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Prognostisch günstige Subgruppen bei Krebs mit unklarem Primarius

Prognostically favourable subgroups in cancer of unknown primary

  • Leitthema
  • Published:
Der Onkologe Aims and scope

Zusammenfassung

Hintergrund

Etwa 10–30 % der CUP-Patienten (CUP: „cancer of unknown primary“) entfallen auf eine prognostisch günstige Subgruppe, für die Behandlungspfade definiert sind, die vom allgemeinen Standard einer unspezifischen zytostatischen Therapie abweichen.

Ziel der Arbeit

Es sollen ein praxisorientierter Überblick über Sonderkategorien beim CUP-Syndrom gegeben und praxisrelevantes Hintergrundwissen vermittelt werden.

Methoden

Der Wissensstand der Autoren, der auf langjähriger wissenschaftlicher und praktischer Tätigkeit auf diesem Fachgebiet beruht, wird im Beitrag wiedergegeben.

Ergebnisse

Prognostisch günstige Subgruppen beim CUP-Syndrom beinhalten zum einen lokalisierte Stadien mit kurativer Option, zum anderen verschiedene Sonderkategorien, bei denen eine Therapie gemäß Arbeitshypothese sinnvoll erscheint. Die Prognose dieser Subgruppen, die in dieser Übersicht einzeln diskutiert werden, ist sehr heterogen und reicht von deutlich über 50 % überlebenden Patienten nach 5 Jahren bis zu Kategorien, die zwar historisch zu den günstigen Subgruppen gezählt werden, bei denen aber das Überleben nicht eindeutig besser ist als das unselektierter CUP-Patienten. Bei vielen dieser Subgruppen besteht das Problem, dass die Evidenzlage sowohl hinsichtlich der Prognose als auch der anzuwendenden Diagnostik und Therapie schlecht ist. Als allgemeine Regel erfolgt die Therapie entweder gemäß den Standards, die für die jeweilige Arbeitshypothese gelten, oder, falls ein lokalisiertes Stadium vorliegt, mittels radikaler Resektion und ggf. Radio(chemo)therapie.

Schlussfolgerung

Zu den wichtigsten Aufgaben des Onkologen bei der Versorgung von CUP-Patienten gehört das Erkennen prognostisch günstiger Subgruppen, die von einer spezifischen Therapie profitieren.

Abstract

Background

About 10–30% of all patients with cancer of unknown primary (CUP) belong to a prognostically favourable subgroup for which treatment algorithms differing from the general standard of nonspecific cytostatic therapy have been defined.

Objectives

This review is intended to give a practice-oriented overview over prognostically favourable categories among CUP and provide background information relevant to the practitioner.

Methods

This is an outline of the authors’ knowledge, which is based on long-standing scientific and practical activity within the field of CUP.

Results

Prognostically favourable subgroups among CUP comprise both localised stages with a curative option and various special categories in which a therapy according to a working hypothesis appears sensible. The prognosis of these subsets, which are discussed in this review in detail, is very heterogeneous, spanning from a rate of surviving patients after 5 years clearly exceeding 50% to categories in which, although historically listed among favourable subgroups, survival rates are not unequivocally better than in unselected CUP patients. A problem is that for many of these subgroups, evidence regarding both the prognosis and the recommended diagnostic and therapeutic approaches is poor. As a general rule, therapy either follows the standards defined for the respective working hypothesis or, in case of a localised stage, radical resection and, if sensible, radio(chemo)therapy should be applied.

Conclusion

In the management of CUP patients, one of the most important tasks of the oncologist is the identification of prognostically favourable subgroups benefiting from a specific therapeutic approach.

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Literatur

  1. Ashikari R, Rosen PP, Urban JA, Denoo T (1976) Breast cancer presenting as an axillary mass. Ann Surg 183:415–417

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Bae JM, Choi YY, Kim DS, Lee JH, Jang HS, Lee JH et al (2015) Metastatic melanomas of unknown primary show better prognosis than those of known primary: a systematic review and meta-analysis of observational studies. J Am Acad Dermatol 72:59–70

    Article  PubMed  Google Scholar 

  3. Bochtler T, Krämer A (2017) Systemtherapie prognostisch ungünstiger CUP-Syndrome. Adeno- und undifferenzierte Karzinome. Onkologe 23(12). doi:10.1007/s00761-017-0206-x

  4. Dalrymple JC, Bannatyne P, Russell P, Solomon HJ, Tattersall MH, Atkinson K et al (1989) Extraovarian peritoneal serous papillary carcinoma: a clinicopathologic study of 31 cases. Cancer 64:110–115

    Article  CAS  PubMed  Google Scholar 

  5. Greco FA, Vaughn WK, Hainsworth JD (1986) Advanced poorly differentiated carcinoma of unknown primary site: recognition of a treatable syndrome. Ann Intern Med 104:547–553

    Article  CAS  PubMed  Google Scholar 

  6. Hainsworth JD, Greco FA (1993) Treatment of patients with cancer of an unknown primary site. N Engl J Med 329:257–263

    Article  CAS  PubMed  Google Scholar 

  7. Hainsworth JD, Wright EP, Johnson DH, Davis BW, Greco FA (1991) Poorly differentiated carcinoma of unknown primary site: clinical usefulness of immunoperoxidase staining. J Clin Oncol 9:1931–1938

    Article  CAS  PubMed  Google Scholar 

  8. Hainsworth JD, Johnson DH, Greco FA (1992) Cisplatin-based combination chemotherapy in the treatment of poorly differentiated carcinoma and poorly differentiated adenocarcinoma of unknown primary site: results of a 12-year experience. J Clin Oncol 10:912–922

    Article  CAS  PubMed  Google Scholar 

  9. Hainsworth JD, Rubin MS, Spigel DR, Boccia RV, Raby S, Quinn R et al (2013) Molecular gene expression profiling to predict the tissue of origin and direct site-specific therapy in patients with carcinoma of unknown primary site: a prospective trial of the Sarah Cannon research institute. J Clin Oncol 31:217–223

    Article  CAS  PubMed  Google Scholar 

  10. Halsted WS (1907) The results of radical operations for the cure of carcinoma of the breast. Ann Surg 46:1–19

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Joseph K, Sawyer MB, Amanie J, Jones Thachuthara J, Ghosh S, Tai P (2014) Carcinoma of unknown primary in the inguinal lymph node region of squamous cell origin: a case series. Pract Radiat Oncol 4:404–408

    Article  PubMed  Google Scholar 

  12. Kamposioras K, Pentheroudakis G, Pectasides D, Pavlidis N (2011) Malignant melanoma of unknown primary site. To make the long story short. A systematic review of the literature. Crit Rev Oncol Hematol 78:112–126

    Article  CAS  PubMed  Google Scholar 

  13. Krämer A, Löffler H (2016) Chapter 5: diagnostic work-up of CUP – an approach to current recommendations and underlying evidence. In: Krämer A, Löffler H (Hrsg) Cancer of unknown primary. Springer, Cham, S 67–74

    Chapter  Google Scholar 

  14. Löffler H, Krämer A (2016) Chapter 4: prognostic factors and their role in the management of CUP. In: Krämer A, Löffler H (Hrsg) Cancer of unknown primary. Springer, Cham, S 45–65

    Chapter  Google Scholar 

  15. Löffler H, Neben K, Krämer A (2014) CUP-Syndrom: Epidemiologie und Pathogenese. Radiologe 54:107–111

    Article  PubMed  Google Scholar 

  16. Löffler H, Puthenparambil J, Hielscher T, Neben K, Krämer A (2014) Patients with cancer of unknown primary: a retrospective analysis of 223 patients with adenocarcinoma or undifferentiated carcinoma. Dtsch Arztebl Int 111:481–487

    PubMed  PubMed Central  Google Scholar 

  17. Löffler H, Pfarr N, Kriegsmann M, Endris V, Hielscher T, Lohneis P et al (2016) Molecular driver alterations and their clinical relevance in cancer of unknown primary site. Oncotarget 7:44322–44329

    Article  PubMed  PubMed Central  Google Scholar 

  18. Patel J, Nemoto T, Rosner D, Dao TL, Pickren JW (1981) Axillary lymph node metastasis from an occult breast cancer. Cancer 47:2923–2927

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  20. Pentheroudakis G, Golfinopoulos V, Pavlidis N (2007) Switching benchmarks in cancer of unknown primary: from autopsy to microarray. Eur J Cancer 43:2026–2036

    Article  PubMed  Google Scholar 

  21. Pentheroudakis G, Lazaridis G, Pavlidis N (2010) Axillary nodal metastases from carcinoma of unknown primary (CUPAx): a systematic review of published evidence. Breast Cancer Res Treat 119:1–11

    Article  PubMed  Google Scholar 

  22. Pentheroudakis G, Pavlidis N (2010) Serous papillary peritoneal carcinoma: unknown primary tumour, ovarian cancer counterpart or a distinct entity? A systematic review. Crit Rev Oncol Hematol 75:27–42

    Article  PubMed  Google Scholar 

  23. Ransom DT, Patel SR, Keeney GL, Malkasian GD, Edmonson JH (1990) Papillary serous carcinoma of the peritoneum: a review of 33 cases treated with platin-based chemotherapy. Cancer 66:1091–1094

    Article  CAS  PubMed  Google Scholar 

  24. Ross JS, Wang K, Gay L, Otto GA, White E, Iwanik K et al (2015) Comprehensive genomic profiling of carcinoma of unknown primary site: new routes to targeted therapies. JAMA Oncol 1:40–49

    Article  PubMed  Google Scholar 

  25. Stoyianni A, Pentheroudakis G, Pavlidis N (2011) Neuroendocrine carcinoma of unknown primary: a systematic review of the literature and a comparative study with other neuroendocrine tumors. Cancer Treat Rev 37:358–365

    Article  PubMed  Google Scholar 

  26. Strnad CM, Grosh WW, Baxter J, Burnett LS, Jones HW 3rd, Greco FA et al (1989) Peritoneal carcinomatosis of unknown primary site in women. A distinctive subset of adenocarcinoma. Ann Intern Med 111:213–217

    Article  CAS  PubMed  Google Scholar 

  27. Tothill RW, Li J, Mileshkin L, Doig K, Siganakis T, Cowin P et al (2013) Massively-parallel sequencing assists the diagnosis and guided treatment of cancers of unknown primary. J Pathol 231:413–423

    Article  CAS  PubMed  Google Scholar 

  28. Varadhachary GR, Karanth S, Qiao W, Carlson HR, Raber MN, Hainsworth JD et al (2013) Carcinoma of unknown primary with gastrointestinal profile: immunohistochemistry and survival data for this favorable subset. Int J Clin Oncol 19:479–484

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Zentrum für Innere Medizin III, Marienhospital Stuttgart, Böheimstraße 37, 70199, Stuttgart, Deutschland

    Harald Löffler

  2. Klinische Kooperationseinheit Molekulare Hämatologie/Onkologie, Deutsches Krebsforschungszentrum (DKFZ) und Medizinische Klinik V, Universitätsklinikum Heidelberg, Heidelberg, Deutschland

    Alwin Krämer

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Correspondence to Harald Löffler.

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Interessenkonflikt

H. Löffler und A. Krämer geben an, dass kein Interessenkonflikt besteht.

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

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Löffler, H., Krämer, A. Prognostisch günstige Subgruppen bei Krebs mit unklarem Primarius. Onkologe 23, 993–999 (2017). https://doi.org/10.1007/s00761-017-0241-7

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