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Zervikales CUP-Syndrom

Cervical CUP syndrome

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Zusammenfassung

Wenn bei Auftreten von Lymphknotenmetastasen die Primärtumorlokalisation durch die initialen diagnostischen Schritte verborgen bleibt, spricht man von „cancer of unknown primary“ (CUP-Syndrom). Diese Manifestationsform betrifft 4–5% aller humanen Malignome und liegt damit unter den 10 häufigsten Krebsarten. Wenn der Primärtumor durch erweiterte Diagnostik schließlich detektiert wird, liegt ein initiales CUP-Syndrom vor, sonst spricht man von einem echten CUP-Syndrom. Die häufigsten CUP-Metastasen sind Adenokarzinome, gefolgt von schlecht differenzierten Karzinomen, Plattenepithelkarzinomen und neuroendokrinen Tumoren. Halslymphknotenmetastasen unbekannten Ursprungs haben generell eine schlechtere Prognose als die meisten Kopf-Hals-Tumoren, was aber von einer Reihe von Einflussfaktoren abhängt. Dieser Beitrag stellt moderne diagnostische Schritte wie PET-CT/MRT vor und erörtert die therapeutischen Optionen und die Prognose der Patienten mit einem zervikalen CUP-Syndrom.

Abstract

Whenever the first diagnosis of a solid malignancy is found in lymphoid tissue and detection of the primary tumor during the initial diagnostic procedures is not successful, this syndrome is called CUP (cancer of unknown primary). When extensive diagnostic steps finally lead to detection of the primary, then an initial cup syndrome can be assumed in contrast to true CUP syndromes, where the primary tumor remains unrevealed. Most CUP metastases are adenocarcinomas, followed by poorly differentiated carcinomas, squamous cell carcinomas, and neuroendocrine tumors. Metastasis of unknown primaries occurring in lymph nodes of the neck region have a poor prognosis and is predominantly associated with worse prognosis than most other head and neck tumors, which however does depend on various factors. In this article, we introduce diagnostic steps with modern techniques like PET-CT/MRI, and review therapeutic options and prognosis in patients with cervical CUP syndrome.

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Literatur

  1. Abbruzzese JL, Raber MN, Frost P (1988) An effective strategy for the evaluation of unknown primary tumors. Cancer Bull 41:157–161

    Google Scholar 

  2. Balaker AE, Abemayor E, Elashoff D, St John MA (2012) Cancer of unknown primary: does treatment modality make a difference? Laryngoscope 122(6):1279–1282

    Article  PubMed  Google Scholar 

  3. Bernier J, Cooper JS, Pajak TF et al (2005) Defining risk levels in locally advanced head and neck cancers: a comparative analysis of concurrent postoperative radiation plus chemotherapy trials of the EORTC (#22931) and RTOG (#9501). Head Neck 27(10):843–850

    Article  PubMed  Google Scholar 

  4. Bernier J, Domenge C, Ozsahin M et al (2004) Postoperative irradiation with or without concomitant chemotherapy for locally advanced head and neck cancer. N Engl J Med 350(19):1945–1952

    Article  PubMed  CAS  Google Scholar 

  5. Chen AM, Farwell DG, Lau DH et al (2011) Radiation therapy in the management of head-and-neck cancer of unknown primary orogin: How does the addition or concurrent chemotherapy affect the therapeutic ratio? Int J Radiat Oncol Biol Phys 81(2):346–352

    Article  PubMed  Google Scholar 

  6. Cooper JS, Pajak TF, Forastiere AA et al (2004) Postoperative concurrent radiotherapy and chemotherapy for high risk squamous-cell carcinoma of the head and neck. N Engl J Med 350(19):1937–1944

    Article  PubMed  Google Scholar 

  7. Fakhrian K, Thamm R, Knapp S et al (2012) Radio(chemo)therapy in the management of squamous cell carcinoma of cervical lymph nodes from an unknown primary site. A retrospective analysis. Strahlenther Onkol 188:56–61

    Article  PubMed  CAS  Google Scholar 

  8. Fietkau R, Lautenschläger C, Sauer R et al (2006) Postoperative concurrent radiochemotherapy versus radiotherapy in high risk SCCA of head and neck: results of the German phase III trial ARO 96-3. J Clin Oncol, ASCO Annual Meeting Proceedings Part I. Vol 24, No. 18 S (June 20 Supplement), 5507

  9. Fogarty GB, Peters LJ, Stewart J et al (2003) The usefulness of fluorine 18-labelled deoxyglucose positron emission tomography in the investigation of patients with cervikal lymphadenopathy from an unknown primary tumor. Head Neck 25(2):138–145

    Article  PubMed  Google Scholar 

  10. Fu KK (1994) Neck node metastasis from unknown primary. Controversies in management. Front Radiat Ther Oncol 28:66–78

    PubMed  CAS  Google Scholar 

  11. Grau C, Johansen LV, Jakobsen J et al (2000) Cervical lymph node metastases from unknown primary tumours. Results from a national survey by the Danish Society for Head and Neck Oncology. Radiother Oncol 55(2):121–129

    Article  PubMed  CAS  Google Scholar 

  12. Greco FA, Briasoulis E (2005) Cancer of unknown primary site. In: DeVita VT Jr, Hellmann S, Rosenberg SA (Hrsg) Cancer-principles and practice of ocology, 7. Aufl. Bd 46. Lippincott Williams and Wilkins, Philadephia, S 2213–2236

  13. Greco FA, Erlander MG (2009) Molecular classification of cancers of unknown primary site. Mol Diagn Ther 13(6):367–373

    Article  PubMed  Google Scholar 

  14. Greco FA, Gray J, Burris HA (2001) Taxane-based chemotherapy for patients with carcinoma of unknown primary site. Cancer J 7:203–212

    PubMed  CAS  Google Scholar 

  15. Greco FA, Hainsworth JD (1999) The evolving role of pacitaxel for patients with carcinoma of unknown primary site. Semin Oncol 26(12):129–133

    PubMed  CAS  Google Scholar 

  16. Gutzeit A, Antoch G, Kühl H et al (2005) Unknown primary tumors; detection with dual modality PET/CT: Initial experience. Radiology 234:227–234

    Article  PubMed  Google Scholar 

  17. Haas I, Hoffmann TK, Engers R, Ganzer U (2002) Diagnostic strategies in zervikal carcinoma of an unknown primary (CUP). Eur Arch Otorhinolaryngol 259(6):325–333

    Article  PubMed  Google Scholar 

  18. Hainsworth JD, Greco FA (2000) Management of patients with cancer of unknown primary site. Oncology 14(4):563–574

    PubMed  CAS  Google Scholar 

  19. Hemminki K, Ji J, Sundquist J, Shu X (2011) Familial risks in cancer of unknown primary: tracking the primary site. J Clin Oncol 29(4):435–440

    Article  PubMed  Google Scholar 

  20. Johansen J, Buus S, Loft A et al (2008) Prospective study of 18FDG-PET in the detection and management of patients with lymph node metastases to the neck from an unknown primary tumor. Results from the DAHANCA-13 study. Head Neck 30(4):471–478

    Article  PubMed  Google Scholar 

  21. Jones AS, Phillips DE, Helliwell TR, Roland NJ (1993) Occult node metastases in head and neck squamous cell carcinoma. Eur Arch Otorhinolaryngol 250(8):446–449

    Article  PubMed  CAS  Google Scholar 

  22. Keller F, Psychogios G, Linke R et al (2011) Carcinoma of unknown primary in the head and neck: comparison between positron emission tomography (PET) and PET/CT. Head Neck 33(11):1569–1575

    Article  PubMed  Google Scholar 

  23. Kirschner MJ, Fietkau R, Waldfahrere F et al (1997) Zur Therapie von zervikalen Metastasen ohne bekannten Primärtumor. Strahlenther Onkol 173:362–368

    Article  PubMed  CAS  Google Scholar 

  24. Lapeyre M, Malissard L, Peiffert D et al (1997) Cervical lymph node metastasis from an unknown primary: is a tonsillectomy necessary? Int J Radiat Oncol Biol Phys 39(2):291–296

    Article  PubMed  CAS  Google Scholar 

  25. Ligey A, Gentil J, Crehange G et al (2009) Impact of target volumes and radiation technique on loco-regional control and survival for patients with unilateral cervical lymph node metastases from an onknown primary. Radiother Oncol 93:483–487

    Article  PubMed  CAS  Google Scholar 

  26. Lindberg R (1972) Distribution of cervical lymph node metastases from squamous cell carcinoma of the upper respiratory and digestive tracts. Cancer 29(6):1446–1449

    Article  PubMed  CAS  Google Scholar 

  27. Macdonald MR, Freemen JL, Hui MF et al (1995) Role of Epstein-Barr virus in fine-needle aspiration of metastatic neck nodes in the diagnosis of nasopharyngeal carcinoma. Head Neck 17:487–493

    Article  PubMed  CAS  Google Scholar 

  28. Madani I, Vakaet L, Bonte K et al (2008) Intensity-modulated radiotherapy for cervical lymph node metastases from unknown primary cancer. Int J Radiat Oncol Biol Phys 71(4):1158–1166

    Article  PubMed  Google Scholar 

  29. Marcial-Vega VA, Cardenes H, Perez CA (1990) Cervical metastases from unknown primary: radiotherapeutic management and appearance of subsequnet primaries. Int J Radiat Oncol Biol Phys 19:919–928

    Article  PubMed  CAS  Google Scholar 

  30. Mendenhall WM, Mancuso AA, Amdur RJ et al (2001) Squamous cell carcinoma metastatic to the neck from an unknown head and neck primary site. Am J Otolaryngol 22(4):261–267

    Article  PubMed  CAS  Google Scholar 

  31. Mendenhall WM, Mancuso AA, Parson JT et al (1998) Diagnostic evaluation of squamous cell carcinoma metastatic to zervikal lymph nodes from an unknown head and neck primary site. Head Neck 20(8):739–744

    Article  PubMed  CAS  Google Scholar 

  32. Nieder C, Gregoire V, Ang KK (2001) Cervical lymph node metastases from occult squamous cell carcinoma: cut down a tree to get an apple? Int J Radiat Oncol Biol Phys 50(3):727–733

    Article  PubMed  CAS  Google Scholar 

  33. Paul SA, Stoeckli SJ, Schulthess GK von, Goerres GW (2007) FDG PET and PET/CT for the detection of the primary tumour in patients with zervikal non-squamous cell carcinoma metastasis of an unknown primary. Eur Arch Otorhinolaryngol 264:189–195

    Article  PubMed  Google Scholar 

  34. Pavlidis N, Briasoulis E, Briasoulis E, Greco FA (2003) Diagnostic and therapeutic management of cancer of unknown primary. Eur J Cancer 39:1990–2005

    Article  PubMed  CAS  Google Scholar 

  35. Rudmik L, Lau HY, Matthews TW et al (2011) Clinical utility of PET/CT in the evaluation of head and neck squamous cell carcinoma with an unknown primary: a prospective clinical trial. Head Neck 33(7):935–940

    Article  PubMed  Google Scholar 

  36. Smeets SJ, Hesselink AT, Speel EJ et al (2007) A novel algorithm for reliable detection of human papillomavirus in paraffin embedded head and neck cancer specimen. Int J Cancer 121(11):2465–2472

    Article  PubMed  CAS  Google Scholar 

  37. Sroeckli SJ, Mosna-Firlejczyk K, Goerres GW (2003) Lymph node metastasis of squamous cell carcinoma from an unknown primary: impact of positron emission tomography. Eur J Nucl Med 30:411–416

    Article  Google Scholar 

  38. Ulbright TM (2005) Germ cell tumors of the gonads: a selective review emphasizing problems in differential diagnosis, newly appreciated, and controversial issues. Mod Pathol 18(2):61–79

    Article  Google Scholar 

  39. Weber A, Schmoz S, Bootz F (2001) CUP (carcinoma of unknown primary) syndrome in head and neck: clinic, diagnostic, and therapy. Onkologie 24(1):38–43

    Article  PubMed  CAS  Google Scholar 

  40. Werner J (2002) Lymphknotenerkrankungen im Kopf-Hals-Bereich. Springer, Berlin

  41. Wittekind C, Meyer H-J, Bootz F (Hrsg) IUCC.TNM Klassifikation maligner Tumoren, 6. Aufl. Springer, Berlin

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Authors and Affiliations

  1. Klinik und Poliklinik für Hals-Nasen- und Ohrenheilkunde, Universität Leipzig, Liebigstraße 10–14, 04103, Leipzig, Deutschland

    C. Mozet, G. Wichmann & A. Dietz

  2. Klinik und Poliklinik für Diagnostische und Interventionelle Radiologie, Universität Leipzig, Leipzig, Deutschland

    P. Stumpp

  3. Klinik für Strahlentherapie, Universität Leipzig, Leipzig, Deutschland

    S. Dietzsch

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  1. C. Mozet
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  2. G. Wichmann
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  4. S. Dietzsch
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  5. A. Dietz
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Correspondence to C. Mozet.

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Mozet, C., Wichmann, G., Stumpp, P. et al. Zervikales CUP-Syndrom. Onkologe 19, 44–51 (2013). https://doi.org/10.1007/s00761-012-2315-x

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  • DOI: https://doi.org/10.1007/s00761-012-2315-x

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