PDF(Pubmed)
Abstract：
BACKGROUND: This case report documents a case of malignant pheochromocytoma manifested as vision changes with lung metastasis and recurrence.
METHODS: A 10-year-old Han Chinese girl presented with vision changes and was eventually diagnosed with pheochromocytoma by contrast-enhanced computed tomography, urine vanillylmandelic acid. After medication for hypertension and surgery, clinical symptoms disappeared. Malignant pheochromocytoma with lung metastasis was confirmed histologically using the Pheochromocytoma of the Adrenal Gland Scaled Score scoring system and genetically with succinate dehydrogenase complex iron sulfur subunit B mutation, and 3 months later, unplanned surgery was performed because of the high risks and signs of recurrence. She is asymptomatic as of the writing of this case report. Our patient\'s case highlights the importance of considering a diagnosis of malignant pheochromocytoma, and long-term follow-up for possible recurrence.
CONCLUSIONS: Although there are well-recognized classic clinical manifestations associated with pheochromocytoma, atypical presentation, such as vision changes in children, should be considered. In addition, malignant pheochromocytoma children with a high Pheochromocytoma of the Adrenal Gland Scaled Score and succinate dehydrogenase complex iron sulfur subunit B mutation require a long-term follow-up or even unplanned surgery because of the higher risk of recurrence.
METHODS: A 10-year-old Han Chinese girl presented with vision changes and was eventually diagnosed with pheochromocytoma by contrast-enhanced computed tomography, urine vanillylmandelic acid. After medication for hypertension and surgery, clinical symptoms disappeared. Malignant pheochromocytoma with lung metastasis was confirmed histologically using the Pheochromocytoma of the Adrenal Gland Scaled Score scoring system and genetically with succinate dehydrogenase complex iron sulfur subunit B mutation, and 3 months later, unplanned surgery was performed because of the high risks and signs of recurrence. She is asymptomatic as of the writing of this case report. Our patient\'s case highlights the importance of considering a diagnosis of malignant pheochromocytoma, and long-term follow-up for possible recurrence.
CONCLUSIONS: Although there are well-recognized classic clinical manifestations associated with pheochromocytoma, atypical presentation, such as vision changes in children, should be considered. In addition, malignant pheochromocytoma children with a high Pheochromocytoma of the Adrenal Gland Scaled Score and succinate dehydrogenase complex iron sulfur subunit B mutation require a long-term follow-up or even unplanned surgery because of the higher risk of recurrence.
摘要：
背景：本病例报道1例恶性嗜铬细胞瘤，表现为视力改变并伴有肺转移和复发。
方法：一名10岁的汉族女孩出现视力变化，最终通过对比增强计算机断层扫描诊断为嗜铬细胞瘤，尿香草扁桃酸.在治疗高血压和手术后，临床症状消失。使用肾上腺刻度评分系统的嗜铬细胞瘤在组织学上证实了具有肺转移的恶性嗜铬细胞瘤，并在遗传上具有琥珀酸脱氢酶复合物铁硫亚基B突变，三个月后,由于高风险和复发迹象,我们进行了非计划手术.在撰写本病例报告时，她没有症状。我们病人的病例突出了考虑诊断恶性嗜铬细胞瘤的重要性，以及可能复发的长期随访。
结论：尽管有公认的与嗜铬细胞瘤相关的经典临床表现，非典型介绍，比如儿童的视力变化，应该考虑。此外,由于复发风险较高,肾上腺嗜铬细胞瘤评分高,琥珀酸脱氢酶复合物铁硫亚基B突变的恶性嗜铬细胞瘤儿童需要长期随访,甚至非计划手术.
方法：一名10岁的汉族女孩出现视力变化，最终通过对比增强计算机断层扫描诊断为嗜铬细胞瘤，尿香草扁桃酸.在治疗高血压和手术后，临床症状消失。使用肾上腺刻度评分系统的嗜铬细胞瘤在组织学上证实了具有肺转移的恶性嗜铬细胞瘤，并在遗传上具有琥珀酸脱氢酶复合物铁硫亚基B突变，三个月后,由于高风险和复发迹象,我们进行了非计划手术.在撰写本病例报告时，她没有症状。我们病人的病例突出了考虑诊断恶性嗜铬细胞瘤的重要性，以及可能复发的长期随访。
结论：尽管有公认的与嗜铬细胞瘤相关的经典临床表现，非典型介绍，比如儿童的视力变化，应该考虑。此外,由于复发风险较高,肾上腺嗜铬细胞瘤评分高,琥珀酸脱氢酶复合物铁硫亚基B突变的恶性嗜铬细胞瘤儿童需要长期随访,甚至非计划手术.
