Edström Elder E, Hjelm Skog A-L, Höög A, Hamberger B
Department of Surgery, Karolinska Hospital, SE-171 76, Stockholm, Sweden.
Eur J Surg Oncol. 2003 Apr;29(3):278-83. doi: 10.1053/ejso.2002.1413.
To report treatment and outcome in patients with malignant and benign pheochromocytoma and abdominal paraganglioma.
Review of clinical and therapeutic features in 85 patients with pheochromocytoma or abdominal paraganglioma between 1976 and 1999.
Thirty-nine of 85 patients presented with symptoms other than classical paroxysmal attacks. Paragangliomas were more often malignant (7/15) than pheochromocytomas (7/70). No recurrences have occurred in 71 patients with tumours initially classified as benign after a median follow-up time of 144 months (range 7-287). Nine of 14 patients with tumours classified as malignant have developed metastasis and/or local recurrence. Treatment of malignant tumours with cyclophosphamide, vincristine and dacarbazine (CVD) improved or stabilised the disease in three of four patients.
Life long follow-up of patients with benign pheochromocytoma is not necessary. Combination chemotherapy (CVD) is a valid option in the treatment of malignant pheochromocytomas and abdominal paragangliomas.
报告恶性和良性嗜铬细胞瘤及腹部副神经节瘤患者的治疗情况及预后。
回顾1976年至1999年间85例嗜铬细胞瘤或腹部副神经节瘤患者的临床及治疗特征。
85例患者中39例出现非典型阵发性发作以外的症状。副神经节瘤比嗜铬细胞瘤更易发生恶变（15例中有7例恶变，70例嗜铬细胞瘤中有7例恶变）。71例最初分类为良性肿瘤的患者，在中位随访时间144个月（范围7 - 287个月）后无复发。14例分类为恶性肿瘤的患者中有9例发生转移和/或局部复发。用环磷酰胺、长春新碱和达卡巴嗪（CVD）治疗恶性肿瘤，4例患者中有3例病情改善或稳定。
良性嗜铬细胞瘤患者无需终身随访。联合化疗（CVD）是治疗恶性嗜铬细胞瘤和腹部副神经节瘤的有效选择。
