Skip to main content
SpringerLink
Log in

Diagnostische Fallstricke beim Cushing-Syndrom

Diagnostic pitfalls with Cushing’s syndrome

  • Kasuistiken
  • Published:
Der Internist Aims and scope Submit manuscript

Zusammenfassung

Das Nebennierenrindenkarzinom ist eine seltene Erkrankung und kann eine Hormonaktivität aufweisen. Die histologische Abgrenzung zwischen Nebennierenadenom und Karzinom kann im Einzelfall außerordentlich schwierig sein. Für die Prognose eines Nebenierenkarzinoms sind eine rasche und korrekte Diagnosesicherung einschließlich eines Tumorstagings und der Versuch der kompletten chirurgischen Exstirpation ausschlaggebend. Bei metastasiertem Nebennierenrindenkarzinom erfolgt eine mitotanbasierte Chemotherapie, wobei hier auf Grund der spärlichen Datenlage in Deutschland ein Einschluss in die „Firm-act-Studie“ angestrebt werden sollte (http://www.firm-act.org). Der vorliegende Fallbericht erläutert diagnostische Fallstricke bei einer Patientin mit Cushing-Syndrom, dem ein metastasiertes Nebennierenkarzinom zugrunde lag.

Abstract

Adrenal cortical carcinoma is a rare diagnosis and may present with hormone secretion. A histological differentiation between an adrenal cortical adenoma and carcinoma can be very difficult. However, a fast diagnosis including staging and complete surgical resection is pivotal for the prognosis of an adrenal cortical carcinoma. Metastasing adrenal cortical carcinoma should be treated with a mitotane based chemotherapy, and inclusion in the “firm-act study” is highly recommended. The present case report demonstrates the diagnostic pitfalls in a female patients with Cushing’s syndrome who suffered from metastasing adrenal cortical carcinoma.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3
Abb. 4
Abb. 5

Literatur

  1. Aiba M, Fujibayashi M (2005) Histopathological diagnosis and prognostic factors in adrenocortical carcinoma. Endocr Pathol 16: 13–22

    Article  PubMed  Google Scholar 

  2. Allolio B, Fassnacht M (2006) Clinical review: Adrenocortical carcinoma: clinical update. J Clin Endocrinol Metab 91: 2027–2037

    Article  CAS  PubMed  Google Scholar 

  3. Assie G, Antoni G, Tissier F et al. (2007) Prognostic parameters of metastatic adrenocortical carcinoma. J Clin Endocrinol Metab 92: 148–154

    Article  CAS  PubMed  Google Scholar 

  4. Aubert S, Wacrenier A, Leroy X et al. (2002) Weiss system revisited: a clinicopathologic and immunohistochemical study of 49 adrenocortical tumors. Am J Surg Pathol 26: 1612–1619

    Article  PubMed  Google Scholar 

  5. Babinska A, Sworczak K, Wisniewski P et al. (2008) The role of immunohistochemistry in histopathological diagnostics of clinically „silent“ incidentally detected adrenal masses. Exp Clin Endocrinol Diabetes 116: 246–251

    Article  CAS  PubMed  Google Scholar 

  6. Bhargav PR, Mishra A, Agarwal G et al. (2008) Adrenal incidentalomas: experience in a developing country. World J Surg 32: 1802–1808

    Article  CAS  PubMed  Google Scholar 

  7. Crucitti F, Bellantone R, Ferrante A et al. (1996) The Italian Registry for Adrenal Cortical Carcinoma: analysis of a multiinstitutional series of 129 patients. The ACC Italian Registry Study Group. Surgery 119: 161–170

    Article  CAS  PubMed  Google Scholar 

  8. de Gennes JL, Kiortsis DN, Dairou F et al. (1995) Metastatic pulmonary carcinoma, revealed by Cushing syndrome, initially considered to have a pituitary origin. Course over 25 years. Presse Med 24: 1605–1607

    Google Scholar 

  9. Ehrhart-Bornstein M, Hilbers U (1998) Neuroendocrine properties of adrenocortical cells. Horm Metab Res 30: 436–439

    Article  CAS  PubMed  Google Scholar 

  10. Fassnacht M, Hahner S, Banfelder N et al. (2005) Diagnostik und Therapie des Nebennierenrinden-Karzinoms. Dtsch Ärztebl 102: A1670–A1675

    Google Scholar 

  11. Haak HR, Fleuren GJ (1995) Neuroendocrine differentiation of adrenocortical tumors. Cancer 75: 860–864

    Article  CAS  PubMed  Google Scholar 

  12. Icard P, Goudet P, Charpenay C et al. (2001) Adrenocortical carcinomas: surgical trends and results of a 253-patient series from the French Association of Endocrine Surgeons study group. World J Surg 25: 891–897

    Article  CAS  PubMed  Google Scholar 

  13. Komminoth P, Roth J, Schroder S et al. (1995) Overlapping expression of immunohistochemical markers and synaptophysin mRNA in pheochromocytomas and adrenocortical carcinomas. Implications for the differential diagnosis of adrenal gland tumors. Lab Invest 72: 424–431

    CAS  PubMed  Google Scholar 

  14. Kreft B, Alber P (2007) Tumoren der Niere und der ableitenden Harnwege. Springer, Berlin Heidelberg New York

  15. Li Q, Johansson H, Kjellman M, Grimelius L (1998) Neuroendocrine differentiation and nerves in human adrenal cortex and cortical lesions. APMIS 106: 807–817

    Article  CAS  PubMed  Google Scholar 

  16. Lucon AM, Pereira MA, Mendonca BB et al. (2002) Adrenocortical tumors: results of treatment and study of Weiss’s score as a prognostic factor. Rev Hosp Clin Fac Med Sao Paulo 57: 251–256

    PubMed  Google Scholar 

  17. Miettinen M (1992) Neuroendocrine differentiation in adrenocortical carcinoma. New immunohistochemical findings supported by electron microscopy. Lab Invest 66: 169–174

    CAS  PubMed  Google Scholar 

  18. Morimoto R, Satoh F, Murakami O et al. (2008) Immunohistochemistry of a proliferation marker Ki67/MIB1 in adrenocortical carcinomas: Ki67/MIB1 labeling index is a predictor for recurrence of adrenocortical carcinomas. Endocr J 55: 49–55

    Article  PubMed  Google Scholar 

  19. Paton BL, Novitsky YW, Zerey M et al. (2006) Outcomes of adrenal cortical carcinoma in the United States. Surgery 140: 914–920

    Article  PubMed  Google Scholar 

  20. Pommier RF, Brennan MF (1992) An eleven-year experience with adrenocortical carcinoma. Surgery 112: 963–970

    CAS  PubMed  Google Scholar 

  21. Saeger W (2000) Histopathological classification of adrenal tumours. Eur J Clin Invest 30 (Suppl 3): 58–62

    Article  PubMed  Google Scholar 

  22. Saeger W (2004) Proliferation markers and cell cycle inhibitors in pituitary adenomas. Front Horm Res 32: 110–126

    Article  CAS  PubMed  Google Scholar 

  23. Schroder S, Padberg BC, Achilles E et al. (1992) Immunocytochemistry in adrenocortical tumours: a clinicomorphological study of 72 neoplasms. Virchows Arch A Pathol Anat Histopathol 420: 65–70

    Article  CAS  PubMed  Google Scholar 

  24. Schulick RD, Brennan MF (1999) Long-term survival after complete resection and repeat resection in patients with adrenocortical carcinoma. Ann Surg Oncol 6: 719–726

    Article  CAS  PubMed  Google Scholar 

  25. Stojadinovic A, Ghossein RA, Hoos A et al. (2002) Adrenocortical carcinoma: clinical, morphologic, and molecular characterization. J Clin Oncol 20: 941–950

    Article  CAS  PubMed  Google Scholar 

  26. Tsvetov G, Shimon I, Benbassat C (2007) Adrenal incidentaloma: clinical characteristics and comparison between patients with and without extraadrenal malignancy. J Endocrinol Invest 30: 647–652

    CAS  PubMed  Google Scholar 

  27. van Slooten H, Schaberg A, Smeenk D, Moolenaar AJ (1985) Morphologic characteristics of benign and malignant adrenocortical tumors. Cancer 55: 766–773

    Article  Google Scholar 

  28. Weiss LM (1984) Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors. Am J Surg Pathol 8: 163–169

    Article  CAS  PubMed  Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor weist auf folgende Beziehungen hin:

Referententätigkeit für Novo Nordisk Pharma GmbH, Bayer Vital AG und Roche Diagnostics.

Author information

Authors and Affiliations

  1. Klinik für Innere Medizin III, Friedrich-Schiller-Universität, Erlanger Allee 101, 07740, Jena, Deutschland

    W. Hunger-Battefeld, A. Mandecka, U.A. Müller & G. Wolf

  2. Institut für Pathologie, Universitätsklinikum Jena, Jena, Deutschland

    M. Gajda

  3. Institut für diagnostische und interventionelle Radiologie , Universitätsklinikum Jena, Jena, Deutschland

    A. Hansch

Authors
  1. W. Hunger-Battefeld
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. Gajda
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. A. Hansch
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. A. Mandecka
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. U.A. Müller
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. G. Wolf
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to W. Hunger-Battefeld.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Hunger-Battefeld, W., Gajda, M., Hansch, A. et al. Diagnostische Fallstricke beim Cushing-Syndrom. Internist 51 (Suppl 1), 293–302 (2010). https://doi.org/10.1007/s00108-009-2493-9

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00108-009-2493-9

Schlüsselwörter

Keywords

Search

Navigation