針對嗜鉻細胞瘤之臨床惡性分析與復發轉移預後，是否不同於過去文獻？
陳鵬1、魏子鈞1,2、張延驊1,2、鍾孝仁1,2、黃逸修1,2、林子平1,2、林登龍1,2、黃志賢1,2
1臺北榮民總醫院 泌尿部；
2國立陽明大學醫學院泌尿學科及書田泌尿科學研究中心
Pheochromocytoma: the “ten-percent” malignant tumor? Ki-67 as an independent factor for recurrence or metastasis
Peng Chen1, Tzu-Chun Wei1,2, Yen-Hwa Chang1,2, Hsiao-Jen Chung1,2, Eric Yi-Hsiu Huang1,2,
Tzu-Ping Lin1,2, Alex T.L. Lin1,2, William J. Huang1,2
1 Department of Urology, Taipei Veterans General Hospital;
2 Department of Urology, School of Medicine and Shu-Tien Urological Institute,
National Yang-Ming University, Taipei, Taiwan
Purpose
Pheochromocytoma or paraganglioma, a relative rare neuroendocrine tumor arising from chromaffin cells of the adrenal medulla or neural crest progenitors outside of the adrenal gland, was known as a ten-percent tumor, with malignancy, bilaterality, inheritance, extra-adrenal location, and metastatic or recurrent potential representing around 10% according to previous epidemiology studies. However, its clinical presentations have been so variable that it is described as "the great masquerader". Besides, it was recently re-classified as a malignant tumor according to WHO Classification of Endocrine Organs (2017, 4th edition) due to its catecholamie-induced possible lethality and metastatic potential. Therefore, we aimed to analyze the disease and to find factors for recurrence or metastasis.
Materials and Methods
Patients diagnosed with pheochromocytoma from 2010 to 2019 were reviewed retrospectively in single institute. Demographic data, including classical symptoms of five Ps (pressure, pain, palpitation, perspiration, pallor) were recorded, together with biochemistry related to pheochromocytoma (vanillylmandelic acid, catecholamine) and pathological features including tumor size, laterality, proliferation index (Ki-67), and disease recurrence or metastasis. Both descriptive and comparative statistical analyses were performed.
Results
Among total 73 patients included, 37 (50.7%) were male, with the mean age of 52.1 ± 12.6 and 2 (2.7%) bilateral tumors at diagnosis. Family history or inherited disease was seen in 3 patients (4.1%) of our study. The mean follow-up duration was 27.9 ± 28.9 months, and 10 (13.7%) patients were observed with disease recurrence or metastasis. Comparing metastasis group with non-metastasis group, demographic data, clinical presentation, vanillylmandelic acid, catecholamine and most of the tumor characteristics were not statistically different. As for Ki-67 index, significantly higher value was noted in metastasis group (p=0.002). By multivariate logistic regression, Ki-67 index with cutoff at 3% was an independent risk factor for metastasis or recurrence after treatment (p=0.014).
Conclusions
Being a relatively rare disease, the diagnosis of pheochromocytoma relies much on clinical presentation and biochemistry. While the latest classification by WHO defined pheochromocytoma as malignant tumor, to search for factors related to the potential of metastasis or recurrence might be crucial. In our study, we found none of the clinical characteristics but Ki-67 index as the independent factor for metastatic or recurrent potential, especially by the cutoff at 3%.