CAJ | 학술논문

目的总结肾上腺外嗜铬细胞瘤的诊治经验.方法回顾性分析34例肾上腺外嗜铬细胞瘤患者的临床资料.就诊原因:高血压27例,血压170～230/110～155 mm Hg(1 mm Hg=0.133 kPa),病程3个月～15年;腹部疼痛10例,病程5个月～6年;间歇性肉眼血尿2例,病程1周和3个月.34例实验室检查,血、尿儿茶酚胺升高20例,尿3-甲氧-4-羟苦杏仁酸升高24例.B超检查34例,肿瘤准确定位31例,阳性率91.2%.CT扫描25例,24例准确定位,阳性率96.0%.MRI检查6例,均准确定位.15例经腹、3例经耻骨上、16例经腰手术,其中32例完整切除肿瘤,1例行包膜下摘除术,1例行术中活检.结果肿瘤位于肾门旁12例、左肾下极2例、下腔静脉后方1例、腹主动脉和下腔静脉之间3例、腹主动脉前方肠系膜根部2例、右髂总动脉前方1例、肝门部1例、胰腺后方1例、膀胱内2例、降结肠旁1例、多发肿瘤8例(2～3枚).良性嗜铬细胞瘤22例,恶性12例.恶性嗜铬细胞瘤患者中肿瘤单发7例,多发5例.手术切除瘤体直径2～15 cm.术后30例随访6个月～13年.术前27例有高血压症状者,22例血压于术后3 d～6个月恢复正常,5例仍有高血压者中3例舒张压恢复正常,收缩压波动于150～170 mm Hg;2例血压较术前稍有下降.1例膀胱良性嗜铬细胞瘤患者术后6个月肿瘤复发,行膀胱全切.12例恶性嗜铬细胞瘤患者中9例获随访,术后1年内均复发、转移,其中2例为术中未能完整切除肿瘤者,6例术后6个月～3年内死亡,其中3例死于脑溢血,3例死于肿瘤广泛转移;3例经131Ⅰ-间位碘代苄胍(MIBG)治疗后病情稳定,荷瘤生存.结论肾上腺外嗜铬细胞瘤定位较为困难,易漏诊,CT扫描定位准确可靠.手术切除肿瘤是最佳治疗方法.恶性肾上腺外嗜铬细胞瘤术后可给予131Ⅰ-MIBG治疗.
목적 총결신상선외기락세포류적진치경험.방법회고성분석34례신상선외기락세포류환자적림상자료.취진원인:고혈압27례,혈압170～230/110～155 mm Hg(1 mm Hg=0.133 kPa),병정3개월～15년;복부동통10례,병정5개월～6년;간헐성육안혈뇨2례,병정1주화3개월.34례실험실검사,혈、뇨인다분알승고20례,뇨3-갑양-4-간고행인산승고24례.B초검사34례,종류준학정위31례,양성솔91.2%.CT소묘25례,24례준학정위,양성솔96.0%.MRI검사6례,균준학정위.15례경복、3례경치골상、16례경요수술,기중32례완정절제종류,1례행포막하적제술,1례행술중활검.결과 종류위우신문방12례、좌신하겁2례、하강정맥후방1례、복주동맥화하강정맥지간3례、복주동맥전방장계막근부2례、우가총동맥전방1례、간문부1례、이선후방1례、방광내2례、강결장방1례、다발종류8례(2～3매).량성기락세포류22례,악성12례.악성기락세포류환자중종류단발7례,다발5례.수술절제류체직경2～15 cm.술후30례수방6개월～13년.술전27례유고혈압증상자,22례혈압우술후3 d～6개월회복정상,5례잉유고혈압자중3례서장압회복정상,수축압파동우150～170 mm Hg;2례혈압교술전초유하강.1례방광량성기락세포류환자술후6개월종류복발,행방광전절.12례악성기락세포류환자중9례획수방,술후1년내균복발、전이,기중2례위술중미능완정절제종류자,6례술후6개월～3년내사망,기중3례사우뇌일혈,3례사우종류엄범전이;3례경131Ⅰ-간위전대변고(MIBG)치료후병정은정,하류생존.결론 신상선외기락세포류정위교위곤난,역루진,CT소묘정위준학가고.수술절제종류시최가치료방법.악성신상선외기락세포류술후가급여131Ⅰ-MIBG치료.
Objective To review the diagnosis and treatment of extra-adrenal pheochromocytoma with a 34cases report. Methods Thirty-four cases of extra-adrenal pheochromocytoma were retrospectively analyzed. Hypertension was observed in 27 cases. Abdominal pain was seen in 10 patients and intermittent hematuria in 2 patients. Serum and urinary catecholamine and urinary VMA were measured in 34 cases. The level of serum or urinary catecholamine elevated in 20 cases and urine VMA elevated in 24 cases. Thirty-four cases had ultrasound examination,25 cases underwent CT scan and 6 cases underwent MER scan.Results Pheochromocytomas of 12 cases were located in the renal hilum, 2 in the lower pole of the left kidney, 1 in the posterior aspect of the inferior vena cava, 3 in the interaortocaval region, 2 in the anterior aspect of the abdominal aorta, 1 in the anterior of the right common iliac artery, 1 in the hilum of the liver, 1 in the posterior o{ the pancreas, 2 in the bladder wall, 1 in the posterior of the descending colon, and 8 cases of multifoci. Twenty-two cases of extraadrenal pheochromocytoma were benign and 12 cases were malignant. Thirty cases were followed up from 6 months to 13 years. Among 27 cases with hypertension, the blood pressure of 22 patients returned to normal and 5 cases were still hypertensive. Nine cases of malignant pheochromocytoma all had tumor recurrence or metastases at one year postoperatively. Six patients died during followed-up from 6 months to 3 years, including 3 cases died of cerebral hemorrhage and 3 cases of tumor metastases. Three cases got stable with 131Ⅰ-MIBG radiotheraphy.Conclusions The accurate detecting extra-adrenal pheochromocytoma is difficult. CT scan could be reliable in localizing the lesions. Surgical resection of the tumor could be the best therapy. Patients of malignant extra-adrenal pheochromocytoma may be treated with 131Ⅰ-MIBG after surgical therapy.