical finds

Middle ear blush or mass was present in 78% of cases. Tumour presented in the external canal in 10% of cases. 6 patients had carotid body tumours. The most common pre-operative Cranial Nerve involvement cases was hearing loss in 83% of cases while the jugular foramen nerves were involved. The 10th nerve was involved in 36% of cases and all the others in about 15% of cases.

Pre-operative procedures : 108 cases

All cases had CT scans with contrast. Class C & D tumours had MRI and Gd contrast. 4 Vessel angiography plus embolisation were performed in 89% of class C & D tumours–24 hrs–7 days pre-op. MR Angiography was done in 11%.

Operative procedures

All Class A tumours were removed transcanal. Class B tumours had a mastoidectopmy and extensive hypo-tympanectomy plus reconstruction of the ossicular chain. Class C & D tumours had the earcanal occluded in all cases and the facial neve transposed.

Average hospital stay was 7 days. The range of 2–21 days.

Complications

Most were mild and decreased as experience was gained.

Cerebro spinal fluid leaks were the most common – 7% of cases [8]. Gustatory pain occured in 5% of cases but usually disappeared after 6-18 months and responded well to Tegretol. Only one case required a tracheotomy after surgery. One injury to the carotid artery could be dealt with without any lasting defects.

Deaths

[1] 2 cases [2%]. Aspiration – One due to aspiration on the 3rd post-operative day and consequent cardiac arrest. Resuscitated and declared brain dead after 5 days.

[2] Pneumococcal meningitis – 2 weeks after cerebro spinal fluid leak which was repaired. Re-admitted in coma and died after 2 weeks.

Results class C & D Tumours N = 70

Facial Nerve

Complete transposition was done in 86% of cases with partial transposition in 7%. 6% of cases required greater auricular or N Suralis grafts and 7% underwent facial hypoglossal anastomoses.

Average recovery after 4 months of rerouted nerves Nr 65 were House Brackmann Gr 1 and 11 in 84% of cases. Recovery still continued up to one year after transposition and 88% of cases improved to Grd 1, 11 and 111.

Post-operative hearing results

Class A tumours–13% [14] had hearing improved or restored in all cases. Class B tumours – 22% [24] was improved or normal in 70% of cases. 24% [6] had a conductive loss of about 30dB and [1] complete loss. All the Class C & D tumours 65% [70] had a 60dB conductive loss. Total loss due to cochlear involvement occurred in 12% of cases [8]. Only one case had unchanged hearing. [Glomus Vagale] 2% [2].

Jugular Foramen Nerves - N = 108

Complete loss after surgery occurred in 10% of cases [7]. Post-operatively we had 60% loss of NXI. 43 cases [10] 60% loss [42] - [11] 44% loss [31] - [12] 28% loss [20]–2 recovered after 2 years. Most of these patients were able to eat and drink satisfactory after 4 months and many after only 6 weeks. Patients with symptoms prior to surgery had very little trouble after surgery with aspiration.

Tumor free status after 5 years - N = 50

Complete removals were possible in 100% of Class A tumours, 91% of Class B tumours, 93% of C1; C2 – 85%; C3 – 75%. In familial cases completely removal was only possible in 45% of cases. Class D1 [e] tumours had complete removal in 90% of cases. Class D1 and D2 – 60%.

Discussion

The results presented here are not perfect but the vast majority of patients accepted cranial nerve deficits and occlusion of the ear canals without complaining. The facial nerve results were also accepted but in two cases plastic surgery for reanimation was suggested and performed. Vocal cord paralysis were treated by thryroplasty procedures.

It must be emphasized that this type of surgery is a team effort between Surgeon, Anaethetist, Physician, Radiologist, Neuro Radiologist, Interventional Radiologists, Neuro Surgeon and Plastic Surgeon, dedicated nursing and theatre personnel as well as intensive care personnel is required. Physiotherapist, speech and swallowing therapist complete the team. With this team approach and modern techniques most Glomus tumours can be successfully treated by surgery. Early diagnosis and early surgery is required and this is gradually improving in South Africa.

Embolisation is safe and beneficial and in expert hands and make surgery easier and safer. No serious complications occurred in this series.

Radiotherapy is reserved for the old and infirm, in operable cases, patients who refuse surgery and recurrent tumours. Gamma knife treatment holds promise in these cases. Onse case developed recurrent tumour after 8 years and was treated with radiotherapy. Four years later he reverted to a Synovial Sarcoma.

Lastly, follow-up 10-15 years is advised because recurrences did occur even 12–14 years after surgery. Genetic studies are being done on 60 cases and will hopefully give further information on these interesting, challenging tumours.


References

1.
Fisch U, Mattox DE. Microsurgery of the skull base. Stuttgart: Georg Thieme Verlag; 1988.
2.
Fisch U. Infratemporal fossa approach to tumours of the temporal bone and base of the skull. J Laryngol Otol. 1978;92(11):949-67.
3.
Fisch U, Fagan P, Valavanis A. The infratemporal fossa approach for the lateral skull base. Otolaryngol Clin North Am. 1984;17(3):513-52.
4.
Glassock ME, Jackson CG. Neuro otologic skull base surgery for Glomus Tumours. Laryngoscope. 1993;103(II Pt2).
5.
Brackmann DE, Arriaga MA. Surgery for Glomus tumors. In: Brackmann DE, Shelton C, Arriaga MA (eds) . Otologic surgery. Phaladelphia: W.R Saunders; 1994. p. 579.