title: Survival of patients with metastatic malignant pheochromocytoma and efficacy of combined cyclophosphamide, vincristine, and dacarbazine chemotherapy.
abstract: BACKGROUND
  About 10% of pheochromocytomas are malignant. Exact survival has not been reported, nor has an analysis of the efficacy of chemotherapy on survival time.
  
  
  OBJECTIVE
  The aim of this study was to analyze the survival curves and survival times of patients with malignant pheochromocytoma and to determine the efficacy of chemotherapy on prolongation of life.
  
  
  METHODS
  An inception cohort and Kaplan-Meier survival analysis was conducted.
  
  
  METHODS
  Thirty-two patients with metastasized malignant pheochromocytoma were analyzed for survival. Twenty-five patients had undergone excision of their primary tumors. Survival curves were compared among the 16 patients in this group treated with combined chemotherapy using cyclophosphamide, vincristine and dacarbazine (CVD) and the nine patients not treated with chemotherapy.
  
  
  RESULTS
  The survival curve of the 32 patients declined continuously and linearly to at least 20 yr after the diagnosis of pheochromocytoma. The 50% survival rate was estimated to be 14.7 yr. In the 25 patients whose primary tumor was excised, patients who already had metastases at the time of pheochromocytoma diagnosis had better survival than those whose metastases were found later. The survival rate after diagnosis of metastasis was worse in the CVD group than in controls. When the effects of CVD were examined after stratifying several factors, female gender and adrenal origin of tumor were found to be negative prognostic factors for CVD chemotherapy.
  
  
  CONCLUSIONS
  The present study revealed a long survival time. CVD chemotherapy was not shown to extend survival, especially for women and patients with adrenal gland-derived primary tumors.
authors.affiliation: Department of General Medicine, Tokyo Women's Medical University, 8-1 Kawadacho, Shinjyuku-ku, Tokyo, Japan. nomurak@pcc.twmu.ac.jp
authors.forename: Kaoru
authors.initials: K
authors.lastname: Nomura
authors.forename: Hironari
authors.initials: H
authors.lastname: Kimura
authors.forename: Satoru
authors.initials: S
authors.lastname: Shimizu
authors.forename: Hitomi
authors.initials: H
authors.lastname: Kodama
authors.forename: Takahiro
authors.initials: T
authors.lastname: Okamoto
authors.forename: Takao
authors.initials: T
authors.lastname: Obara
authors.forename: Kazue
authors.initials: K
authors.lastname: Takano
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000188:drug therapy / Q000401:mortality* / Q000473:pathology; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D003520:Cyclophosphamide / Q000008:administration & dosage; D003606:Dacarbazine / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D010673:Pheochromocytoma / Q000188:drug therapy / Q000401:mortality* / Q000473:pathology; D015996:Survival Rate; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D003606:Dacarbazine; D003520:Cyclophosphamide