Advertisement

Der Chirurg

, Volume 90, Issue 1, pp 29–36 | Cite as

Chirurgische Therapieoptionen bei zervikalen Paragangliomen

  • R. SchneiderEmail author
  • M. Elwerr
  • K. Lorenz
  • S. Plontke
  • H. Dralle
  • J. Ukkat
Leitthema
  • 208 Downloads

Zusammenfassung

Die Therapie der seltenen Tumorentität zervikales Paragangliom (PG) durchläuft aktuell einen Paradigmenwechsel. Während bei kleinen Glomus-caroticum-Tumoren, malignen sowie endokrin aktiven Tumoren die chirurgische Resektion die Therapie der Wahl darstellt, sollte eine Operation bei lokal fortgeschrittenen Glomus-caroticum-Tumoren und vagalen PG kritisch diskutiert werden. Aufgrund der unmittelbaren Nähe dieser hypervaskularisierten Tumoren zu den kaudalen Hirnnerven besteht nach einer Resektion insbesondere bei lokal fortgeschrittenen zervikalen PG das Risiko gravierender nervaler Schäden mit einer erheblichen Beeinträchtigung der Lebensqualität, sodass ein restriktives operatives Vorgehen angebracht ist. Die externe Strahlentherapie kann eine gleichwertige primäre Therapieoption hinsichtlich Rezidivfreiheit bei geringerer Morbidität darstellen. Das langsame Tumorwachstum und die Tendenz der hereditären zervikalen PG zur Multifokalität bzw. signifikante Komorbiditäten älterer, asymptomatischer Patienten rechtfertigen ein zunehmend weniger aggressives Vorgehen in der Therapiestrategie dieser Tumoren. Mit dem Wait-and-scan-Management ist eine engmaschige, bildgebende und klinische Reevaluierung verbunden. Die Größe und Lokalisation des Tumors, dessen Wachstumsgeschwindigkeit, Genetik, Patientenalter und Allgemeinzustand sowie therapiebeeinflussende Komorbiditäten, das Vorliegen synchroner PG und/oder vasoaktiver katecholaminproduzierender Tumoren müssen vor dem Therapiebeginn identifiziert und in der multidisziplinären Therapieplanung zwingend berücksichtigt werden. Obwohl Best-practice-Algorithmen zur Therapie der zervikalen PG zur Verfügung stehen, haben die innovativen Entwicklungen der letzten Jahre zu patientenadaptierten, individuellen Therapieansätzen geführt.

Schlüsselwörter

Epidemiologie Familiäre Syndrome Glomustumoren Shamblin-Klassifikation SDH-Mutation 

Surgical treatment options for cervical paragangliomas

Abstract

The therapies available for the rare tumor entity of cervical paraganglioma (PG) are currently undergoing a paradigm shift. The treatment of choice for small carotid body tumors, malignant and active endocrine tumors is surgical resection; however, for locally advanced carotid body tumors and vagal PG, surgical therapy should be critically evaluated. Due to the immediate proximity of these hypervascularized tumors to the caudal cranial nerves, there is a risk of severe nerve damage with a significant impairment of quality of life after resection, particularly for locally advanced cervical PG, emphasizing further the importance of a restrictive surgical strategy. External radiotherapy can provide an equivalent primary therapeutic option with respect to the rate of recurrence and is accompanied by a lower morbidity. The slow rate of tumor progression and the multifocality of the familial variant of cervical PG or significant comorbidities in older, asymptomatic patients warrant a less aggressive treatment strategy for these tumors. When a wait and scan approach is implemented, a closely monitored radiological and clinical re-evaluation is of upmost importance. In a multidisciplinary approach the following critical points require consideration before a therapy is implemented,: size and location of the tumor, progression rate, genetic background, patient age and general condition, relevant comorbidities, the presence of synchronous PG and/or vasoactive catecholamine-producing tumors. Although best practice algorithms for the treatment of cervical PG have already been devised, recent innovative developments have led to more patient-tailored, individualized treatment approaches.

Keywords

Epidemiology Familial syndrome Glomus tumors Shamblin classification SDH mutation 

Notes

Einhaltung ethischer Richtlinien

Interessenkonflikt

R. Schneider, M. Elwerr, K. Lorenz, S. Plontke, H. Dralle und J. Ukkat geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

Literatur

  1. 1.
    Abu-Ghanem S, Yehuda M, Carmel NN, Abergel A, Fliss DM (2016) Impact of preoperative embolization on the outcomes of carotid body tumor surgery: a meta-analysis and review of the literature. Head Neck 38(Suppl 1):E2386–E2394CrossRefGoogle Scholar
  2. 2.
    Amin MF, El Ameen NF (2013) Diagnostic efficiency of multidetector computed tomography versus magnetic resonance imaging in differentiation of head and neck paragangliomas from other mimicking vascular lesions: comparison with histopathologic examination. Eur Arch Otorhinolaryngol 270:1045–1053CrossRefGoogle Scholar
  3. 3.
    Bausch B, Schiavi F, Ni Y et al (2017) Clinical characterization of the pheochromocytoma and paraganglioma susceptibility genes SDHA, TMEM127, MAX, and SDHAF2 for gene-informed prevention. JAMA Oncol 3:1204–1212CrossRefGoogle Scholar
  4. 4.
    Baysal BE (2002) Hereditary paraganglioma targets diverse paraganglia. J Med Genet 39:617–622CrossRefGoogle Scholar
  5. 5.
    Blanchet EM, Gabriel S, Martucci V et al (2014) 18F-FDG PET/CT as a predictor of hereditary head and neck paragangliomas. Eur J Clin Invest 44:325–332CrossRefGoogle Scholar
  6. 6.
    Boedeker CC, Hensen EF, Neumann HP et al (2014) Genetics of hereditary head and neck paragangliomas. Head Neck 36:907–916CrossRefGoogle Scholar
  7. 7.
    Bradshaw JW, Jansen JC (2005) Management of vagal paraganglioma: is operative resection really the best option? Surgery 137:225–228CrossRefGoogle Scholar
  8. 8.
    Burnichon N, Rohmer V, Amar L et al (2009) The succinate dehydrogenase genetic testing in a large prospective series of patients with paragangliomas. J Clin Endocrinol Metab 94:2817–2827CrossRefGoogle Scholar
  9. 9.
    Cobb AN, Barkat A, Daungjaiboon W et al (2018) Carotid body tumor resection: just as safe without preoperative embolization. Ann Vasc Surg 46:54–59CrossRefGoogle Scholar
  10. 10.
    Corssmit EP, Romijn JA (2014) Clinical management of paragangliomas. Eur J Endocrinol 171:R231–R243CrossRefGoogle Scholar
  11. 11.
    Ellis RJ, Patel D, Prodanov T, Nilubol N, Pacak K, Kebebew E (2014) The presence of SDHB mutations should modify surgical indications for carotid body paragangliomas. Ann Surg 260:158–162CrossRefGoogle Scholar
  12. 12.
    Favier J, Amar L, Gimenez-Roqueplo AP (2015) Paraganglioma and phaeochromocytoma: from genetics to personalized medicine. Nat Rev Endocrinol 11:101–111CrossRefGoogle Scholar
  13. 13.
    Fisch U, Mattox D (1988) Microsurgery of the skull base. Thieme, Stuttgart, New York, S 149–153Google Scholar
  14. 14.
    Gilbo P, Morris CG, Amdur RJ et al (2014) Radiotherapy for benign head and neck paragangliomas: a 45-year experience. Cancer 120:3738–3743CrossRefGoogle Scholar
  15. 15.
    Hermsen MA, Sevilla MA, Llorente JL et al (2010) Relevance of germline mutation screening in both familial and sporadic head and neck paraganglioma for early diagnosis and clinical management. Cell Oncol 32:275–283PubMedPubMedCentralGoogle Scholar
  16. 16.
    Hinerman RW, Amdur RJ, Morris CG, Kirwan J, Mendenhall WM (2008) Definitive radiotherapy in the management of paragangliomas arising in the head and neck: a 35-year experience. Head Neck 30:1431–1438CrossRefGoogle Scholar
  17. 17.
    Hu K, Persky MS (2016) Treatment of head and neck paragangliomas. Cancer Control 23:228–241CrossRefGoogle Scholar
  18. 18.
    Işik AC, Imamoğlu M, Erem C, Sari A (2007) Paragangliomas of the head and neck. Med Princ Pract 16:209–214CrossRefGoogle Scholar
  19. 19.
    Jansen JC, van den Berg R, Kuiper A et al (2000) Estimation of growth rate in patients with head and neck paragangliomas influences the treatment proposal. Cancer 88:2811–2816CrossRefGoogle Scholar
  20. 20.
    Kim GY, Lawrence PF, Moridzadeh RS et al (2017) New predictors of complications in carotid body tumor resection. J Vasc Surg 65:1673–1679CrossRefGoogle Scholar
  21. 21.
    Kollert M, Minovi AA, Draf W, Bockmühl U (2006) Cervical paragangliomas-tumor control and long-term functional results after surgery. Skull Base 16:185–191CrossRefGoogle Scholar
  22. 22.
    Konukiewitz B, Agaimy A, Weichert W, Klöppel G (2018) Neuroendocrine neoplasms of the head and neck. Pathologe 39:27–34CrossRefGoogle Scholar
  23. 23.
    Künzel J, Bahr K, Hainz M, Rossmann H, Matthias C (2015) Head and neck paragangliomas: an interdisciplinary challenge. HNO 63:821–824CrossRefGoogle Scholar
  24. 24.
    Langerman A, Athavale SM, Rangarajan SV, Sinard RJ, Netterville JL (2012) Natural history of cervical paragangliomas: outcomes of observation of 43 patients. Arch Otolaryngol Head Neck Surg 138:341–345CrossRefGoogle Scholar
  25. 25.
    Lee JH, Barich F, Karnell LH et al (2002) National Cancer Data Base report on malignant paragangliomas of the head and neck. Cancer 94:730–737CrossRefGoogle Scholar
  26. 26.
    Linder F (1953) Tumors of the carotid gland. Langenbecks Arch Klin Chir Ver Dtsch Z Chir 276:156–161PubMedGoogle Scholar
  27. 27.
    Luchetti A, Walsh D, Rodger F et al (2015) Profiling of somatic mutations in phaeochromocytoma and paraganglioma by targeted next generation sequencing analysis. Int J Endocrinol.  https://doi.org/10.1155/2015/138573 CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Luna-Ortiz K, Rascon-Ortiz M, Villavicencio-Valencia V, Herrera-Gomez A (2006) Does Shamblin’s classification predict postoperative morbidity in carotid body tumors? A proposal to modify Shamblin’s classification. Eur Arch Otorhinolaryngol 263:171–175CrossRefGoogle Scholar
  29. 29.
    Makeieff M, Raingeard I, Alric P et al (2008) Surgical management of carotid body tumors. Ann Surg Oncol 15:2180–2186CrossRefGoogle Scholar
  30. 30.
    Mendenhall WM, Amdur RJ, Vaysberg M, Mendenhall CM, Werning JW (2011) Head and neck paragangliomas. Head Neck 33:1530–1534CrossRefGoogle Scholar
  31. 31.
    Metheetrairut C, Chotikavanich C, Keskool P, Suphaphongs N (2016) Carotid body tumor: a 25-year experience. Eur Arch Otorhinolaryngol 273:2171–2179CrossRefGoogle Scholar
  32. 32.
    Moore MG, Netterville JL, Mendenhall WM, Isaacson B, Nussenbaum B (2016) Head and neck paragangliomas: an update on evaluation and management. Otolaryngol Head Neck Surg 154:597–605CrossRefGoogle Scholar
  33. 33.
    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 Head Neck Surg 124:1133–1140CrossRefGoogle Scholar
  34. 34.
    Neumann HP, Erlic Z, Boedeker CC 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 69:3650–3656CrossRefGoogle Scholar
  35. 35.
    NGS in PPGL Study Group, Toledo RA, Burnichon N, Cascon A et al (2017) Consensus Statement on next-generation-sequencing-based diagnostic testing of hereditary phaeochromocytomas and paragangliomas. Nat Rev Endocrinol 13:233–247CrossRefGoogle Scholar
  36. 36.
    Obholzer RJ, Hornigold R, Connor S, Gleeson MJ (2011) Classification and management of cervical paragangliomas. Ann R Coll Surg Engl 93:596–602CrossRefGoogle Scholar
  37. 37.
    Offergeld C, Brase C, Yaremchuk S et al (2012) Head and neck paragangliomas: clinical and molecular genetic classification. Clinics (Sao Paulo) 67(Suppl 1):19–28CrossRefGoogle Scholar
  38. 38.
    Papaspyrou K, Mewes T, Rossmann H et al (2012) Head and neck paragangliomas: report of 175 patients (1989–2010). Head Neck 34:632–637CrossRefGoogle Scholar
  39. 39.
    Pellitteri PK, Rinaldo A, Myssiorek D et al (2004) Paragangliomas of the head and neck. Oral Oncol 40:563–575CrossRefGoogle Scholar
  40. 40.
    Piccini V, Rapizzi E, Bacca A et al (2012) Head and neck paragangliomas: genetic spectrum and clinical variability in 79 consecutive patients. Endocr Relat Cancer 19:149–155CrossRefGoogle Scholar
  41. 41.
    Plouin PF, Amar L, Dekkers OM et al (2016) European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma. Eur J Endocrinol 174:G1–G10CrossRefGoogle Scholar
  42. 42.
    Schneider R, Sekulla C, Machens A, Lorenz K, Nguyen Thanh P, Dralle H (2015) Postoperative vocal fold palsy in patients undergoing thyroid surgery with continuous or intermittent nerve monitoring. Br J Surg 102:1380–1387CrossRefGoogle Scholar
  43. 43.
    Schneider R, Ukkat J, Nguyen-Thanh P et al (2012) Endocrine surgery for neck paraganglioma: operation, radiation therapy or wait and scan? Chirurg 83:1060–1067CrossRefGoogle Scholar
  44. 44.
    Shamblin WR, ReMine WH, Sheps SG, Harrison EG (1971) Carotid body tumor (chemodectoma). Clinicopathologic analysis of ninety cases. Am J Surg 122:732–739CrossRefGoogle Scholar
  45. 45.
    Smith JD, Harvey RN, Darr OA et al (2017) Head and neck paragangliomas: A two-decade institutional experience and algorithm for management. Laryngoscope Investig Otolaryngol 2:380–389CrossRefGoogle Scholar
  46. 46.
    Suárez C, Fernández-Alvarez V, Neumann HP et al (2015) Modern trends in the management of head and neck paragangliomas. Eur Arch Otorhinolaryngol 272:3595–3599CrossRefGoogle Scholar
  47. 47.
    Suárez C, Rodrigo JP, Bödeker CC et al (2013) Jugular and vagal paragangliomas: systematic study of management with surgery and radiotherapy. Head Neck 35:1195–1204CrossRefGoogle Scholar
  48. 48.
    Suárez C, Rodrigo JP, Mendenhall WM et al (2014) Carotid body paragangliomas: a systematic study on management with surgery and radiotherapy. Eur Arch Otorhinolaryngol 271:23–34CrossRefGoogle Scholar
  49. 49.
    Taïeb D, Kaliski A, Boedeker CC et al (2014) Current approaches and recent developments in the management of head and neck paragangliomas. Endocr Rev 35:795–819CrossRefGoogle Scholar
  50. 50.
    Welander J, Andreasson A, Juhlin CC et al (2014) Rare germline mutations identified by targeted next-generation sequencing of susceptibility genes in pheochromocytoma and paraganglioma. J Clin Endocrinol Metab 99:E1352–E1360CrossRefGoogle Scholar
  51. 51.
    Welander J, Söderkvist P, Gimm O (2011) Genetics and clinical characteristics of hereditary pheochromocytomas and paragangliomas. Endocr Relat Cancer 18:R253–R276CrossRefGoogle Scholar

Copyright information

© Springer Medizin Verlag GmbH, ein Teil von Springer Nature 2018

Authors and Affiliations

  • R. Schneider
    • 1
    Email author
  • M. Elwerr
    • 1
  • K. Lorenz
    • 1
  • S. Plontke
    • 2
  • H. Dralle
    • 3
  • J. Ukkat
    • 1
  1. 1.Universitätsklinik und Poliklinik für Viszerale, Gefäß und Endokrine ChirurgieMartin-Luther-Universität Halle-WittenbergHalle/SaaleDeutschland
  2. 2.Universitätsklinik und Poliklinik für Hals‑, Nasen‑, Ohrenheilkunde, Kopf- und HalschirurgieMartin-Luther-UniversitätHalle/SaaleDeutschland
  3. 3.Sektion Endokrine Chirurgie, Klinik für Allgemeinchirurgie, Viszeral- und TransplantationschirurgieUniversitätsklinikum EssenEssenDeutschland

Personalised recommendations