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Grand Rounds

Grand Rounds. Carotid Body Tumours. Intro. Tumours Derived from neural crest cells Called nonchromaffin paragangliomas Slow growing tumours. Historical Background. Swiss anatomist Von Haller in 1743 Alfred Kohn coined term paraganglion

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Grand Rounds

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  1. Grand Rounds Carotid Body Tumours

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

  3. HistoricalBackground 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

  6. 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

  7. 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

  8. All well

  9. Patient goes home

  10. 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

  11. We don’t know

  12. How quickly will it grow ? No solid data Reality is imaging will tell us

  13. Imaging ? CT MRI MRI angiogram Angiography U/S Functional scans

  14. Paragangliomas have somatostatin receptors Stage disease Guide therapy Monitor response May provide long term palliation Otolaryngol Head Neck Surg 2000;122:358-62.

  15. 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

  16. How did I get it? Genetics Bad luck Bad luck Bad luck Vs

  17. 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

  18. 7 genes known to cause hereditary paragangliomas or phaeochromocytomas NF I VHL RET MEN II SDHD SDHC SDHB

  19. 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

  20. A Mitochondria complex dysfunction Plasma membrane Mitochondrion Mimics chronic hypoxia at a microscopic level B Functional hyperplasia ?? Loss of oncosuppression

  21. What does the carotid body do ?

  22. What will happen when its gone ? Unilateral – nothing Bilateral – Something ? Anecdotal reports

  23. 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

  24. 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

  25. Conclusion In bilateral tumour resection we should tell patients about the risks of labile blood pressure Timmers

  26. Do I need pre operative embolisation ?

  27. Embolisation Transfemoral under angiographic control

  28. 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.

  29. Type 1 – NO Types 2 and 3 - Yes

  30. 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

  31. Radiotherapy

  32. 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

  33. Mayer et al Strahlenther Onkol 2000 Aug; 176 (8): 356 -60Good as post op in malignant cases – postpone spreadingEradicating existing nodal diseaseProlong local control

  34. Vascular surgery and complications ?

  35. Smith JJ / Netterville JL et alAnn 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)

  36. Thank you

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