Horm Metab Res 2009; 41(9): 687-696
DOI: 10.1055/s-0029-1231025

© Georg Thieme Verlag KG Stuttgart · New York

Treatment of Malignant Pheochromocytoma

R. Adjallé 1 , P. F. Plouin 2 , K. Pacak 3 , H. Lehnert 1
  • 11st Department of Medicine, University of Lübeck, Lübeck, Germany
  • 2Hypertension Unit, European Hospital Georges Pompidou, University of Paris Descartes, Paris, France
  • 3Endocrinology Branch, NICHD, NIH, Bethesda, MD, USA
Further Information

Publication History

received 26.03.2009

accepted 15.06.2009

Publication Date:
11 August 2009 (online)


Pheochromocytoma (PCC) is a rare disease, mainly sporadic, but also associated with some familial disorders, with a malignancy frequency of approximately 10%. Only the presence of distant metastases, derived from large pleomorphic chromaffin cells, is widely accepted as a criterion of malignancy. Variable symptoms may be caused by production and release of catecholamines. Since there is no curative treatment for malignant PCC and due to its unfavorable prognosis, assuring quality of life is one of the main therapeutic objectives. Besides a long-term medical treatment of symptoms using selective α-1 blockers and nonselective, noncompetitive α- and/or β-blockers, debulking surgery is the first treatment step. In case of a sufficient uptake of 123I-MIBG treatment with targeted radiation therapy, use of 131I-MIBG is an option as an adjuvant therapy, following debulking surgery. Chemotherapy should be applied to patients without positive MIBG-scan, with no response to 131I-MIBG or progression after radionuclide treatment, and especially in cases with high proliferation index. The most effective chemotherapy regimen appears to be the CVD-scheme, including cyclophosphamide, vincristine, and dacarbazine. The so-called targeted molecular therapies with treatment combinations of temozolomide and thalidomide, or sunitinib monotherapy, and novel therapeutic somatostatin analogues have shown promising results and should thus encourage clinical trials to improve the prognosis of metastatic PCC. Within this review the current treatment modalities and novel molecular strategies in the management of this disease are discussed and a treatment algorithm is suggested.



Prof. H. Lehnert

1st Medical Department

University of Lübeck

Ratzeburger Allee 160

23538 Lübeck


Phone: +49/451/500 23 06

Fax: 49/451/500 33 39

Email: [email protected]