中华病理学杂志
中華病理學雜誌
중화병이학잡지
Chinese Journal of Pathology
2011年
11期
762-766
,共5页
江昌新%曾智%王婷%刘欣%刘蓉%李颖
江昌新%曾智%王婷%劉訢%劉蓉%李穎
강창신%증지%왕정%류흔%류용%리영
肾上腺肿瘤%嗜铬细胞瘤%副神经节瘤%多发性内分泌瘤病
腎上腺腫瘤%嗜鉻細胞瘤%副神經節瘤%多髮性內分泌瘤病
신상선종류%기락세포류%부신경절류%다발성내분비류병
Adrenal gland neoplasms%Pheochromocytoma%Paraganglioma%Multiple endocrine neoplasia
目的 分析肾上腺髓质内、外嗜铬细胞瘤与多发性内分泌肿瘤2型( MEN2)的发病变化,探讨它们在临床症状、体征、病理变化方面的异同点和相互关系.方法 运用游程检验、方差分析、t检验、x2检验,对天津医科大学总医院病理科1993-2008年181例肾上腺髓质内、外嗜铬细胞瘤(根据世界卫生组织《内分泌器官肿瘤病理学和遗传学2004年版》分别将其分为良性、恶性倾向、恶性病变3组)及伴MEN2的检出率、构成比、平均诊断年龄、性别比例及临床表现、病理变化进行统计学分析.结果 16年外检总数167 702例,肾上腺疾病、肾上腺髓质内、外嗜铬细胞瘤的例数(外检率)分别是910例(0.54％)、139例(0.08％)、42例(0.03％),其中嗜铬细胞瘤的良性、恶性倾向、恶性3组的例数(构成比)在肾上腺髓质内分别是102例(73.4％)、29例(20.9％)、8例(5.7％),102例良性病变中伴MEN2者共8例(7.8％)；在肾上腺髓质外分别是18例(42.8％)、12例(28.6％)、12例(28.6％).16年间肾上腺疾病、肾上腺髓质内、外嗜铬细胞瘤及良性病变伴MEN2的检出率和构成比及嗜铬细胞瘤的良性、恶性倾向、恶性3组的构成比均无变化趋势(P＞0.05),并随病变恶性度的增加逐渐由女性多见转变为男性多见的发病规律.嗜铬细胞瘤患者总平均诊断年龄在髓质内良性组、恶性倾向组分别为42.7和40.1岁,均低于恶性组患者的51.6岁.髓质外良性组、恶性倾向组分别为43.1和45.2岁,均高于恶性组的37.8岁(P＜0.05).恶性病变患者中,髓质内的发病年龄(51.6岁)明显高于髓质外(37.8岁,P＜0.05)；伴MEN2患者只见于患肾上腺髓质内良性嗜铬细胞瘤的女性,其平均诊断年龄(38.9岁)低于良性病变(42.7岁),而且甲状腺髓样癌的发生均早于肾上腺髓质内嗜铬细胞瘤.肾上腺髓质内、外嗜铬细胞瘤患者均显示恶性病变伴有高血压症状者的比例较良性、恶性倾向者明显下降(P＜0.05).仅肾上腺髓质内嗜铬细胞瘤发生双侧病变,其中恶性病变( 2/8)的发生率明显高于良性(15.7％)、恶性倾向(6.9％).复发病变在肾上腺髓质内、外嗜铬细胞瘤的比例均随良性(11.8％,0)、恶性倾向(13.8％,25％)、恶性(33.3％,37.5％)的病变恶性度的上升而逐渐增加.肾上腺髓质内、外嗜铬细胞瘤体积平均直径亦随良性(4.2、4.0 cm)、恶性倾向(5.3、5.6 cm)、恶性病变(7.3、6.9 cm)的恶性度上升而逐渐增大(P＜0.05).结论 肾上腺髓质内、外嗜铬细胞瘤良性、恶性倾向、恶性病变3组的临床表现与病理变化密切相关,确切的病变类型和肿瘤性质仍要由病理学检查确定.
目的 分析腎上腺髓質內、外嗜鉻細胞瘤與多髮性內分泌腫瘤2型( MEN2)的髮病變化,探討它們在臨床癥狀、體徵、病理變化方麵的異同點和相互關繫.方法 運用遊程檢驗、方差分析、t檢驗、x2檢驗,對天津醫科大學總醫院病理科1993-2008年181例腎上腺髓質內、外嗜鉻細胞瘤(根據世界衛生組織《內分泌器官腫瘤病理學和遺傳學2004年版》分彆將其分為良性、噁性傾嚮、噁性病變3組)及伴MEN2的檢齣率、構成比、平均診斷年齡、性彆比例及臨床錶現、病理變化進行統計學分析.結果 16年外檢總數167 702例,腎上腺疾病、腎上腺髓質內、外嗜鉻細胞瘤的例數(外檢率)分彆是910例(0.54％)、139例(0.08％)、42例(0.03％),其中嗜鉻細胞瘤的良性、噁性傾嚮、噁性3組的例數(構成比)在腎上腺髓質內分彆是102例(73.4％)、29例(20.9％)、8例(5.7％),102例良性病變中伴MEN2者共8例(7.8％)；在腎上腺髓質外分彆是18例(42.8％)、12例(28.6％)、12例(28.6％).16年間腎上腺疾病、腎上腺髓質內、外嗜鉻細胞瘤及良性病變伴MEN2的檢齣率和構成比及嗜鉻細胞瘤的良性、噁性傾嚮、噁性3組的構成比均無變化趨勢(P＞0.05),併隨病變噁性度的增加逐漸由女性多見轉變為男性多見的髮病規律.嗜鉻細胞瘤患者總平均診斷年齡在髓質內良性組、噁性傾嚮組分彆為42.7和40.1歲,均低于噁性組患者的51.6歲.髓質外良性組、噁性傾嚮組分彆為43.1和45.2歲,均高于噁性組的37.8歲(P＜0.05).噁性病變患者中,髓質內的髮病年齡(51.6歲)明顯高于髓質外(37.8歲,P＜0.05)；伴MEN2患者隻見于患腎上腺髓質內良性嗜鉻細胞瘤的女性,其平均診斷年齡(38.9歲)低于良性病變(42.7歲),而且甲狀腺髓樣癌的髮生均早于腎上腺髓質內嗜鉻細胞瘤.腎上腺髓質內、外嗜鉻細胞瘤患者均顯示噁性病變伴有高血壓癥狀者的比例較良性、噁性傾嚮者明顯下降(P＜0.05).僅腎上腺髓質內嗜鉻細胞瘤髮生雙側病變,其中噁性病變( 2/8)的髮生率明顯高于良性(15.7％)、噁性傾嚮(6.9％).複髮病變在腎上腺髓質內、外嗜鉻細胞瘤的比例均隨良性(11.8％,0)、噁性傾嚮(13.8％,25％)、噁性(33.3％,37.5％)的病變噁性度的上升而逐漸增加.腎上腺髓質內、外嗜鉻細胞瘤體積平均直徑亦隨良性(4.2、4.0 cm)、噁性傾嚮(5.3、5.6 cm)、噁性病變(7.3、6.9 cm)的噁性度上升而逐漸增大(P＜0.05).結論 腎上腺髓質內、外嗜鉻細胞瘤良性、噁性傾嚮、噁性病變3組的臨床錶現與病理變化密切相關,確切的病變類型和腫瘤性質仍要由病理學檢查確定.
목적 분석신상선수질내、외기락세포류여다발성내분비종류2형( MEN2)적발병변화,탐토타문재림상증상、체정、병리변화방면적이동점화상호관계.방법 운용유정검험、방차분석、t검험、x2검험,대천진의과대학총의원병이과1993-2008년181례신상선수질내、외기락세포류(근거세계위생조직《내분비기관종류병이학화유전학2004년판》분별장기분위량성、악성경향、악성병변3조)급반MEN2적검출솔、구성비、평균진단년령、성별비례급림상표현、병리변화진행통계학분석.결과 16년외검총수167 702례,신상선질병、신상선수질내、외기락세포류적례수(외검솔)분별시910례(0.54％)、139례(0.08％)、42례(0.03％),기중기락세포류적량성、악성경향、악성3조적례수(구성비)재신상선수질내분별시102례(73.4％)、29례(20.9％)、8례(5.7％),102례량성병변중반MEN2자공8례(7.8％)；재신상선수질외분별시18례(42.8％)、12례(28.6％)、12례(28.6％).16년간신상선질병、신상선수질내、외기락세포류급량성병변반MEN2적검출솔화구성비급기락세포류적량성、악성경향、악성3조적구성비균무변화추세(P＞0.05),병수병변악성도적증가축점유녀성다견전변위남성다견적발병규률.기락세포류환자총평균진단년령재수질내량성조、악성경향조분별위42.7화40.1세,균저우악성조환자적51.6세.수질외량성조、악성경향조분별위43.1화45.2세,균고우악성조적37.8세(P＜0.05).악성병변환자중,수질내적발병년령(51.6세)명현고우수질외(37.8세,P＜0.05)；반MEN2환자지견우환신상선수질내량성기락세포류적녀성,기평균진단년령(38.9세)저우량성병변(42.7세),이차갑상선수양암적발생균조우신상선수질내기락세포류.신상선수질내、외기락세포류환자균현시악성병변반유고혈압증상자적비례교량성、악성경향자명현하강(P＜0.05).부신상선수질내기락세포류발생쌍측병변,기중악성병변( 2/8)적발생솔명현고우량성(15.7％)、악성경향(6.9％).복발병변재신상선수질내、외기락세포류적비례균수량성(11.8％,0)、악성경향(13.8％,25％)、악성(33.3％,37.5％)적병변악성도적상승이축점증가.신상선수질내、외기락세포류체적평균직경역수량성(4.2、4.0 cm)、악성경향(5.3、5.6 cm)、악성병변(7.3、6.9 cm)적악성도상승이축점증대(P＜0.05).결론 신상선수질내、외기락세포류량성、악성경향、악성병변3조적림상표현여병리변화밀절상관,학절적병변류형화종류성질잉요유병이학검사학정.
Objective To analyze the change in the incidence of pheochromocytomas in adrenal medulla or extra-adrenal and multiple endocrine neoplasm type 2 (MEN2),to summarize the clinical characteristics of benign,potentially malignant and malignant pheochromocytomas and to investigate the correlation between clinical manifestations and pathological changes.Methods Statistic analysis was performed to detect the incidence,constituent ratio,mean diagnostic age,sex proportion and correlation between clinical manifestions and pathologic changes in pheochromocytomas in adrenal medulla or extraadrenal gland and MEN2 from 1993 to 2008 in the Department of Pathology,the General Hospital of Tianjin Medical University with Runs test,ANOVA,t test and chi-square test.Results The total number of biopsies within the 16 years was 167 702 cases (average 10 481 cases per year).The numbers (detectable rate) of total adrenal diseases,pheochromocytomas in adrenal medulla and extra-adrenal glands were 910 (0.54％ ),139 ( 0.08％ ),and 42 ( 0.03％ ) cases,respectively.The numbers ( constituent ratio) of benign,potentially malignant and malignant of pheochromocytomas in adrenal medulla were 102 cases (73.4％),29 cases (20.9％ ) and 8 cases (5.7％ ),respectively; in the 102 cases of benign tumors,patients with MEN2 were 8 (7.8％ ) ; the three groups of the tumors in extra-adrenal sites were 18(42.8％ ) cases,12 (28.6％ ) cases and 12 (28.6％) cases.There were no changes in the detectable rate and constituent ratio of adrenal diseases,benign,potential malignant and malignant pheochromocytomas in adrenal medulla or extra-adrenal glands and patients with MEN2 during the past 16 years (P ＞ 0.05 ),but there was a tendency that malignant transformation was gradually increased with age,which was more commonly found in male patients than females.The mean age at diagnosis of patients with benign and potentially malignant pheochromocytomas was 42.7 years ( ranged from 10-74 years),and 40.1 years ( 13-66 years),respectively,which were younger than patients with malignant pheochromocytomas (51.6 years,P ＜ 0.05 ) ; the mean age of patients with benign and potentially malignant pheochromocytomas in extraadrenal sites was 43.1 years (ranged from 20-75 years) and 45.2 years (28-65 years) that were older than those with malignant ( 37.8 years,ranged from 14-58 years,P ＜ 0.05 ).It was spectacular that patients with malignant pheochromocytoma in adrenal medulla (51.6 years) were older than that in extra-adrenal sites (37.8 years) ; all the patients with MEN2 were female benign pheochromocytoma in adrenal medulla,whose age (38.9 years) was younger than that of benign lesions (42.7 years,P ＜ 0.05 ),in which thyroid medullary carcinoma appeared early than pheochromocytomas in adrenal medulla.The detectable rate of hypertension in patients with malignant pheochromocytomas in adrenal medulla and in extra-adrenal sites were less than that in benign and potentially malignant ones ( P ＜ 0.05 ).The bilateral lesions more commonly found in malignant pheochromocytoma (25.0％) than benign ( 15.7％ ) and potentially malignant pheochromocytomas (6.9％ ) only in adrenal medulla.Relapse rates in both adrenal and extra-adrenal tumors were rising from benign ( 11.8％,0 ),potentially malignant ( 13.8％,25.0％ ),to malignant (33.3％,37.5％ ) groups; the average diameter of pheochromocytomas in both adrenal and extra-adrenal sites was increasing from benign (4.2 cm,4.0 cm),potentially malignant (5.3 cm,5.6 cm) to malignant (7.3 cm,6.9 cm ) groups ( P ＜ 0.05 ).Conclusions The diagnostic criteria of benign,potentially malignant and malignant pheochromocytomas in adrenal medulla and in extra-adrenal sites are well established according to the WHO classification of endocrine tumors (2004).The closer relationship is found between clinical manifestations and pathologic changes.The definite type and nature of pheochromocytomas are mainly rested upon the pathologic examination.
