Department of Urology, Urological Science Institute, Yonsei University College of Medicine, Seoul, Korea.
Yonsei Med J. 2011 Jan;52(1):45-50. doi: 10.3349/ymj.2011.52.1.45.
We report herein 119 patients with pheochromocytoma at our institute over the last 23 years.
Between 1986 and 2009, 119 patients were diagnosed with pheochromocytoma at our institute. We reviewed the medical records of these patients.
Of 119 patients, 45 were male and 74 were female, and mean age was 43.83 ± 13.49 years. Forty-three patients (36.1%) were diagnosed incidentally, and 8 patients (6.7%) were found to have familial pheochromocytoma. The mean dimension of the tumors was 5.89 ± 3.18 cm. 4 patients had bilateral tumors; three of these patients were found to have familial pheochromocytoma and 1 patient was diagnosed with malignant pheochromocytoma. A total of eight patients (6.7%) were found to have malignant pheochromocytoma. In 1 patient, metastasis to a lymph node was found at the time of diagnosis. Metastases were found at a mean of 49 ± 25.83 (6-75) months after surgery in the other seven patients. 6 patients died of malignant pheochromocytoma at a mean of 31 ± 28.71 months (1-81) after diagnosis, and the other 2 patients survived for 15 and 24 months, respectively.
Approximately 35% of patients with pheochromocytoma are diagnosed incidentally, and the number of detected cases is increasing. Although familial pheochromocytoma was found only in 6.7% of the patients, genetic testing should be considered in all patients, especially in patients with a family history, young age, or multifocal, bilateral, extra-adrenal, or malignant tumors. Given that malignant pheochromocytomas are frequently diagnosed during the follow-up period, long-term follow-up is necessary to confirm the absence of recurrence or metastasis.
我们在此报告了过去 23 年来我院诊治的 119 例嗜铬细胞瘤患者。
1986 年至 2009 年,我院共诊断 119 例嗜铬细胞瘤患者。我们回顾了这些患者的病历资料。
119 例患者中,男 45 例,女 74 例,平均年龄 43.83±13.49 岁。43 例(36.1%)为偶然发现,8 例(6.7%)为家族性嗜铬细胞瘤。肿瘤平均大小为 5.89±3.18cm。4 例为双侧肿瘤,其中 3 例为家族性嗜铬细胞瘤,1 例为恶性嗜铬细胞瘤。共 8 例(6.7%)为恶性嗜铬细胞瘤。1 例患者在诊断时发现淋巴结转移。术后 75 个月(6-75 个月),其余 7 例患者分别发现远处转移。6 例患者在诊断后 31 个月(1-81 个月)死于恶性嗜铬细胞瘤,其余 2 例患者分别存活 15 个月和 24 个月。
约 35%的嗜铬细胞瘤患者为偶然发现,且检出病例数呈增加趋势。尽管家族性嗜铬细胞瘤仅占患者的 6.7%,但应考虑对所有患者进行基因检测,特别是有家族史、年龄较小、多发、双侧、肾上腺外或恶性肿瘤的患者。由于恶性嗜铬细胞瘤常在随访期间被诊断,因此需要进行长期随访以确认是否有复发或转移。