Korean J Urol.  2011 Apr;52(4):241-246.

Predictive Characteristics of Malignant Pheochromocytoma

• Park J
• Song C
• Park M
• Yoo S
• Park SJ
• Hong S
• Hong B
• Kim CS
• Ahn H

Abstract

PURPOSE
The prognosis of patients with malignant pheochromocytoma is poor, but the predictive factors are not well understood. We aimed to identify the clinical characteristics predictive of malignancy after initial surgical removal in patients with pheochromocytoma.
MATERIALS AND METHODS
We retrospectively reviewed the records of 152 patients diagnosed with pheochromocytoma, including 5 (3.3%) with metastasis at the time of the initial surgical excision and 12 (7.9%) who developed metastasis during follow-up. To determine the factors predictive of malignancy, we compared clinical, radiographical, and urinary chemical findings between patients with benign and malignant disease. Mean follow-up was 41.5 months (range, 0.9-298 months) after surgery.
RESULTS
Malignant tumors were significantly larger than benign tumors (11.1+/-4.0 cm vs. 6.2+/-3.4 cm, p<0.001), and postoperative persistence of arterial hypertension was more frequent after removal of malignant than benign tumors (p=0.001). Among the 147 patients without metastatic disease at diagnosis, those who developed metastasis had significantly lower concentrations of urinary catecholamine metabolites per unit of tumor, including vanillylmandelic acid (1.2 vs. 3.7 mg/day/cm, p=0.049), epinephrine (4.5 vs. 168.9 microg/day/cm, p=0.008), and norepinephrine (13.1 vs. 121.8 mg/day/cm, p<0.001). The overall 5-year metastasis-free survival rate was 84.4% and was significantly higher in patients with smaller tumors (< or =5.5 vs. >5.5 cm; 90.6% vs. 81.2%, p=0.025) and higher 24-hour secretion of vanillylmandelic acid (>2.1 vs. < or =2.1 mg/day/cm; 94.9% vs. 70.9%, p=0.019).
CONCLUSIONS
Large tumor size (>5.5 cm) and minimally elevated 24-hour urinary vanillylmandelic acid (< or =2.1 mg/day/cm) were significantly associated with a higher probability of a malignant pheochromocytoma portending a lower metastasis-free survival and mandating more rigorous follow-up after surgery.

Keyword

Adrenal gland neoplasms; Catecholamines; Pheochromocytoma; Tumor burden

MeSH Terms

Adrenal Gland Neoplasms
Catecholamines
Epinephrine
Follow-Up Studies
Humans
Hypertension
Neoplasm Metastasis
Norepinephrine
Pheochromocytoma
Prognosis
Retrospective Studies
Survival Rate
Tumor Burden
Vanilmandelic Acid
Catecholamines
Epinephrine
Norepinephrine
Vanilmandelic Acid

Figure

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  FIG. 1 Comparison of 24-hour urinary catecholamine secretion in patients with benign and malignant pheochromocytomas (A: benign vs. all cases malignant, B: benign vs. postoperative recurrent malignant).

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  FIG. 2 Metastasis-free survival relative to tumor size (A) and 24-hour urinary secretion of VMA/unit of tumor diameter (B).

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