title: Controlling Tumor Progression with Cyclophosphamide, Vincristine, and Dacarbazine Treatment Improves Survival in Patients with Metastatic and Unresectable Malignant Pheochromocytomas/Paragangliomas.
abstract: Evidence has not been established to support that combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) improves survival in patients with malignant pheochromocytoma and paraganglioma (M-PPGL). To investigate the efficacy of CVD for this disease, we retrospectively analyzed data of 23 patients with metastatic and unresectable M-PPGL (mean age, 41.7 ± 15.4 years) who received at least 2 cycles of this regimen. The follow-up period after initiation of CVD ranged from 0.3 to 13.7 years, with a median of 3.3 years. CVD therapy achieved a complete tumor response (CR) in 1 patient (4%), a partial response (PR) in 5 (22%), stable disease (SD) in 5 (22%), and progressive disease (PD) in 13 (52%), respectively. All of the responders (CR and PR) but 6% of the non-responders (SD and PD) showed substantial biochemical improvement. The progression-free survival period in the responders was significantly longer than in the non-responders (p < 0.01). Although the overall survival and survival after the diagnosis of M-PPGL were longer in the responders than the non-responders, the difference was not statistically significant (p = 0.08). The progression-free and overall survival period were significantly longer in the non-progression group (CR, PR, and SD) than in the progression group (PD) (1.7 ± 3.3 vs. 0.3 ± 0.3 years, p < 0.01, and 4.6 ± 3.6 vs. 2.0 ± 3.7 years, p = 0.01, respectively). It is therefore suggested that CVD chemotherapy could be useful in controlling tumor progression and improving survival in patients with metastatic and progressive M-PPGL.
authors.affiliation: Division of Metabolism and Endocrinology, Department of Internal Medicine, St. Marianna University School of Medicine Yokohama City Seibu Hospital, 1197-1, Yasashicho, Asahi-ku, Yokohama, Kanagawa, 241-0811, Japan.
authors.forename: Shiko
authors.initials: S
authors.lastname: Asai
authors.affiliation: Division of Metabolism and Endocrinology, Department of Internal Medicine, St. Marianna University School of Medicine Yokohama City Seibu Hospital, 1197-1, Yasashicho, Asahi-ku, Yokohama, Kanagawa, 241-0811, Japan. t2kataba@marianna-u.ac.jp.
authors.forename: Takuyuki
authors.initials: T
authors.lastname: Katabami
authors.affiliation: Division of Endocrinology and Metabolism, National Hospital Organization Kyoto Medical Center, 1-1, Mukaihatacho, Fukakusa, Fushimi-ku, Kyoto, 612-8555, Japan.
authors.forename: Mika
authors.initials: M
authors.lastname: Tsuiki
authors.affiliation: Division of Metabolism and Endocrinology, Department of Internal Medicine, St. Marianna University School of Medicine, 2-16-1 Sugao, Miyamae-ku, Kawasaki, Kanagawa, 216-8511, Japan.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Tanaka
authors.affiliation: Division of Endocrinology, Metabolism and Hypertension, Clinical Research Institute, National Hospital Organization Kyoto Medical Center, 1-1, Mukaihatacho, Fukakusa, Fushimi-ku, Kyoto, 612-8555, Japan.
authors.forename: Mitsuhide
authors.initials: M
authors.lastname: Naruse
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000188:drug therapy*; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D003520:Cyclophosphamide / Q000008:administration & dosage* / Q000627:therapeutic use; D003606:Dacarbazine / Q000008:administration & dosage* / Q000627:therapeutic use; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D010235:Paraganglioma / Q000188:drug therapy*; D010673:Pheochromocytoma / Q000188:drug therapy*; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage* / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D014750:Vincristine; D003606:Dacarbazine; D003520:Cyclophosphamide
keywords: Antineoplastic combined chemotherapy; CVD therapy; Malignant pheochromocytoma; Myelodysplastic syndromes; Paraganglioma; Survival time