无功能性肾上腺肿瘤的诊断与治疗(附39例报告)

目的：提高无功能性肾上腺肿瘤的诊治水平。方法：回顾性分析我院17年来39例无功能性肾上腺肿瘤患者的临床资料。结果：39例中,38例行肿瘤切除或剜除术,1例仅行活组织检查;30例病理检查证实为良性无功能性肾上腺肿瘤,其中节细胞神经瘤7例,平滑肌瘤1例,皮质腺瘤10例,髓脂瘤3例,神经鞘瘤1例．肾上腺囊肿8例。随访6个月～7年,临床症状消失,无肿瘤复发。9例病理检查证实为恶性无功能性肾上腺肿瘤,其中脂肪肉瘤1例,皮质腺癌4例,转移癌4例,术后2年内,8例死亡,1例无癌生存14个月。结论：无功能性肾上腺肿瘤临床少见,早期诊断困难,CT是首选检查方法,确诊依赖于病理检查。手术切除是良性肿瘤的有效手段,但对恶性肿瘤预后较差。     

肾上腺外恶性肿瘤伴发肾上腺原发肿瘤的临床特点

目的 了解肾上腺外恶性肿瘤伴发肾上腺原发肿瘤的临床特点.方法 1990年至2004年收治有恶性肿瘤病史或同期其他部位恶性肿瘤伴肾上腺原发肿瘤患者13例.男10例,女3例.中位年龄47岁.肾上腺肿瘤位于左侧6例、右侧4例、双侧3例.肿瘤最大直径中位数4.0cm.肾上腺外肿瘤分别为结直肠腺癌4例、甲状腺髓样癌3例、肺癌2例,乳腺癌、前列腺癌、膀胱癌和十二指肠类癌各1例.结果 原发肾上腺肿瘤13例,占同期肾上腺伴有恶性肿瘤病史或同期其他部位恶性肿瘤的30%(13/44).其中肾上腺皮质腺瘤8例、嗜铬细胞瘤3例、皮质腺癌和血管瘤各1例.8例皮质腺瘤中,B超、CT和MRI诊断准确率分别为3/8、3/7和4/4;3例嗜铬细胞瘤患者均为甲状腺髓样癌术后,B超、CT或MRI检查均确诊;1例肾上腺血管瘤B超、CT或MRI扫描均误诊为转移.失随访1例.伴发肾上腺腺瘤患者8例中,6例无瘤生存3～8年.嗜铬细胞瘤3例无瘤生存8～12年,但2例术后肾上腺皮质功能不全需要激素替代.肾上腺血管瘤患者术后长期生存12年.结论有恶性肿瘤病史患者伴发的肾上腺原发肿瘤以腺瘤多见,影像学是定性、定位诊断的主要手段.对于肾上腺外恶性肿瘤伴发孤立的原发与转移不明的肾上腺肿瘤,建议手术切除.     

肾上腺偶发瘤治疗的临床研究

目的 探讨肾上腺偶发瘤治疗方法.方法 回顾性分析1996年1月至2006年1月156例肾上腺偶发瘤患者的临床资料及随访结果.结果 156例患者中1例因多器官转移行放疗、化疗,4例肿瘤较小者随诊观察,151例接受手术治疗.肿瘤直径1.3～15.0 cm.病理结果提示嗜铬细胞瘤34例,皮质腺瘤83例,皮质癌5例,转移癌3例,肾上腺囊肿等其他良性肿瘤26例.随访136例,随访时间1～7年,3例转移癌患者1.5年内均死亡;随访到的2例皮质癌患者分别于术后2.0、2.5年死于复发、转移.余131例均健康存活,其中3例口服地塞米松至术后1年.结论 恶性肿瘤、有功能肿瘤、亚临床型肾上腺皮质肿瘤、嗜铬细胞瘤、直径大于3cm的肿瘤应手术治疗;而小于3cm的无功能肿瘤可定期严密随访.     

肾和肾上腺恶性肿瘤合并下腔静脉瘤栓的外科治疗

目的 提高肾和肾上腺恶性肿瘤伴下腔静脉瘤栓的治疗效果.方法 1985年1月至2008年4月收治肾和肾上腺恶性肿瘤合并下腔静脉瘤栓患者29例,均经彩色多普勒超声、CT及MRI检查确诊.瘤栓分型:I型7例、Ⅱ型10例、Ⅲ型8例、Ⅳ型4例.其中肾上腺肿瘤4例,肾肿瘤25例.TNM分期:T2NoMo 23例,T2N1Mo 1例,T2N1M1 1例,T3N.Mo 1例,T3N1M1 2例,T3N2Mo 1例.肿瘤平均直径8.7(4.O～16.0)cm.瘤栓平均长度:I型3.2(2.5～4.O)cm,Ⅱ型5.3(4.5～6.O)cm,Ⅲ型8.2(6.5～9.0)cm,IV型15.1(12.O～18.5)cm.29例均在全麻下行肾或肾上腺肿瘤根治性切除加下腔静脉瘤栓切除术.结果 29例手术均获成功.术后病理报告:肾透明细胞癌18例、肾肉瘤样癌3例、肾乳头腺癌2例、肾细胞癌(未分化型)1例、肾颗粒细胞癌1例、肾上腺皮质癌3例、肾上腺转移性恶性黑色索瘤1例.失访3例,余26例平均随访35(0～62)个月,患者3年生存率58%(15/26),5年生存率42%(11/26).T2、T3患者3年生存率分别为64%(14/22)、25%(1/4),5年生存率分别为45%(10/22)、25%(1/4).I、II、Ⅲ和Ⅳ型瘤栓患者3年生存率分别为4/6、5/8、5/8和1/4,5年生存率分别为3/6、4/8、3/8和1/4.膈肌以下瘤栓患者3、5年生存率分别为64%(14/22)、45%(10/22),膈肌以上瘤栓患者分别为1/4、1/4.肾和肾上腺恶性肿瘤伴下腔静脉瘤栓无转移患者的3、5年生存率为12/18、9/18,有转移患者分别为3/8、2/8.3例术前出现转移患者生存时间分别为6、10、22个月.结论 根治性肿瘤切除和下腔静脉取栓治疗无淋巴和远处转移的肾和肾上腺恶性肿瘤合并下腔静脉瘤栓效果良好;即使存在远处转移,外科手术仍可以提高患者生活质量,延长患者生存期.     

肾上腺恶性肿瘤的诊治（附34例报告）

目的：探讨肾上腺恶性肿瘤的诊断和治疗方法。方法：回顾性分析34例肾上腺恶性肿瘤的临床资料。其中肾上腺皮质癌13例;恶性嗜铬细胞瘤8例;肾上腺转移癌8例;皮质癌并发同侧肾盂癌1例;节神经母细胞瘤1例;恶性纤维组织瘤1例;脂肪肉瘤1例;肾上腺髓外浆细胞瘤1例。全部34例患者中,有临床症状者23例,有内分泌功能改变者22例。结果：所有患者均接受手术治疗,其中29例肿瘤全切,4例肿瘤小部分或包膜残留,1例仅探查取活检。32例获得随访,时间为2个月-5年。死亡11例,死亡患者生存期为5-29个月,平均21个月。结论：对于肾上腺恶性肿瘤患者。手术切除是首选治疗方法,并且术后的随访很重要。肾上腺皮质癌患者总体预后较差,化疗和放疗可作为术后辅助治疗。恶性嗜铬细胞瘤患者预后较好,MIBG和酚苄明可改善患者生存。对于转移性肾上腺肿瘤患者,手术切除孤立的转移灶可明显提高患者的5年生存率。     

肾上腺转移癌37例临床分析

目的 :探讨肾上腺转移癌的临床特征 ,以祈得到及时诊治。方法 :回顾性分析 37例肾上腺转移癌的临床资料 ,2 6例于诊断原发肿瘤之后发现 ,4例于诊断原发肿瘤的同时发现 ,7例于诊断原发肿瘤之前发现。 5例有局部压迫或疼痛症状。 11例为双侧肾上腺转移癌 (2 9.7% )。 4例肾上腺转移癌经手术切除 ,31例采用化疗、放疗或免疫治疗 ;2例未作处理。结果 :16例 (4 3.2 % )为肺癌肾上腺转移 ,7例 (18.9% )为肾癌 ,5例 (13.5 % )为乳癌 ,3例 (8.1% )为胃癌 ,2例 (5 .4 % )为结肠癌 ,肝癌、胆管癌、甲状腺癌、绒毛膜上皮癌各 1例 (均为 2 .7% )。经手术切除者平均生存期为 (17.5± 2 .9)个月 ,比非手术者的平均生存期稍长 (P <0 .0 5 )。结论 :对肾上腺转移癌应争取早诊断 ;对未发现其他器官或淋巴结转移的孤立性肾上腺转移癌 ,应尽可能行积极的外科手术 ;对于原发肿瘤已无法完全切除或已多处转移者 ,应争取行放、化疗或免疫治疗。     

肾上腺偶发瘤的外科治疗选择

目的 探讨肾上腺偶发瘤的手术指征.方法 总结147例肾上腺偶发瘤患者资料,分析偶发瘤的手术指征,比较腹腔镜和开放性手术的适应证.147例患者中,恶性肿瘤19例(12.9%),平均直径(8.5±3.9)cm,≤4 cm者4例(21.1%),其中肾上腺皮质癌15例、恶性嗜铬细胞瘤4例;良性功能性肿瘤29例(19.7%),平均直径(4.1±2.1)cm,其中≥3 cm者22例(75.9%);19例良性嗜铬细胞瘤中24 h尿香草扁桃酸升高2例,5例醛固酮瘤中血钾稍低2例,5例亚临床库欣综合征腺瘤患者血皮质醇升高,均无明显内分泌异常症状;良性无功能性肿瘤99例(67.3%),其中肾上腺皮质腺瘤61例(61.6%).结果 147例均行手术治疗.腹腔镜下手术组102例,开放性手术组45例,2组肿瘤直径分别为(3.4±1.8)cm和(6.7±1.5)cm(P＜0,0001);手术并发症发生率分别为5.9%(6/102)和15.6%(7/45)(P=0.1103).腹腔镜中转开放手术5例(4.9%),主要见于黏连严重的恶性肿瘤或肿瘤直径＞6 cm者.结论 肾上腺偶发瘤常见类型为无功能肾上腺腺瘤、嗜铬细胞瘤、肾上腺皮质癌.可疑恶性、有内分泌功能、直径≥3 cm的肾上腺偶发瘤应积极手术治疗.腹腔镜手术创伤小、恢复快,为手术首选;肿瘤直径＞6 cm、黏连严重、可疑恶性者应以开放性手术为主.     

无功能性肾上腺肿瘤22例临床分析

目的：探讨无功能性肾上腺肿瘤的诊断与治疗方法。方法：回顾性分析22例无功能性肾上腺肿瘤患者的影像学特点、诊断及治疗方法。结果：22例均行肾上腺肿瘤切除或肾上腺切除术。20例经术后病理明确为良性肿瘤,17例获随访7月～14年,无肿瘤复发;2例经病理诊断为皮质腺癌,1例1年内死亡,另1例术后3年死亡。14例术前经影像学检查明确诊断。结论：无功能性肾上腺肿瘤早期诊断困难,CT及MRI检查有助定性诊断。实质性无功能性肾上腺肿瘤应积极手术治疗。     

肾上腺肿瘤自发性破裂出血5例报告

目的 提高对肾上腺肿瘤自发性破裂出血的认识及诊治水平.方法 回顾性分析2004年1月到2009年3月,共收治5例肾上腺肿瘤白发性破裂出血患者的诊治资料,并进行总结.所有患者均以突发患侧剧烈腰痛急诊,行B超、CT检查发现肾上腺肿块较大,直径4.5cm～12cm不等,平均8.5cm,肿瘤性质均有病理明确.结果 5例自发性破裂出血的肾上腺肿瘤中,3例为嗜铬细胞瘤(其中1例失随访),1例肺癌肾上腺转移,1例肾上腺皮质癌.结论 肾上腺肿瘤自发出血较为少见,临床表现以突发性患侧腰痛、胸闷、心悸为主.B超、CT是有效的诊断方法.肾上腺肿瘤白发性破裂出血多见于较大功能性肿瘤和恶性肿瘤破裂出血.应在稳定生命体征的情况下,积极手术.     

1006例肾上腺肿瘤诊治体会

目的　提高肾上腺肿瘤的诊断和治疗水平。　方法　总结 10 0 6例肾上腺肿瘤的诊治经验。　结果　 10 0 6例中有功能肿瘤 845例 ( 84.0 % ) ,其中恶性肿瘤 12例 ( 1.4% ) ;无功能肿瘤 161例 ( 16% ) ,其中恶性肿瘤 11例 ( 6.8% ) ;180例肾上腺偶发瘤中发现嗜铬细胞瘤 19例。本组例行肿瘤切除 996例 ,手术探查 10例 ,手术死亡率为 0 .3 %。　结论　肾上腺肿瘤好发年龄为 3 0～ 5 0岁 ,库兴综合征女性明显多于男性。对肾上腺肿瘤应行内分泌检测 ,定位方法首选B超和CT。功能性肿瘤和体积大的无功能肿瘤应行手术治疗 ,<3cm无功能肾上腺肿瘤可以长期随访     

Adrenal involvement in multiple endocrine neoplasia type 1

Adrenal lesions belong to the spectrum of multiple endocrine neoplasia type 1 (MEN-1) syndrome. However, the prevalence of adrenal involvement, the characteristics, and the clinical management of adrenal lesions have not yet been clearly defined. A total of 66 patients with confirmed MEN1 germline mutations and 1 additional patient with typical manifestations in three organ systems were monitored in a regular screening program that included evaluation of the adrenals (median follow-up 96 months; range 12 to 300 months). Age at the diagnosis of MEN-1 and of adrenal tumors and the clinical characteristics, genotype, treatment, and follow-up of adrenal disease were analyzed. Adrenal lesions were identified in 18 of 67 (26.8%) MEN-1 patients and were diagnosed 5 years later than MEN-1. The median tumor diameter at diagnosis was 3.0 cm (range 1.2-15.0 cm), with most tumors being 3 cm or smaller. Eight patients had bilateral tumors. Ten patients had nonfunctional benign tumors, three had benign adrenal Cushing syndrome, and one patient had a pheochromocytoma. Four patients developed adrenocortical carcinomas (ACCs), three of which were functional. Nine adrenalectomies and one subtotal adrenalectomy were performed in six patients. Three patients with ACC died owing to the tumor. Patients with mutations in exons 2 and 10 developed adrenal tumors significantly more often than patients with other mutations (p <0.01). Adrenal tumors are a common feature of MEN-1 but occur later in the course of the disease. The lesions are often small and nonfunctional and can therefore be managed by close surveillance; others have significant malignant potential and should be considered for surgery when they are 3 cm or larger.     

桥本氏病合并甲状腺恶性肿瘤(附10例报告)

本文报告86例桥本氏病,发现同时合并甲状腺恶性肿瘤10例。男1例,女9例,年龄31～69岁,平均46岁;其中乳头状癌5例,滤泡状癌1例,恶性淋巴瘤3例,肉瘤变1例。术后随访1～5年,存活8例,2例死于其它疾病。作者强调术前甲状腺同位素扫描,主张对桥本氏病合并发结节者应积极手术探查。     

Results of laparoscopic adrenalectomy for large and potentially malignant tumors

ndoscopic adrenalectomy is the procedure of choice for patients with small functioning adrenal tumors. For most surgeons invasive adrenal carcinoma is an absolute contraindication for laparoscopic adrenalectomy (LA). Whether LA should be proposed for large (> 6 cm), potentially malignant tumors is questionable. The aim of this study was to evaluate the risks and outcome of LA performed in our department in patients with tumors > 6 cm and potentially malignant. We performed a retrospective study of 216 patients who underwent 233 LAs in our department from 1994 to 2000. We selected 19 patients with a tumor > 6 cm and potentially malignant: 8 nonfunctional tumors, 4 cortisol-secreting tumors, 1 virilizing tumor, and 6 pheochromocytomas. In none of these patients did preoperative investigations demonstrated invasive carcinoma. The median tumor size was 70 mm. LA was performed by a transperitoneal flank approach. Conversion to open adrenalectomy was performed in two patients owing to intraoperative evidence of invasive carcinoma. The median operating time was 150 minutes (range 95–240 minutes). Capsular disruption occurred during the dissection of two pheochromocytomas. There was no postoperative morbidity. Six patients had an adrenocortical carcinoma on pathologic diagnosis: three of the eight nonfunctional tumors, one of the four cortisol-secreting tumors, and one virilizing tumor. One patient presented with liver metastases 6 months after surgery and died. The five other patients are disease-free with a follow-up ranging from 8 to 83 months. The 13 patients with benign lesions (6 cortical adenomas, 1 ganglioneuroma, 6 pheochromocytomas) are disease-free with a median follow-up of 47 months (range 10–81 months). In experienced hands LA can be proposed for large, potentially malignant tumors. Conversion to open adrenalectomy should be performed if local invasion is observed during surgery. At present the risk of intraabdominal recurrence is unknown.     

原发性膀胱小细胞癌诊治体会

目的 探讨膀胱小细胞癌的临床及病理特点.方法 回顾性分析9例膀胱小细胞癌的基本资料.男6例,女3例.年龄45～79岁,平均62岁.临床表现为肉眼血尿7例,排尿困难及下腹部疼痛2例.肿瘤直径0.5～7.0 cm,平均2.0 cm;多发2例,单发5例,全膀胱弥漫性生长2例.7例行尿脱落细胞学检查,阳性4例.9例均行手术治疗,其中4例术前诊断为浅表性者行TURBt,术后均定期行吡柔比星膀胱灌注,1例化疗3个周期;膀胱部分切除2例,均定期行吡柔比星膀胱灌注,1例化疗2个周期;根治性膀胱全切3例,静脉化疗2例.结果 术后病理检查:肿瘤细胞体积小,呈圆形,胞质稀少,核浓染,缺乏巢状结构.免疫组化染色:嗜铬素A(+)、神经元特异性烯醇化酶(+).诊断为小细胞癌,其中1例含移行细胞癌成分,1例合并前列腺癌.1例术前检查显示高血钙(3.15 mmol/L)和低血磷(0.61 mmol/L),术后1个月血钙及血磷恢复正常.保留膀胱者随访4例,3例分别于术后4、9、25个月死于转移,1例术后化疗者随访24个月未见复发及转移.膀胱全切3例中2例分别于术后2、28个月死亡,1例术后随访32个月未见肿瘤复发及转移.结论膀胱小细胞癌恶性程度高,预后差,根治性膀胱全切加全身化疗是主要的治疗方法,保留膀胱的手术应配合全身化疗.决定预后的是肿瘤的临床分期及治疗方法.

Abstract：

Objective To investigate the clinical and pathological features of small cell carcinoma of the urinary bladder. Methods The pathological and clinical data of 9 cases of small cell carcinoma were analyzed retrospectively. There were 6 males and 3 females, ages 45 to 79 years (mean age, 62 years). Clinical manifestations of 7 cases included gross hematuria and dysuria, the other 2 cases experienced lower abdominal pain. The mean tumor size was 2.0 cm (ranged, 0.5 to 7.0 cm). Two cases had multiple tumors and 5 cases had single tumors. The growth pattern in 2 cases was diffuse growth in the whole bladder. In 4 cases tumor cells were found in urine cytology. All 9 patients underwent surgical treatment, including TURBt. Four patients were diagnosed as superficial tumors before operation. All the patients underwent regular theprubicine irrigation in the bladder. One case underwent additional intravenous chemotherapy for 3 cycles. Partial cystectomy was performed in 2 cases, with regular theprubicine irrigation in bladder and 1 case underwent intravenous chemotherapy for 2 cycles. Radical cystectomy was performed in 3 cases, with 2 cases undergoing intravenous chemotherapy after operation. Results Pathological findings showed that tumor cells were small and round in shape. These hyperchromatic nuclei showed limited cytoplasm with lack of nesting characters. CgA and NSE were positive in immunohistochemistry. The final diagnosis was small cell carcinoma, with 1 case accompanied with transitional cell carcinoma and 1 case accompanied with prostate cancer. One case showed high preoperative serum calcium (3.15 mmol/L) and low serum phosphate (0.61 mmol/L), which returned to normal 1 month after operation. Four cases who′s bladder was preserved were followed up, 3 cases were alive for 4, 9 and 25 months after operation. The 1 case who underwent intravenous chemotherapy was followed up for 24 months and there was no sign of relapse or metastasis. In all the 3 cases with radical cystectomy, 2 cases died 2 and 28 months postoperativly. Another case with adjuvant chemotherapy was followed up for 24 months without recurrence or metastasis. Conclusions Small cell carcinoma of the urinary bladder is highly malignant with poor prognosis. Radical cystectomy in combination with systemic chemotherapy has better efficacy. Retained bladder surgery with systemic chemotherapy is an alternative choice. The most important factors which influence the prognosis of the tumor are clinical stage and therapeutic methods.     

肾上腺偶发瘤的诊治

目的探讨肾上腺偶发瘤的诊断步骤和治疗方法的选择。方珐回顾性分析手术证实的129例肾上腺偶发瘤的临床资料。结果129例中125例完整切除肿瘤，肿瘤直径1．2—18cm。嗜铬细胞瘤31例，皮质腺瘤37例，皮质癌9例，转移癌4例，淋巴瘤3例，其他良性肿瘤45例。结论肾上腺偶发瘤中大部分为皮质和髓质肿瘤，因此需在术前做内分泌检查，确定有无功能；对直径大于3cm的无功能肿瘤及有功能或疑有恶变的需手术治疗；而小于3cm无功能肿瘤可定期随访。     

22例原发性气管肿瘤的诊断与外科治疗

目的总结22例原发性气管肿瘤手术治疗的临床经验,以提高手术疗效。方法22例原发性气管肿瘤患者,其中良性肿瘤4例,恶性肿瘤18例。行气管对端吻合14例,隆凸重建4例,纤维支气管镜下摘除肿瘤1例,局部搔刮2例,气管修补1例。气管切除长度2．0～5．2cm,平均3．8cm。结果22例中确诊前有17例在门诊误诊,误诊率77．3％,大多数患者被误诊为支气管哮喘。术后30d内死亡1例,死于急性呼吸衰竭。发生并发症7例（31．8％）,分别为肺部感染4例,吻合口瘘1例,乳糜胸2例;远期并发症吻合口狭窄3例,经再次手术治疗,狭窄改善。随访20例,随访时间1个月～8年。4例良性肿瘤患者在随访期间均无瘤生存;16例恶性肿瘤患者术后随访满5年以上生存6例,因肿瘤脑、肝和骨远处转移死亡3例。结论手术切除是治疗气管肿瘤最有效的方法,气管节段切除是治疗气管恶性肿瘤的主要术式,良性肿瘤可以考虑保守术式,降低手术并发症是取得良好手术疗效的关键,掌握气管肿瘤的临床特点、提高对该病的认识是减少误诊的有效手段。     

肾上腺髓性脂肪瘤的诊断与治疗

目的：探讨肾上腺髓性脂肪瘤的影像学特点和诊治方法.方法：肾上腺肿瘤患者12例.男5例,女7例.平均年龄48（33～64）岁.左侧4例,右侧8例.血压高2例.腰腹部疼痛1例,无特异性症状9例.行B超、CT及MRI检查.术前提示为肾上腺占位,其中肾上腺髓性脂肪瘤10例.嗜铬细胞瘤1例,肾上腺肿瘤1例.12例均行肾上腺肿瘤切除术,其中开放手术7例.腹腔镜手术5例.结果：12例手术均顺利完成.平均手术时间1.5 h,平均术中出血量50 ml.术后无明显外科并发症,平均7天顺利出院.切除肿瘤最小3.0 cm×3.5 cm×4.0 cm.最大6.5 cm×7.5 cm×8.0 cm.病理诊断均为肾上腺髓件脂肪瘤.术后随访0.5～8年（平均2年）.所有患者临床症状消失,B超及CT检查末见肿瘤复发及恶性变.结论：肾上腺髓件脂肪瘤多无特异性临床表现,术前诊断主要靠影像学检查,确诊依赖于病理检查 手术切除预后良好,其中腹腔镜手术创伤小,疗效满意,为治疗本病的理想方法.