中华临床医师杂志（电子版）
中華臨床醫師雜誌（電子版）
중화림상의사잡지（전자판）
CHINESE JOURNAL OF CLINICIANS(ELECTRONIC VERSION)
2015年
2期
200-205
,共6页
曹万里%黄宝星%成康%宿恒川%黄欣%戴军%周文龙%祝宇沈周俊%费晓春%宁光%孙福康%Sun Fukang
曹萬裏%黃寶星%成康%宿恆川%黃訢%戴軍%週文龍%祝宇瀋週俊%費曉春%寧光%孫福康%Sun Fukang
조만리%황보성%성강%숙항천%황흔%대군%주문룡%축우침주준%비효춘%저광%손복강%Sun Fukang
嗜铬细胞瘤%副神经节瘤%甲氧基肾上腺素%甲氧基去甲肾上腺素
嗜鉻細胞瘤%副神經節瘤%甲氧基腎上腺素%甲氧基去甲腎上腺素
기락세포류%부신경절류%갑양기신상선소%갑양기거갑신상선소
Phaechromocytoma%Paraganglioma%Metanephrine%Normetanephrine
目的：进一步提高血浆游离甲氧基肾上腺素（MN）、甲氧基去甲肾上腺素（NM N）正常的嗜铬细胞瘤（Pheos）和副神经节瘤（PGLs）患者的诊断水平。方法本研究组回顾性分析了本中心2004年到2012年电子数据库中病理确认的Pheos和PGLs（PPGLs）患者的临床症状与体征、内分泌激素水平、遗传综合征特征、增强CT表现和核素显像特点。根据血浆游离MNs的水平将患者分为M Ns正常组与M Ns增高组，比较两组患者的上述临床特点。结果病理证实的PPGLs患者共189例，其中MNs正常组24例，增高组165例。MNs正常组患者高血压、阵发性高血压、阵发性的头痛、心悸、大汗三联征症状≥2项、体重下降发生率明显低于增高组（ P＜0.05）；M Ns正常组患者24 h尿肾上腺素、去甲肾上腺素、多巴胺的水平相对增高组明显降低，尤其24 h尿去甲肾上腺素仅是后者平均水平的1/6（P＝0.001）。虽然增强CT上MNs正常组PPGLs肿块明显不均匀强化发生率较增高组低14.8%（P＝0.101），但是它仍占该组PPGLs肿块CT增强形式的主要部分；两组中患者肿瘤增强CT上所有增强形式的发生率无明显统计学差异（P＞0.05）。核素显像对两种肿瘤的发现率相似（P＝1.000）。结论血浆游离M Ns正常的PPGLs患者的临床症状与体征不典型，儿茶酚胺水平降低；增强 CT 上肿块明显不均匀强化的特征以及核素扫描阳性比内分泌激素检测更有助于血浆游离MNs正常PPGLs的诊断。
目的：進一步提高血漿遊離甲氧基腎上腺素（MN）、甲氧基去甲腎上腺素（NM N）正常的嗜鉻細胞瘤（Pheos）和副神經節瘤（PGLs）患者的診斷水平。方法本研究組迴顧性分析瞭本中心2004年到2012年電子數據庫中病理確認的Pheos和PGLs（PPGLs）患者的臨床癥狀與體徵、內分泌激素水平、遺傳綜閤徵特徵、增彊CT錶現和覈素顯像特點。根據血漿遊離MNs的水平將患者分為M Ns正常組與M Ns增高組，比較兩組患者的上述臨床特點。結果病理證實的PPGLs患者共189例，其中MNs正常組24例，增高組165例。MNs正常組患者高血壓、陣髮性高血壓、陣髮性的頭痛、心悸、大汗三聯徵癥狀≥2項、體重下降髮生率明顯低于增高組（ P＜0.05）；M Ns正常組患者24 h尿腎上腺素、去甲腎上腺素、多巴胺的水平相對增高組明顯降低，尤其24 h尿去甲腎上腺素僅是後者平均水平的1/6（P＝0.001）。雖然增彊CT上MNs正常組PPGLs腫塊明顯不均勻彊化髮生率較增高組低14.8%（P＝0.101），但是它仍佔該組PPGLs腫塊CT增彊形式的主要部分；兩組中患者腫瘤增彊CT上所有增彊形式的髮生率無明顯統計學差異（P＞0.05）。覈素顯像對兩種腫瘤的髮現率相似（P＝1.000）。結論血漿遊離M Ns正常的PPGLs患者的臨床癥狀與體徵不典型，兒茶酚胺水平降低；增彊 CT 上腫塊明顯不均勻彊化的特徵以及覈素掃描暘性比內分泌激素檢測更有助于血漿遊離MNs正常PPGLs的診斷。
목적：진일보제고혈장유리갑양기신상선소（MN）、갑양기거갑신상선소（NM N）정상적기락세포류（Pheos）화부신경절류（PGLs）환자적진단수평。방법본연구조회고성분석료본중심2004년도2012년전자수거고중병리학인적Pheos화PGLs（PPGLs）환자적림상증상여체정、내분비격소수평、유전종합정특정、증강CT표현화핵소현상특점。근거혈장유리MNs적수평장환자분위M Ns정상조여M Ns증고조，비교량조환자적상술림상특점。결과병리증실적PPGLs환자공189례，기중MNs정상조24례，증고조165례。MNs정상조환자고혈압、진발성고혈압、진발성적두통、심계、대한삼련정증상≥2항、체중하강발생솔명현저우증고조（ P＜0.05）；M Ns정상조환자24 h뇨신상선소、거갑신상선소、다파알적수평상대증고조명현강저，우기24 h뇨거갑신상선소부시후자평균수평적1/6（P＝0.001）。수연증강CT상MNs정상조PPGLs종괴명현불균균강화발생솔교증고조저14.8%（P＝0.101），단시타잉점해조PPGLs종괴CT증강형식적주요부분；량조중환자종류증강CT상소유증강형식적발생솔무명현통계학차이（P＞0.05）。핵소현상대량충종류적발현솔상사（P＝1.000）。결론혈장유리M Ns정상적PPGLs환자적림상증상여체정불전형，인다분알수평강저；증강 CT 상종괴명현불균균강화적특정이급핵소소묘양성비내분비격소검측경유조우혈장유리MNs정상PPGLs적진단。
Objective This research is to increase our ability to make more accurate diagnosis about phaechromocytoma and paraganglioma (PPGLs) patients with normal plasma free MNs. Methods A retrospective research was conducted in our hospital’ selectronic data base recorded from 2004 to 2012, where pathological confirmed phaechromocytoma(Pheos) and paragangliomas (PGLs) patients were included for analyzing the clinical symptoms and signs, endocrine hormone level, enhanced-contrast CT and radionuclide imaging feathers. Patients were assigned to Group 1 with normal plasma free MNs and Group 2 with elevated plasma free MNs. The above characteristics were compared between both groups. Results 189 patients were included for comprehensive analysis, of which 24 patients were enrolled into Group 1. The prevalence of hypertension, paroxysmal hypertension, no less than two symptoms of paroxysmal episodes of headache, sweating and palpitations, weight losses in Group 1 were significantly lower than patients in Group 2 (P<0.05). The average 24-h urinary epinephrine, dopamine and norepinephrine in Group 1 were lower than the matched group, especially for the average 24-h urinary norepinephrine, which was 1/6 that of patients in Group 2 (P=0.001). Despite the prevalence of hyperattenuation and heterogenous enhancement decreased by 14.8% compared to that of patients in Group 2 (P=0.101), it was still predominant among all enhancement patterns of enhanced-contrast CT in Group 1. There was no significant difference when it came to the prevalence of all kinds of enhance patterns on enhanced-contrast CT between both groups (P>0.05). Radionuclide imaging showed a similar positive detecting rate between two groups (P=1.000). Conclusion The suspicious clinical symptoms and signs for PPGLs were not that apparent in PPGLs patients with normal plasma free MNs, who were associated with a decreased catecholamine secretion. Hyperattenuation and heterogenous enhancement at enhanced-contrast CT or positive radionuclide imaging may attribute to a more accurate diagnosis of those patients than biochemical tests.
