title: Clinical review: Current treatment of malignant pheochromocytoma.
abstract: BACKGROUND
  Pheochromocytomas are rare tumors of predominantly adrenal origin that often produce and secrete catecholamines. Malignancy occurs in a variable percentage of cases depending on genetic background and tumor location. Definitive diagnosis relies on the detection of distant metastases. Treatments for malignant pheochromocytoma include surgical debulking, pharmacological control of hormone-mediated symptoms, targeted methods such as external irradiation, and systemic antineoplastic therapy. Different agents and protocols for this purpose are reviewed, and their therapeutic potential is discussed.
  
  
  METHODS
  Literature on antineoplastic therapies for malignant pheochromocytoma was identified by searching the PubMed database with restriction to articles published in English during the past 30 yr.
  
  
  RESULTS
  Because of the rarity of the condition, no randomized clinical trials concerning the treatment of malignant pheochromocytoma have been performed. The strategy established best is [131I]meta-iodobenzylguanidine (MIBG) therapy, which is well tolerated. Similar to cytotoxic chemotherapy with cyclophosphamide, vincristine, and dacarbazine, MIBG can induce remission for a limited period in a significant proportion of patients. Octreotide as a single agent seems to be largely ineffective.
  
  
  CONCLUSIONS
  MIBG radiotherapy and cyclophosphamide, vincristine, and dacarbazine chemotherapy are comparable with respect to response rate and toxicity. It is unclear whether combining both can improve the outcome. Future developments may include new multimodal concepts with focus on inhibition of angiogenetic factors and heat shock protein 90. Any present or new therapeutic approach must take into account the highly variable natural course of the disease.
authors.affiliation: Department of Endocrinology and Metabolism, Otto von Guericke University Medical School, Magdeburg, Germany, and University Hospital of Coventry, UK.
authors.forename: Tim
authors.initials: T
authors.lastname: Scholz
authors.forename: Graeme
authors.initials: G
authors.lastname: Eisenhofer
authors.forename: Karel
authors.initials: K
authors.lastname: Pacak
authors.forename: Henning
authors.initials: H
authors.lastname: Dralle
authors.forename: Hendrik
authors.initials: H
authors.lastname: Lehnert
mesh_terms: D019797:3-Iodobenzylguanidine / Q000627:therapeutic use; D000310:Adrenal Gland Neoplasms / Q000188:drug therapy* / Q000532:radiotherapy*; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003606:Dacarbazine / Q000008:administration & dosage; D006801:Humans; D007457:Iodine Radioisotopes / Q000627:therapeutic use; D010673:Pheochromocytoma / Q000188:drug therapy* / Q000532:radiotherapy*; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D007457:Iodine Radioisotopes; D019797:3-Iodobenzylguanidine; D014750:Vincristine; D003606:Dacarbazine; D003520:Cyclophosphamide