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DOI: 10.1055/s-0029-1231025
© Georg Thieme Verlag KG Stuttgart · New York
Treatment of Malignant Pheochromocytoma
Publikationsverlauf
received 26.03.2009
accepted 15.06.2009
Publikationsdatum:
11. August 2009 (online)

Abstract
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.
Key words
pheochromocytoma - paraganglioma - adrenal mass
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Correspondence
Prof. H. Lehnert
1st Medical Department
University of Lübeck
Ratzeburger Allee 160
23538 Lübeck
Germany
Telefon: +49/451/500 23 06
Fax: 49/451/500 33 39
eMail: Hendrik.Lehnert@uk-sh.de