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Abstract
Purpose:
We compared clinical characteristics between pheochromocytoma and abdominal paragangliomas and identified predictive factors of malignancy.
Methods:
Between November, 1995 and January, 2011, we retrospectively reviewed the medical records of 145 patients with pheochromocytoma and abdominal paraganglioma at Samsung Medical Center. We compared two tumors (pheochromocytoma vs abdominal paraganglioma) about a potential of hypersecretion of cathecholamines and identified predictive factors of malignancy by analysis of clinical characteristics, biochemical markers, tumor features. Their postoperative results were also evaluated.
Results:
This study included 103 (71%) pheochromocytomas and 42 (29%) abdominal paragangliomas. Eighty-six percent were benign and 14% were malignant. Patients with paraganglioma were more predominantly men and exhibited a higher malignancy rate (P＜0.01) than pheochromocytoma patients. Most (95%) pheochromocytoma was hyperfunctional, but abdominal paraganglioma were hyperfunctional in 74%. There were no significant differences in biochemical markers between the pheochromocytoma and paraganglioma groupd. When compared with benign tumor, malignant tumors were significantly related with higher mean PASS (P＜0.01) and higher 24-hour urinary VMA (P=0.02), but not with larger tumor size.
Conclusion:
It is not easy to distinguish malignant from be-nign tumors by clinical characteristics and pathologic features in the management of pheochromocytoma and paraganglioma. We should keep in mind that abdominal paraganglioma can be also hyperfunctional in many pheochromocytoma patients and has a higher risk of malignancy.
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Table 1.
Clinical data of patients with 103 pheochromocytomas and 42 paragangliomas
| Parameter | Pheochromocytoma | Paraganglioma | P value | ||
|---|---|---|---|---|---|
| Mean age | 52.2 | 56.4 | 0.30 | ||
| No. men/women (proportion of men %) | 41/62 | (40) | 29/13 | (69) | ＜0.01 |
| No. hypertension (%) | 93 | (90) | 34 | (81) | 0.51 |
| Hyperfunction (%) | 98 | (95) | 31 | (74) | |
| Mean tumor largest diameter (cm) | 6.23 | 6.75 | 0.74 | ||
| No. tumor site (%): | |||||
| Rt adrenal gland | 57 | (55) | – | ||
| Lt adrenal gland | 41 | (40) | – | ||
| Bilateral | 5 | (5) | – | ||
| No. malignancy (%) | 10 | (9.7) | 10 | (23.8) | ＜0.01 |
| PASS∗ | 0.95 | 1.41 | 0.07 | ||
| Mean mg/day urine(refrence) [sensitivity %] | |||||
| VMA† (＜6.8) | 21.9 | [76.0] | 18.9 | [71.4] | 0.86 |
| Mean ug/day urine | |||||
| MN‡ (＜290) | 2,226.0 | [60.0] | 1,287.1 | [50.0] | 0.25 |
| NMN§ (＜500) | 5,506 | [84.5] | 4,898 | [61.5] | 0.71 |
| Epi∥ (＜20) | 222.5 | [77.5] | 131.4 | [47.0] | 0.38 |
| NE¶ (＜80) | 1,018.8 | [80.9] | 771.3 | [64.7] | 0.76 |
| Dopa∗∗ (＜400) | 1,181.0 | [36.0] | 940.7 | [35.3] | 0.69 |
| Mean pg/ml plasma | |||||
| Epi∥ (＜90) | 717.1 | [73.4] | 365.9 | [53.8] | 0.18 |
| NE¶ (＜700) | 2,540.3 | [76.6] | 3,177.8 | [69.2] | 0.67 |
| Dopa∗∗ (＜87) | 136.8 | [32.8] | 81.2 | [25.0] | 0.30 |
Table 2.
Location of abdominal paraganglioma in 42 patients
Table 3.
Clinical data of 125 benigns and 20 malignants of pheochromocytoma
| Parameter | Benign | Malignant | P value | ||
|---|---|---|---|---|---|
| Mean age | 53.9 | 50.4 | 0.35 | ||
| No. men/women (proportion of men %) | 57/68 | (46) | 13/7 | (65) | 0.08 |
| No. preop hypertension (%) | 109 | (87) | 18 | (90) | 0.30 |
| Mean tumor largest diameter (cm) | 6.3 | 6.9 | 0.20 | ||
| No. tumor site (%): | |||||
| Rt adrenal gland | 50 | (40) | 6 | (30) | 0.39 |
| Lt adrenal gland | 36 | (29) | 3 | (15) | 0.19 |
| Bilateral | 4 | (3) | 1 | (5) | 0.68 |
| Extra-adrenal | 35 | (28) | 10 | (50) | 0.04 |
| No. tumor recurrence (%) | 0 | 5 | (25) | ＜0.01 | |
| NO. 5-yr survival (%) | 121 | (96.8)†† | 14 | (70.0) | ＜0.01 |
| PASS∗ | 0.84 | 3.6 | ＜0.01 | ||
| Mean, mg/day, urine(reference) [sensitivity %] | |||||
| VMA† (＜6.8) | 17.1 | [73.2] | 35.9 | [86.6] | 0.02 |
| Mean, ug/day, urine | |||||
| MN‡ (＜290) | 1,854.8 | [59.3] | 2,867. | [63.6] | 0.54 |
| NMN§ (＜500) | 4,640 | [82.6] | 6,513 | [77.7] | 0.78 |
| Epi∥ (＜20) | 180.9 | [71.6] | 55.3 | [72.7] | 0.34 |
| NE¶ (＜80) | 801.0 | [77.9] | 1,332.5 | [81.8] | 0.13 |
| Dopa∗∗ (＜400) | 839.1 | [32.6] | 1,117.7 | [35.0] | 0.83 |
| Mean, pg/ml, plasma | |||||
| Epi∥ (＜90) | 571.3 | [70.6] | 707.3 | [75.0] | 0.69 |
| NE¶ (＜700) | 340.3 | [75.0] | 18,581 | [87.5] | 0.25 |
| Dopa∗∗ (＜87) | 88.3 | [29.8] | 326.9 | [50.0] | 0.11 |
Table 4.
Features of 20 patients with malignant pheochromocytoma & paraganglioma
| Patient | Sex | Age | Location | Tumor size (cm) | PASS∗ | Lymph node metastasis | No. of operations | Elapsed time at recurrence or metastasis since 1st diagnosis (yrs) | Follow-up period (yrs) | Survival | Remarks |
|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 62 | Lt.adrenal | 17 | 5 | 2 | ＜1 | 1 | O | ||
| 2 | F | 54 | Rt.adrenal | 8 | 5 | (＋) | 1 | 1 | O | ||
| 3 | M | 48 | Rt.perirenal | 5.5 | 2 | (＋) | 1 | 4 | X | ||
| 4 | F | 47 | Lt.adrenal | 10 | 5 | 0 | 0 | ＜1 | X | Liver, lung meta | |
| 5 | M | 59 | Liver | 7.5 | 2 | 1 | 5 | O | |||
| 6 | M | 54 | Bladder | 7 | 4 | (＋) | 1 | 0 | 7 | O | Multiple bone meta |
| 7 | F | 50 | Small bowel | 4 | 2 | (＋) | 1 | 1 | 5 | X | |
| 8 | M | 43 | Rt.perirenal | 6 | 3 | (＋) | 1 | 4 | O | ||
| 9 | M | 22 | Rt.adrenal | 7.5 | 3 | 1 | 0 | 5 | O | Lung meta | |
| 10 | M | 75 | Rt.perirenal | 3 | 4 | (＋) | 1 | 14 | O | ||
| 11 | M | 65 | Precaval | 5 | 0 | 0 | 8 | O | Liver meta | ||
| 12 | F | 58 | Rt.perirenal | 7.3 | 2 | 1† | 4 | 4 | X | Liver, bone meta | |
| 13 | F | 44 | Rt.adrenal | 4 | (＋) | 1 | 11 | O | |||
| 14 | M | 55 | Aortocaval | 5 | 5 | (＋) | 1 | 1 | O | ||
| 15 | F | 38 | Rt.adrenal | 10 | 3 | 7 | 11 | O | |||
| 16 | M | 48 | Bilateral | 9.5 | 8 | 1 | 0 | 2 | X | Lllung, bone, liver meta | |
| 17 | F | 55 | Rt.adrenal | 9 | 2 | 1 | 6 | 8 | O | ||
| 18 | M | 70 | Portocaval | 3 | 2 | (＋) | 1 | ＜1 | X | ||
| 19 | M | 40 | Lt.adrenal | 4.5 | 2 | 5 | 9 | O | 1 re-op. d/t recurrence | ||
| 20 | M | 8 | Rt.adrenal | 4.5 | 3 | 4 | 14 | O | 2 re-op. d/t recurrence |
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