Table 2.
Clinical profiles of the patients with malignant pheochromocytoma/paraganglioma treated with CVD chemotherapy
| All | Responders | Non-responders | p valuea | |
|---|---|---|---|---|
| Number of patientsNumber of deaths | 239 | 63 | 177 | – |
| Tumor response to CVD, n | – | CR 1/PR 5 | SD 5/PD 12 | – |
| Sex, n (M/F) | 18/5 | 3/3 | 15/2 | 0.09c |
| Primary tumor, n (adrenal/extra-adrenal/multiple) | 7/14/2 | 2/4/0 | 5/10/2 | 0.68d |
| Initial Dx, n (benign/malignant/unknown) | 9/6/8 | 5/0/1 | 4/6/7 | 0.03d |
| Age at Dx of PPGL, years (range) | 41.7 ± 15.4(16∼65) | 32.5 ± 15.5 | 44.9 ± 14.4 | 0.09b |
| Age at Dx of M-PPGL, years (range) | 46.5 ± 13.8(19∼68) | 44.3 ± 13.7 | 47.3 ± 14.2 | 0.70b |
| Maximum tumor diameter, cm (range) | 6.5 ± 5.3(1∼22) | 5.8 ± 4.8 | 6.9 ± 5.7 | 0.84b |
| Metastasis Lung, n (%) | 12 (52.2%) | 3 (50%) | 9 (53%) | 0.99c |
| Liver, n (%) | 9 (39.1%) | 3 (50%) | 6 (35%) | 0.64c |
| Bone, n (%) | 14 (60.9%) | 3 (50%) | 11 (65%) | 0.64c |
| Lymph nodes, n (%) | 11 (47.8%) | 5 (83%) | 6 (35%) | 0.06c |
| Number of metastatic organs | 2.0 ± 0.9 | 2.3 ± 0.8 | 1.9 ± 0.9 | 0.24b |
| Number of CVD cycles (range) | 11.6 ± 10.8(2∼41) | 24.5 ± 10.4 | 7.0 ± 6.5 | <0.01 |
| Biochemical response, n(CR/PR/NC/PD) | 6/1/8/8 | 5/1/0/0 | 1/0/8/8 | <0.01 |
| Dx of PPGL∼Dx of M-PPGL, years (median/range) | 5.2 ± 6.0(4.0/0∼25.3) | 12.4 ± 3.4(10.0/2.8∼25.3) | 3.1 ± 2.4(2.7/0∼8.7) | <0.01 |
The results are expressed as the mean ± standard deviation. Values of p < 0.05 were considered statistically significant (Mann–Whitney U test, Fisher’s exact test, or Pearson’s χ 2 test). Responders were defined as patients whose tumor responded to the CVD treatment (partial response or complete response). Non-responders were defined as patients whose tumors did not show a response to the treatment (stable disease or progressive disease)
CVD cyclophosphamide, vincristine, and dacarbazine, M men, F female, Dx diagnosis, age at Dx of PPGL age at the first diagnosis of pheochromocytoma/paraganglioma, age at Dx of M-PPGL age at diagnosis of malignant pheochromocytoma/paraganglioma, CR complete response, PR partial response, SD stable disease, PD progression disease, NC no change
aResponders vs. non-responders
bMann–Whitney U test
cFisher’s exact test
d χ 2 test