摘要
目的:总结异位嗜铬细胞瘤的临床特点,以增进对本病的认识,提高诊断和治疗水平。方法:回顾性分析本院1975年至2008年共17例经病理证实为异位嗜铬细胞瘤患者的临床资料。结果:17例中,良性者占70.59%(12/17),恶性者占29.41%(5/17)。单发者占88.24%(15/17),多发者占11.76%(2/17)。血压正常者占52.94%(9/17),伴有高血压病史者占47.06%(8/17)。17例患者中检测24小时尿3-甲氧基4羟基苦杏仁酸(VMA)共11例,其中7例高于正常,VMA异常升高阳性率为63.64%(7/11)。17例中1例发生于膀胱,余16例均发生于腹部:腹主动脉旁41.18%(7/17)、下腔静脉旁17.65%(3/17)、肾门附近11.76%(2/17),邻近胰头、降结肠、十二指肠水平部、骶骨前各5.88%(1/17)。位于脊柱左侧占52.94%(9/17),位于脊柱右侧占41.18%(7/17),位于脊柱中央占5.88%(1/17)。病灶最大直径>5cm者占82.35%(14/17),<5cm者占17.65%(3/17)。17例中手术治疗者占94.12%(16/17),根治性切除肿瘤者占70.59%(12/17),姑息性切除肿瘤者占5.88%(1/17),无法切除仅行病理学检查明确病理者占17.65%(3/17)。开腹手术中触摸或挤压肿物时,46.67%(7/15)出现血压明显增高。结论:异位嗜铬细胞瘤很少见,早期诊断较困难。其恶性发病率较发生在肾上腺的嗜铬细胞瘤高;病理学检查不是恶性嗜铬细胞瘤诊断的金标准。患者常伴高血压病史;病灶多邻近腹主动脉、下腔静脉、肾门;单发者较多;就诊时肿瘤最大直径常在5cm以上;检测24小时尿VMA是诊断的重要生化指标;手术切除是唯一的根治性治疗手段,术中触摸或挤压肿物时易出现血压明显增高。根治性切除预后良好;手术成败关键之一是良好的术前扩容准备。
Objective： To summarize clinical data of ectopic pheochromocytoma in order to improve the diagnosis and treatment of this disease. Methods： Clinical data of 17 cases of pathologically proved ectopic pheochromocytoma seen in our institution between 1975 and 2008 were retrospectively analyzed. Results： Of the 17 patients with ectopic pheochromocytoma, 70.59% （12/17） were benign, 29.41% （5/17） were malignant, 88.24% （15/17） were single, 11.76% （2/17） were multiple, 52.94% （9/17） had normal biood pressure and 47.06% （8/17） had hypertension. 24-hour urine vanillylmandelic acid was measured in 11 cases and 7 of them had increased level. The anatomic locations of the ectopic pheochromocytomas were as follows： adjacent to the abdominal aorta in 41.18% （7/17）, adjacent to the inferior vena cava in 17.65% （3/17）, adjacent to the renal hilum in 11.76% （2/17）, in the bladder in 5.88% （1/17）, adjacent to the head of pancreas in 5.88% （1/17）, adjacent to the descending colon in 5.88% （1/17）, adjacent to the horizontal part of duodenum in 5.88% （1/ 17）, adjacent to the sacrum in 5.88% （1/17）, on the left of the lumbar in 52.94% （9/17）, on the right of the lumbar in 41.18% （7/17）, in the middle of the lumbar in 5.88% （1/17）. The maximum diameter of the lesions was longer than 5 cm in 82.35% （14/17） patients and shorter than 5 cm in 17.65% （3/17） patients. Of the 17 cases, 16 cases received surgery. Twelve cases had radical resection, 1 case had palliative resection, and 3 cases were unresectable. Blood pressure was obviously increased in 46.67% patients during surgery when the tumor was touched or pressed. Conclusion： Ectopic pheochromocytoma is a rare tumor and its early diagnosis is rather difficult. The rate of malignancy of ectopic pheochromocytoma is higher than that of renal pheochro-mocytoma. Routine pathological examination is not a gold standard of judging malignant pheochromocytoma. Patients often have hypertension and a single lesion. The abdominal aorta, inferior vena cava and renal hilum are common locations of ectopic lesions. The maximum diameter of the lesions is usually longer than 5 cm at diagnosis. 24-hour urine vanillylmandelic acid is important for diagnosis. Surgical resection is the only option of radical treatment. Blood pressure maybe obviously increased when the tumor is touched or pressed during surgery. Prognosis is good for patients treated with radical surgery. Good premedication is a key factor for a successful surgery.
出处
《中国肿瘤临床》
CAS
CSCD
北大核心
2009年第10期568-571,共4页
Chinese Journal of Clinical Oncology
关键词
异位嗜铬细胞瘤
诊断
治疗
Ectopic pheochromocytoma
Diagnosis
Treatment
