Pham Tuan H, Moir Christopher, Thompson Geoffrey B, Zarroug Abdalla E, Hamner Chad E, Farley David, van Heerden Jon, Lteif Aida N, Young William F
Department of General and Pediatric Surgery, Mayo Clinic, Rochester, Minnesota 55905, USA.
Pediatrics. 2006 Sep;118(3):1109-17. doi: 10.1542/peds.2005-2299.
The aim of this study was to review our institutional experience managing pheochromocytomas and paragangliomas in children.
A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients < 18 years of age with histologically confirmed pheochromocytoma or paraganglioma.
There were 12 patients with pheochromocytomas and 18 with paragangliomas. The most common presenting symptoms were hypertension (64%), palpitation (53%), headache (47%), and mass-related effects (30%). Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma. Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease. Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of > 6 cm. Surgical resection was performed for 28 patients (93%), with perioperative mortality and major morbidity rates of 0% and 10%, respectively. Resection achieved symptomatic relief for 25 patients (83%). All patients with benign disease appeared cured after resection. For patients with malignant disease, the 5- and 10-year disease-specific survival rates were 78% and 31%, respectively, and the mean survival time was 157 +/- 32 months.
The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of > 6 cm. Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates. Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.
本研究旨在回顾我们机构在治疗儿童嗜铬细胞瘤和副神经节瘤方面的经验。
对梅奥诊所1975年至2005年数据库进行回顾性图表审查，确定了30例年龄小于18岁、经组织学确诊为嗜铬细胞瘤或副神经节瘤的患者。
其中12例为嗜铬细胞瘤患者，18例为副神经节瘤患者。最常见的症状为高血压（64%）、心悸（53%）、头痛（47%）以及与肿块相关的症状（30%）。9例患者（30%）存在基因突变或有嗜铬细胞瘤或副神经节瘤的家族病史记录。14例患者（47%）患有恶性疾病，16例（53%）患有良性疾病。逻辑分析表明，恶性肿瘤的统计学显著危险因素为：（1）副神经节瘤；（2）明显散发型，而非家族型，嗜铬细胞瘤或副神经节瘤；（3）肿瘤大小>6 cm。28例患者（93%）接受了手术切除，围手术期死亡率和主要并发症发生率分别为0%和10%。手术切除使25例患者（83%）症状缓解。所有良性疾病患者术后似乎均已治愈。对于恶性疾病患者，5年和10年疾病特异性生存率分别为78%和31%，平均生存时间为157±32个月。
儿童恶性嗜铬细胞瘤/副神经节瘤的发生率较高（47%），尤其是那些明显散发型疾病、副神经节瘤以及肿瘤直径>6 cm的患者。已知存在基因突变或家族性嗜铬细胞瘤/副神经节瘤的患者更有可能实现显微镜下切缘阴性的切除，且疾病特异性死亡率有所改善。手术切除仍然是嗜铬细胞瘤和副神经节瘤的首选治疗方法。
