Skip to main content
SpringerLink
Log in

Malignes Phäochromozytom

  • Chapter
  • First Online:
Uroonkologie

  • 2906 Accesses

Zusammenfassung

Hinweise auf die Pathogenese maligner Phäochromozytome ergeben sich aus der Untersuchung hereditärer Tumorsyndrome. Diverse Malignome sind mit gehäuft mit einem Phäochromozytom vergesellschaftet (von-Hippel-Lindau-Erkrankung, medulläres Schilddrüsenkarzinom, primärer Hyperparathyroidismus, Neurofibromatose 1, familiäres Paragangliom). Eine sinnvolle Stadieneinteilung ist für diese urologische Krebserkrankung bisher nicht etabliert. Es existieren jedoch Scores zur Beurteilung des malignen Potenzials von Phäochromozytomen.

Der Beitrag beschreibt die prognostischen Faktoren ebenso wie das diagnostische wie therapeutische Vorgehen. Hier ist eine präoperative medikamentöse Vorbehandlung von besonderer Bedeutung, um hypertensive Krisen, ausgelöst durch Anästhesie oder Tumormanipulation, zu vermeiden. Die Notwendigkeit zur Nachsorge besteht lebenslang.

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

Access this chapter

Log in via an institution

Subscribe and save

Springer+ Basic
$34.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or eBook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 229.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

Literatur

  • Amar L, Baudin E, Burnichon N, Peyrard S, Silvera S, Bertherat J, Bertagna X, Schlumberger M, Jeunemaitre X, Gimenez-Roqueplo AP and Plouin PF (2007). Succinate dehydrogenase B gene mutations predict survival in patients with malignant pheochromocytomas or paragangliomas. J Clin Endocrinol Metab 92 (10): 3822–8

    Article  CAS  PubMed  Google Scholar 

  • Averbuch SD, Steakley CS, Young RC et al. (1988) Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine. Ann Intern Med 109: 267–273

    Article  CAS  PubMed  Google Scholar 

  • Castellani MR, Chiti A, Seregni E et al. (2000) Role of 131I-metaiodobenzylguanidine (MIBG) in the treatment of neuroendocrine tumours. Experience of the National Cancer Institute of Milan. Q J Nucl Med 44: 77–87

    CAS  PubMed  Google Scholar 

  • Druce MR, Kaltsas GA, Fraenkel M, Gross DJ, Grossman AB (2009) Novel and evolving therapies in the treatment of malignant phaeochromocytoma: experience with the mTOR inhibitor everolimus (RAD001). Horm Metab Res 41 (9): 697–702

    Article  CAS  PubMed  Google Scholar 

  • Edstrom Elder E, Hjelm Skog AL, Hoog A et al. (2003) The management of benign and malignant pheochromocytoma and abdominal paraganglioma. Eur J Surg Oncol 29: 278–283

    Article  CAS  PubMed  Google Scholar 

  • Eisenhofer G, Bornstein SR, Brouwers FM et al. (2004) Malignant pheochromocytoma: current status and initiatives for future progress. Endocr Relat Cancer 11: 423–436

    Article  CAS  PubMed  Google Scholar 

  • Eisenhofer G, Lenders JW, Siegert G, Bornstein SR, Friberg P, Milosevic D, Mannelli M, Linehan WM, Adams K, Timmers HJ, Pacak K (2011) Plasma methoxytyramine: A novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. Eur J Cancer [Epub ahead of print]Forrer F, Riedweg I, Maecke HR, Mueller-Brand J (2008) Radiolabeled DOTATOC in patients with advanced paraganglioma and pheochromocytoma. Q J Nucl Med Mol Imaging 52 (4): 334–40

    Google Scholar 

  • Forrer F, Riedweg I, Maecke HR, Mueller-Brand J (2008) Radiolabeled DOTATOC in patients with advanced paraganglioma and pheochromocytoma. Q J Nucl Med Mol Imaging 52 (4): 334–340

    CAS  PubMed  Google Scholar 

  • Gonias S, Goldsby R, Matthay KK, Hawkins R, Price D, Huberty J, Damon L, Linker C, Sznewajs A, Shiboski S, Fitzgerald P (2009) Phase II study of high-dose [131I]metaiodobenzylguanidine therapy for patients with metastatic pheochromocytoma and paraganglioma. J Clin Oncol 27 (25): 4162–8

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  • Grogan RH, Mitmaker EJ, Duh QY (2011) Changing paradigms in the treatment of malignant pheochromocytoma. Cancer Control 18 (2): 104–12

    PubMed  Google Scholar 

  • Hartley A, Spooner D Brunt AM (2001) Management of malignant phaeochromocytoma: a retrospective review of the use of MIBG and chemotherapy in the West Midlands. Clin Oncol (R Coll Radiol) 13: 361–366

    CAS  Google Scholar 

  • Havekes B, King K, Lai EW, Romijn JA, Corssmit EP, Pacak K (2010) New imaging approaches to phaeochromocytomas and paragangliomas. Clin Endocrinol 72 (2): 137–45

    Article  CAS  Google Scholar 

  • Huang H, Abraham J, Hung E, Averbuch S, Merino M, Steinberg SM, Pacak K, Fojo T (2008) Treatment of malignant pheochromocytoma/paraganglioma with cyclophosphamide, vincristine, and dacarbazine: recommendation from a 22-year follow-up of 18 patients. Cancer 113 (8): 2020–8

    Article  CAS  PubMed  Google Scholar 

  • Joshua AM, Ezzat S, Asa SL, Evans A, Broom R, Freeman M, Knox JJ (2009) Rationale and evidence for sunitinib in the treatment of malignant paraganglioma/pheochromocytoma. J Clin Endocrinol Metab 94 (1): 5–9

    Article  CAS  PubMed  Google Scholar 

  • Kimura N, Watanabe T, Noshiro T, Shizawa S, Miura Y (2005) Histological grading of adrenal and extra-adrenal pheochromocytomas and relationship to prognosis: a clinicopathological analysis of 116 adrenal pheochromocytomas and 30 extra-adrenal sympathetic paragangliomas including 38 malignant tumors. Endocr Pathol 16 (1): 23–32

    Google Scholar 

  • Korevaar TI, Grossman AB (2011) Pheochromocytomas and paragangliomas: assessment of malignant potential. Endocrine 40 (3) 354–65

    Article  CAS  PubMed  Google Scholar 

  • Mundschenk J, Dieterich KD, Kopf D et al. (2001) Phäochromozytom: Klinik, Diagnostik und Therapie. Dtsch Ärztebl 98: 2502–2510

    Google Scholar 

  • Neumann HP, Bausch B, McWhinney SR et al. (2002) Germ-line mutations in nonsyndromic pheochromocytoma. N Engl J Med 346: 1459–1466

    Article  CAS  PubMed  Google Scholar 

  • Rose B, Matthay KK, Price D et al. (2003) High-dose 131I-metaiodobenzylguanidine therapy for 12 patients with malignant pheochromocytoma. Cancer 98: 239–248

    Article  CAS  PubMed  Google Scholar 

  • Shapiro B, Gross MD Shulkin B (2001) Radioisotope diagnosis and therapy of malignant pheochromocytoma. Trends Endocrinol Metab 12: 469–475

    Article  CAS  PubMed  Google Scholar 

  • Shilkrut M, Bar-Deroma R, Bar-Sela G, Berniger A, Kuten A (2010) Low-dose iodine-131 metaiodobenzylguanidine therapy for patients with malignant pheochromocytoma and paraganglioma: single center experience. Am J Clin Oncol 33 (1): 79–82

    Article  CAS  PubMed  Google Scholar 

  • Thompson LD (2002) Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases. Am J Surg Pathol 26 (5): 551–66

    Article  PubMed  Google Scholar 

  • Unger N, Pitt C, Lopez Schmidt (2006) Diagnostic value of various biochemical parameters for the diagnosis of pheochromocytoma in patients with adrenal mass. Eur J Endocrinol 154: 409–417

    Article  CAS  PubMed  Google Scholar 

  • Unger N, Serdiuk I, Sheu SY, Walz MK, Schulz S, Saeger W, Schmid KW, K. Mann and Petersenn S (2008). Immunohistochemical localization of somatostatin receptor subtypes in benign and malignant adrenal tumours. Clin Endocrinol (Oxf) 68 (6): 850–7

    Google Scholar 

  • Welander J, Söderkvist P, Gimm O (2011) Genetics and clinical characteristics of hereditary pheochromocytomas and paragangliomas. Endocr Relat Cancer 18 (6): R253–76

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. ENDOC Zentrum für Endokrine Tumoren, und Praxis für Endokrinologie, Andrologie und medikamentöse Tumortherapie, Altonaerstraße 59, D-20357, Hamburg, Deutschland

    S. Petersenn

  2. Institut für Hämatopathologie, MVZ Hanse Histologikum, Fangdieckstraße 75, D-22547, Hamburg, Deutschland

    K. Mann

  3. Klinik für Nuklearmedizin, Universitätsklinikum Essen, Hufelandstraße 55, D-45122, Essen, Deutschland

    T. Pöppel

  4. Klinik und Poliklinik für Nuklearmedizin, Universitätsklinik Köln, Kerpener Straße 62, D-50937, Köln, Deutschland

    H. Schicha

  5. Klinik für Nuklearmedizin, Universitätsklinikum Essen, Hufelandstraße 55, D-45122, Essen, Deutschland

    A. Bockisch

  6. Klinik und Poliklinik für Urologie, Universitätsklinikum Essen, Hufelandstraße 55, D-45122, Essen, Deutschland

    H. Rübben

Authors
  1. S. Petersenn
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. K. Mann
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. T. Pöppel
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. H. Schicha
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. A. Bockisch
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. H. Rübben
    View author publications

    You can also search for this author in PubMed Google Scholar

Editor information

Editors and Affiliations

  1. Klinik und Poliklinik für Urologie, Universitätsklinikum Essen, Essen, Germany

    Herbert Rübben

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer-Verlag Berlin Heidelberg

About this chapter

Cite this chapter

Petersenn, S., Mann, K., Pöppel, T., Schicha, H., Bockisch, A., Rübben, H. (2014). Malignes Phäochromozytom. In: Rübben, H. (eds) Uroonkologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-35032-0_19

Download citation

  • DOI: https://doi.org/10.1007/978-3-642-35032-0_19

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-35031-3

  • Online ISBN: 978-3-642-35032-0

  • eBook Packages: Medicine (German Language)

Publish with us

Policies and ethics

Search

Navigation