Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine, and dacarbazine.
OBJECTIVE
To determine the efficacy and toxicity of combination chemotherapy in patients with advanced, malignant pheochromocytoma.


METHODS
Nonrandomized, single-arm trial.


METHODS
Governmental medical referral center.


METHODS
Fourteen patients with malignant pheochromocytoma confirmed by histologic tests. All patients had metastatic disease and elevated urinary catecholamine secretion.


METHODS
After optimization of antihypertensive therapy, patients received cyclophosphamide, 750 mg/m2 body surface area on day 1; vincristine, 1.4 mg/m2 on day 1, and dacarbazine, 600 mg/m2 on days 1 and 2, every 21 days.


RESULTS
Combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine produced a complete and partial response rate of 57% (median duration, 21 months; range, 7 to more than 34). Complete and partial biochemical responses were seen in 79% of patients (median duration, more than 22 months; range, 6 to more than 35). All responding patients had objective improvement in performance status and blood pressure. Toxicity included expected hematologic, neurologic, and gastrointestinal effects of chemotherapy without serious sequelae. There were four minor hypotensive episodes and one minor hypertensive episode.


CONCLUSIONS
Combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine is effective for advanced malignant pheochromocytoma. Urinary catecholamines are useful to ascertain biochemical response to therapy.
Averbuch
S D
SD
National Cancer Institute, Uniformed Services, University of the Health Sciences, Bethesda, Maryland.
Steakley
C S
CS
Young
R C
RC
Gelmann
E P
EP
Goldstein
D S
DS
Stull
R
R
Keiser
H R
HR
D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000188:drug therapy; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use*; D014408:Biomarkers, Tumor / Q000032:analysis; D002395:Catecholamines / Q000032:analysis; D003520:Cyclophosphamide / Q000008:administration & dosage; D003606:Dacarbazine / Q000008:administration & dosage; D005260:Female; D006801:Humans; D008297:Male; D008676:Metanephrine / Q000032:analysis; D008875:Middle Aged; D010673:Pheochromocytoma / Q000188:drug therapy* / Q000378:metabolism / Q000556:secondary; D014642:Vanilmandelic Acid / Q000032:analysis; D014750:Vincristine / Q000008:administration & dosage
D014408:Biomarkers, Tumor; D002395:Catecholamines; D008676:Metanephrine; D014642:Vanilmandelic Acid; D014750:Vincristine; D003606:Dacarbazine; D003520:Cyclophosphamide