[恶性双侧嗜铬细胞瘤与冯雷克林霍增氏病]
[Malignant bilateral pheochromocytoma and von Recklinghausen disease].
作者信息
Martín Huéscar A, Fernández Gómez J M, Martínez Gómez F J, Regadera Sejas F J, Sahagun Argùello J L, Rabade Rey C J
机构信息
Servicio de Urología, Hospital Covadonga, Oviedo, Asturias, España.
出版信息
Arch Esp Urol. 1996 Jul-Aug;49(6):627-9.
OBJECTIVES
To review the main features of malignant pheochromocytoma and its relationship to von Recklinghausen's neurofibromatosis.
METHODS/RESULTS: We report on a case of malignant bilateral pheochromocytoma with hepatic and pulmonary metastasis, associated with von Recklinghausen's neurofibromatosis. Surgery achieved good results and the patient is well seven years postoperatively.
CONCLUSIONS
Approximately 10% of the pheochromocytomas are malignant and treatment is principally by surgery. (131) metaiodobenzylguanidine may be useful in cases with metastasis, residual tumor or local relapse.
目的
回顾恶性嗜铬细胞瘤的主要特征及其与冯雷克林霍增氏神经纤维瘤病的关系。
方法/结果：我们报告一例伴有肝和肺转移的双侧恶性嗜铬细胞瘤病例，该病例与冯雷克林霍增氏神经纤维瘤病相关。手术取得了良好效果，患者术后七年情况良好。
结论
约10%的嗜铬细胞瘤为恶性，主要治疗方法是手术。对于有转移、残留肿瘤或局部复发的病例，（131）间碘苄胍可能有用。
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