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Kopf- und Halsparagangliome

Eine interdisziplinäre Herausforderung

Head and neck paragangliomas

An interdisciplinary challenge

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Zusammenfassung

Hintergrund

Aktuelle Behandlungsstrategien von Kopf- und Halsparagangliomen (KHPG) wenden sich von radikalen Ansätzen ab und favorisieren eine funktionserhaltende Therapie. Strahlentherapeutische Methoden stellen Alternativen in der Primärtherapie dar.

Material und Methoden

Es erfolgte eine PubMed-Recherche der relevanten Literatur bzgl. Genetik und Therapie von KHPG.

Ergebnisse

Der schnelle Fortschritt in der Genforschung beruht auf der Einrichtung zentraler Register für Paragangliompatienten und der Verfügbarkeit von Sequenzierungsmethoden der nächsten Generation. Exomsequenzierung und die Gene-Panel-Sequencing-Methode wurden bereits erfolgreich bei Paragangliomsyndromen angewandt. Letztere Methode ist besonders schnell und kosteneffektiv und wird womöglich in naher Zukunft komplexe Genotypisierungsalgorithmen ablösen. Die Literatur bietet gute Evidenz für diverse moderne Möglichkeiten einer individualisierten Therapie aller KHPG-Manifestationen. Im Allgemeinen sollten kleinere oder symptomatische Tumoren, insbesondere bei jungen Patienten, komplett reseziert werden. Größere Tumoren sollten unter Berücksichtigung von Patientenalter, Symptomen, Morbiditätsrisiko und Komorbiditäten funktionserhaltend operiert werden. Eine primäre Strahlentherapie ist eine gleichwertige Alternative. Eine „Wait and scan“-Strategie ist in selektierten Fällen eine Option.

Diskussion

Die potenzielle Morbidität der chirurgischen Verfahren muss der zu erwartenden Lebensqualität gegenübergestellt werden. Eine umfassende Aufklärung der Patienten über die möglichen Behandlungsoptionen ist obligat. Ein multidisziplinäres Expertenteam ist für eine adäquate Betreuung von Paragangliompatienten zu empfehlen.

Abstract

Background

Current treatment strategies for head and neck paragangliomas are moving away from radical resection and toward surgical tumor reduction, in order to preserve function and reduce morbidity. Radiotherapy modalities are alternative primary treatment options.

Materials and methods

A PubMed search of the relevant literature on genetics and treatment of head and neck paragangliomas was conducted.

Results

The rapid progress made in genetic research was mainly triggered by two factors: firstly, the establishment of central registries for paraganglioma patients and secondly, the availability of next-generation sequencing methods. Exome sequencing and a gene-panel sequencing approach have already been successfully applied to paraganglioma syndromes. The latter method in particular is rapid and cost-effective, and may soon replace complex genotyping algorithms. The literature provides good evidence that diversified modern treatment options are available to realize individual treatment concepts for almost all paraganglioma manifestations. Generally, small and symptomatic tumors should be completely resected, particularly in younger patients. Considering the patient’s age, symptoms, morbidity risk, and comorbidities, larger tumors should be surgically treated in a function-preserving manner. In these cases, primary radiotherapy is an equivalent alternative option. A “wait and scan” strategy is possible in selected cases.

Discussion

The potential morbidity of surgical treatment must be weighed against the expectable quality of life. Comprehensive consultation with the patient about possible treatment modalities is mandatory. Treatment decision making should involve a multidisciplinary team of experts.

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Literatur

  1. Offergeld C, Brase C, Yaremchuk S, Mader I, Rischke HC, Glasker S et al (2012) Head and neck paragangliomas: clinical and molecular genetic classification. Clinics 67(Suppl 1):19–28

    Article  PubMed Central  PubMed  Google Scholar 

  2. Boedeker CC, Ridder GJ, Schipper J (2005) Paragangliomas of the head and neck: diagnosis and treatment. Fam Cancer 4(1):55–59

    Article  CAS  PubMed  Google Scholar 

  3. Kunzel J, Iro H, Hornung J, Koch M, Brase C, Klautke G et al (2012) Function-preserving therapy for jugulotympanic paragangliomas: a retrospective analysis from 2000 to 2010. Laryngoscope 122(7):1545–51

    Article  PubMed  Google Scholar 

  4. Langerman A, Athavale SM, Rangarajan SV, Sinard RJ, Netterville JL (2012) Natural history of cervical paragangliomas: outcomes of observation of 43 patients. Arch Otolaryngol 138(4):341–345

    Article  Google Scholar 

  5. Lee JH, Barich F, Karnell LH, Robinson RA, Zhen WK, Gantz BJ et al (2002) National cancer data base report on malignant paragangliomas of the head and neck. Cancer 94(3):730–737

    Article  PubMed  Google Scholar 

  6. Papaspyrou K, Mewes T, Rossmann H, Fottner C, Schneider-Raetzke B, Bartsch O et al (2012) Head and neck paragangliomas: report of 175 patients (1989-2010). Head Neck 34(5):632–637

    Article  PubMed  Google Scholar 

  7. Dahia PL (2014) Pheochromocytoma and paraganglioma pathogenesis: learning from genetic heterogeneity. Nat Rev Cancer 14(2):108–119

    Article  CAS  PubMed  Google Scholar 

  8. Astuti D, Latif F, Dallol A, Dahia PL, Douglas F, George E et al (2001) Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma. Am J Hum Genet 69(1):54–49

    Article  Google Scholar 

  9. McInerney-Leo AM, Marshall MS, Gardiner B, Benn DE, McFarlane J, Robinson BG et al (2014) Whole exome sequencing is an efficient and sensitive method for detection of germline mutations in patients with phaeochromcytomas and paragangliomas. Clin Endocrinol 80(1):25–33

    Article  CAS  Google Scholar 

  10. Welander J, Andreasson A, Juhlin CC, Wiseman RW, Backdahl M, Hoog A et al (2014) Rare germline mutations identified by targeted next-generation sequencing of susceptibility genes in pheochromocytoma and paraganglioma. J Clin Endocrinol Metab 99(7):E1352–E1360

    Article  CAS  PubMed  Google Scholar 

  11. Knudson AG Jr (1986) Genetics of human cancer. Annu Rev Genet 20:231–251

    Article  PubMed  Google Scholar 

  12. Grimley PM, Glenner GG (1967) Histology and ultrastructure of carotid body paragangliomas. Comparison with the normal gland. Cancer 20(9):1473–1488

    Article  CAS  PubMed  Google Scholar 

  13. van den Berg R (2005) Imaging and management of head and neck paragangliomas. Eur Radiol 15(7):1310–1318

    Article  PubMed  Google Scholar 

  14. Zhang TH, Jiang WL, Li YL, Li B, Yamakawa T (2012) Perioperative approach in the surgical management of carotid body tumors. Ann Vasc Surg 26(6):775–782

    Article  PubMed  Google Scholar 

  15. Zeng G, Zhao J, Ma Y, Huang B (2012) Resection of carotid body tumors and the additional choice of intraoperative shunt in complicated tumors. Ann Vasc Surg 26(4):511–515

    Article  PubMed  Google Scholar 

  16. Ulrich S, Lehmann M, Ebmeyer J, Hamberger U, Reineke U, Dietrich U et al (2009) Direct percutaneous embolization of a carotid body tumor with Onyx. HNO 57(12):1305–1310

    Article  CAS  PubMed  Google Scholar 

  17. Timmers HJ, Chen CC, Carrasquillo JA, Whatley M, Ling A, Havekes B et al (2009) Comparison of 18F-fluoro-L-DOPA, 18F-fluoro-deoxyglucose, and 18F-fluorodopamine PET and 123I-MIBG scintigraphy in the localization of pheochromocytoma and paraganglioma. J Clin Endocrinol Metab 94(12):4757–4767

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  18. Ivan ME, Sughrue ME, Clark AJ, Kane AJ, Aranda D, Barani IJ et al (2011) A meta-analysis of tumor control rates and treatment-related morbidity for patients with glomus jugulare tumors. J Neurosurgery 114(5):1299–1305

    Google Scholar 

  19. Mazzoni A, Zanoletti E (2015) Observation and partial targeted surgery in the management of tympano-jugular paraganglioma: a contribution to the multioptional treatment. Eur Arch Oto Rhino Laryngol. doi:10.1007/s00405-015-3605-x (Epub ahead of print)

    Google Scholar 

  20. Chen PG, Nguyen JH, Payne SC, Sheehan JP, Hashisaki GT (2010) Treatment of glomus jugulare tumors with gamma knife radiosurgery. Laryngoscope 120(9):1856–1862

    Article  PubMed  Google Scholar 

  21. Dupin C, Lang P, Dessard-Diana B, Simon JM, Cuenca X, Mazeron JJ et al (2014) Treatment of head and neck paragangliomas with external beam radiation therapy. Int J Radiat Oncol Biol Phys 89(2):353–359

    Article  PubMed  Google Scholar 

  22. Kunzel J, Koch M, Brase C, Fietkau R, Iro H, Zenk J (2014) Treatment of cervical paragangliomas: is surgery the only way. Am J Otolaryngol 35(2):186–191

    Article  PubMed  Google Scholar 

  23. Lindner F (1953) Tumoren der Karotisdrüse. Langenbecks Arch Chir 276:156–161

    Article  Google Scholar 

  24. Shamblin WR, ReMine WH, Sheps SG, Harrison EG Jr (1971) Carotid body tumor (chemodectoma). Clinicopathologic analysis of ninety cases. Am J Surg 122(6):732–739

    Article  CAS  PubMed  Google Scholar 

  25. Netterville JL, Jackson CG, Miller FR, Wanamaker JR, Glasscock ME (1998) Vagal paraganglioma: a review of 46 patients treated during a 20-year period. Arch Otolaryngol 124(10):1133–1140

    Article  CAS  Google Scholar 

  26. Economopoulos KP, Tzani A, Reifsnyder T (2015) Adjunct endovascular interventions in carotid body tumors. J Vasc Surg 61(4):1081e2–1091e2

    Article  Google Scholar 

  27. Miller RB, Boon MS, Atkins JP, Lowry LD (2000) Vagal paraganglioma: the Jefferson experience. Otolaryngol Head Neck Surg 122(4):482–487

    CAS  PubMed  Google Scholar 

  28. Thabet MH, Kotob H (2001) Cervical paragangliomas: diagnosis, management and complications. J Laryngol Otol 115(6):467–474

    Article  CAS  PubMed  Google Scholar 

  29. Hinerman RW, Mendenhall WM, Amdur RJ, Stringer SP, Antonelli PJ, Cassisi NJ (2001) Definitive radiotherapy in the management of chemodectomas arising in the temporal bone, carotid body, and glomus vagale. Head Neck 23(5):363–371

    Article  CAS  PubMed  Google Scholar 

  30. Guss ZD, Batra S, Limb CJ, Li G, Sughrue ME, Redmond K et al (2011) Radiosurgery of glomus jugulare tumors: a meta-analysis. Int J Radiat Oncol Biol Phys 81(4):e497–e502

    Article  PubMed Central  PubMed  Google Scholar 

  31. Carrasco V, Rosenman J (1993) Radiation therapy of glomus jugulare tumors. Laryngoscope 103(11 Pt 2 Suppl 60):23–27

    CAS  PubMed  Google Scholar 

  32. Tanbouzi Husseini S, Piccirillo E, Taibah A, Paties CT, Rizzoli R, Sanna M (2011) Malignancy in vestibular schwannoma after stereotactic radiotherapy: a case report and review of the literature. Laryngoscope 121(5):923–928

    Article  PubMed  Google Scholar 

  33. Sanna M, Shin SH, De Donato G, Sivalingam S, Lauda L, Vitullo F et al (2011) Management of complex tympanojugular paragangliomas including endovascular intervention. Laryngoscope 121(7):1372–1382

    Article  PubMed  Google Scholar 

  34. Fisch U (1978) Infratemporal fossa approach to tumours of the temporal bone and base of the skull. J Laryngol Otol 92(11):949–967

    Article  CAS  PubMed  Google Scholar 

  35. Jackson CG, McGrew BM, Forest JA, Netterville JL, Hampf CF, Glasscock ME 3rd (2001) Lateral skull base surgery for glomus tumors: long-term control. Otol Neurotol 22(3):377–382

    Article  CAS  PubMed  Google Scholar 

  36. Tran Ba Huy P (2014) Radiotherapy for glomus jugulare paraganglioma. Eur Ann Otorhinolaryngol Head Neck Dis 131(4):223–226

    Article  CAS  PubMed  Google Scholar 

  37. Capatina C, Ntali G, Karavitaki N, Grossman AB (2013) The management of head-and-neck paragangliomas. Endocr Relat Cancer 20(5):R291–R305

    Article  PubMed  Google Scholar 

  38. Jordan JA, Roland PS, McManus C, Weiner RL, Giller CA (2000) Stereotastic radiosurgery for glomus jugulare tumors. Laryngoscope 110(1):35–38

    Article  CAS  PubMed  Google Scholar 

  39. Gjuric M, Wigand ME, Weidenbecher M, Wolf SR, Seidinger L (1997) Function preserving surgery of glomus jugulare tumors. An achievable goal. HNO 45(12):983–989

    Article  CAS  PubMed  Google Scholar 

  40. Neumann HP, Erlic Z, Boedeker CC, Rybicki LA, Robledo M, Hermsen M et al (2009) Clinical predictors for germline mutations in head and neck paraganglioma patients: cost reduction strategy in genetic diagnostic process as fall-out. Cancer Res 69(8):3650–3656

    Article  CAS  PubMed  Google Scholar 

  41. Alvarez-Morujo RJ, Ruiz MA, Serafini DP, Delgado IL, Friedlander E, Yurrita BS (2014) Management of multicentric paragangliomas: Review of 24 patients with 60 tumors. Head Neck. doi:10.1002/hed.23894 (Epub ahead of print)

    PubMed  Google Scholar 

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Authors and Affiliations

  1. Hals-Nasen-Ohren-Klinik, Universitätsmedizin Mainz, Langenbeckstr. 1, 55131, Mainz, Deutschland

    J. Künzel, K. Bahr & C. Matthias

  2. Institut für Pathologie, Universitätsmedizin Mainz, Mainz, Deutschland

    M. Hainz

  3. Institut für Klinische Chemie und Laboratoriumsmedizin, Universitätsmedizin Mainz, Mainz, Deutschland

    H. Rossmann

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  1. J. Künzel
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  4. H. Rossmann
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Correspondence to J. Künzel.

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Interessenkonflikt

J. Künzel, K. Bahr, M. Hainz, H. Rossmann und C. Matthias geben an, dass kein Interessenkonflikt besteht.

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Künzel, J., Bahr, K., Hainz, M. et al. Kopf- und Halsparagangliome. HNO 63, 821–830 (2015). https://doi.org/10.1007/s00106-015-0085-7

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