Enucleation for prepubertal leydig cell tumor

PURPOSE: Leydig cell tumors in children are rare, comprising only 4% to 9% of all primary testis tumors in prepubertal males. Almost all of these boys present with isosexual precocious pseudopuberty associated with increased testosterone, low gonadotropin levels and a testis mass. We present our experience with testis sparing enucleation of Leydig cell tumor in prepubertal boys. MATERIALS AND METHODS: Two patients presented with isosexual precocious puberty at ages 6 and 9 years. Each patient had a well circumscribed, painless testicular mass, increased serum testosterone (101 and 444 ng/dl [normal 0 to 25]), normal gonadotropins and negative alpha-fetoprotein levels. Both patients underwent successful enucleation of the testis mass following proper testis oncological surgical principles. RESULTS: Both patients had normalization of the serum testosterone following enucleation of the Leydig cell tumor. At 9 and 44 months of followup they have maintained normal ipsilateral testicular volume compared to the contralateral gonad, and 1 patient entered puberty spontaneously at 1 year postoperatively. Neither patient suffered any morbidity, and both have presumably benefited from preservation of the involved gonad with preserved testicular volume. CONCLUSIONS: Prepubertal boys with isosexual precocious pseudopuberty, an isolated testis mass, increased testosterone and low or normal gonadotropin levels can reliably be diagnosed with Leydig cell tumors. Based on the ability to establish the diagnosis preoperatively and the universal benign behavior of unilateral, prepubertal Leydig cell tumor, we believe these patients are best treated with testis sparing enucleation of the tumor. In view of the high likelihood that this tumor in prepubertal boys is benign, a transscrotal surgical approach should be considered.     

睾丸间质细胞瘤2例报告并文献复习

目的 探讨睾丸间质细胞瘤的临床病理特点,进而提高睾丸间质细胞瘤的诊治水平。方法 回顾性分析2例睾丸间质细胞瘤的临床病理资料并复习相关文献。结果 1例术中快速冷冻切片病理诊断为睾丸间质细胞瘤,行根治性睾丸切除术,1例直接行根治性睾丸切除术,术后病理均诊断为睾丸间质细胞瘤,其中1例精索残端及周围脂肪组织可见肿瘤细胞,精索静脉可见肿瘤浸润。术后分别随访24和6个月,未见复发或转移。结论 睾丸间质细胞瘤临床罕见,术前诊断较为困难,确诊仍依赖组织病理学检查,外科手术是睾丸间质细胞瘤唯一有效的治疗方法。     

双侧睾丸间质细胞瘤1例报告并文献复习

目的 探讨睾丸间质细胞瘤的诊断和治疗经验.方法 报告1例双侧睾丸间质细胞瘤患者的临床资料并文献复习.结果 根据患者临床表现及相关检查,术前诊断考虑为双侧睾丸间质细胞瘤,术中快速冰冻病理检查示双侧睾丸间质细胞瘤,行睾丸肿瘤剜除术.术后随访6个月未见肿瘤复发或转移.结论 睾丸间质细胞瘤是一种较为罕见的睾丸肿瘤.术前诊断较为困难,睾酮、雌二醇等性激素水平升高及男性乳房发育等症状对睾丸间质细胞瘤的诊断有重要意义,确诊仍需依赖病理组织学检查.传统的手术方法采用根治性睾丸切除术,然而对于青春期前患者、双侧睾丸间质细胞瘤等患者,可以施行保留睾丸的肿瘤剜除术.     

功能性胰岛细胞瘤的手术切除分析

目的探讨如何提高功能性胰岛细胞瘤的手术切除率,减少肿瘤遗漏,增加治疗效果。方法回顾分析2003-2005年功能性胰岛细胞瘤4例的临床诊治过程。结果功能性胰岛细胞瘤4例,1例影像学检查未能明确定位者,行保守治疗;2例分别行胰头部肿瘤及胰尾肿瘤切除术,术后血糖恢复正常;1例行胰体尾加脾切除术,术后2个月复查血糖为1.53mmol/L并有低血糖临床症状。结论DSA的检查,对进一步明确细肿瘤的位置有肯定的作用;术前影像学定位检查资料的综合分析,可提高肿瘤的手术切除率。     

睾丸间质细胞瘤二例报告并文献复习

目的探讨睾丸间质细胞瘤的临床病理特点及鉴别诊断方法。方法总结2例睾丸间质细胞瘤患者的临床病理资料并复习文献。2例患者年龄均为27岁。例1因左侧睾丸胀痛不适4个月余,例2因婚后3年不育、查体发现右侧睾丸占位1个月入院。B超分别显示睾丸1.0 cm×1.3 cm、0.8 cm×0.6 cm强回声光团,边界锐利。2例均行睾丸肿瘤切除术。结果术中快速冰冻病理诊断为睾丸间质细胞瘤。病理组织学表现为瘤细胞呈团、条索或弥漫分布,体积较大,呈多角形,胞质丰富嗜酸性,边界清楚。免疫组化:波形蛋白,钙结合蛋白和抑制素均阳性。2例术后病理诊断均为睾丸间质细胞瘤。2例术后分别随访17、9个月未见复发。结论睾丸间质细胞瘤发病率低,临床易误诊,确诊需依赖病理组织学检查,尤其对年轻未育者,术中快速冰冻检查有助于手术范围的选择。     

Elevated serum estradiol suggesting recurrence of Leydig cell tumor nine years after radical orchiectomy

We report a case of a Leydig cell tumor that showed retroperitoneal lymph node metastasis 9 years after radical orchiectomy. Elevated serum estradiol (E2) suggested recurrence of the Leydig cell tumor. Retroperitoneal lymph node dissection (RPLND) was performed and the lymph node was proved histopathologically to have a metastatic Leydig cell tumor. After RPLND, serum E2 returned to the normal range. This is the first reported case in which changes in the endocrinological findings were useful as likely tumor markers to detect the recurrence of a Leydig cell tumor.     

Long-term followup and clinical characteristics of testicular Leydig cell tumor: experience with 24 cases

PURPOSE: The natural history of Leydig cell testicular tumors is not well known, and differentiation between malignant and benign forms is not easy. We performed a retrospective multicenter evaluation of clinical and histological characteristics as well as followup of Leydig cell tumors. MATERIALS AND METHODS: From 1990 to August 2004 surgery was performed on 24 Leydig cell tumors. Before surgery all patients underwent clinical examination, tumor markers (alpha-fetoprotein, beta-human chorionic gonadotropin, lactate dehydrogenase), scrotal ultrasound and chest x-ray. Surgery was performed under ultrasound or palpation guidance via inguinotomy with clamping of the spermatic cord. Lesions less than 3 cm in diameter were resected and sent for frozen section examination. Histological criteria were considered. Abdominal computerized tomography or ultrasound and chest x-ray were performed postoperatively every 3 months for the first 2 years and every 6 months thereafter. RESULTS: Patient age range was 22 to 61 years (mean 37.75). In 7 patients (29.2%) the lesion was palpable and incidental diagnosis was made in 10 patients (41.7%). In the other patients diagnosis was made by ultrasound performed for testicular pain (4 patients, 16.6%) or the appearance of gynecomastia (3 patients, 12.5%). Frozen section examination was done in 20 of 24 patients (83%). In 17 of 20 patients (85%) Leydig cell tumor was diagnosed on frozen section examination. All patients underwent radical orchiectomy. All definitive diagnoses interpreted the neoplasia as benign. Average followup was 117 months (range 11 to 241). There was no disease recurrence or progression and all patients are currently disease-free. CONCLUSIONS: In our case study all Leydig cell tumors were interpreted as benign and long-term followup was negative.     

骨巨细胞瘤临床分级与手术方法

目的探讨骨巨细胞瘤临床、病理、X线分级和各种术式的选择与疗效。方法对107例经手术病理证实的骨巨细胞瘤患者,根据临床、病理、X线分级程度采用刮除植骨、骨水泥填充、大块切除、人工关节置换等不同手术方式进行治疗。通过随访观察疗效。结果随访6～70个月（平均24个月）。刮除植骨14例,复发2例;刮除骨水泥填充术42例,复发4例;肿瘤骨大块切除,带血管腓骨髂骨重建25例,复发1例;瘤段切除26例,均无复发。结论骨巨细胞瘤的临床症状、病例、X线三结合综合分期,对确定其属性和程度,正确估计预后和选择适当的手术方案有重要的临床意义。     

Epididymal leiomyoma associated with leydig cell hyperplasia: A case report and immunohistochemical study

Leiomyomas are rare soft tissue neoplasms thatcan affect virtually any part of themale genital tract. Although they have beendescribed in association with other lesions, tobest our knowledge coexistence with leydig cellhyperplasia has not been reported before.We present here the clinical, histopathologicand immunhistochemical characteristics of anepididymal leiomyoma associated with leydigcell hyperplasia and an epididymal cyst in asixty-eight year old man.     

股骨近端巨细胞瘤的诊断和手术治疗

[目的]探讨股骨近端巨细胞瘤患者的影像学表现特点和病灶刮除同种异体植骨内固定手术的疗效.[方法] 2000～2010年本科共收治并随访股骨近端巨细胞瘤患者11例.男7例,女4例;年龄17 ～44岁,平均28.9岁.Campanacci's分级:Ⅰ级5例,Ⅱ级5例,Ⅲ级1例(合并病理性骨折).Campanacci's分级为Ⅲ级的患者行肿瘤广泛切除人工髋关节置换术,其他患者均行肿瘤病灶刮除同种异体松质骨移植锁定钢板内固定手术.[结果]本组病例术后髋关节疼痛均完全消退,所有患者术后均经病理证实为巨细胞瘤.术后随访时间24 ～ 72个月,平均42个月.随访期间1例Campanacci'sⅡ级的患者在术后24个月时肿瘤复发,未出现其他术后并发症.[结论]股骨近端巨细胞瘤影像学表现往往缺少典型的偏心性和膨胀性改变,穿刺活检成功率也不高,诊断具有一定难度.病灶刮除同种异体松质骨移植锁定钢板内固定手术是治疗股骨近端巨细胞瘤安全有效的术式.术中用高频电刀反复烧灼残腔骨壁有助于消灭残留的肿瘤细胞,而移植的松质骨愈合后可提供良好的远期功能学预后.     

Leydig cell tumor of the spermatic cord in an adolescent affected by congenital adrenal hyperplasia

We report the first case of a patient with extratesticular Leydig cell tumor associated with congenital adrenal hyperplasia. An 18‐year‐old congenital adrenal hyperplasia patient presented with a palpable and asymptomatic right extratesticular mass. Color Doppler sonography confirmed the presence of a capsulated and vascularised lesion. Sieric tumor markers were negative. The patient underwent surgical scrotal exploration through an inguinal right incision. The mass, 18 mm in size and located within the spermatic cord, was removed and final pathology diagnosed a benign Leydig cell tumor.     

无功能性胰腺内分泌肿瘤外科诊治的研究进展

无功能性胰腺内分泌肿瘤在临床上较少见，其虽有恶变倾向，但与胰腺癌相比预后较好，故该病的明确诊断及规范的外科治疗将提高本病治愈率及患者生存率。现主要对无功能性胰腺内分泌肿瘤的诊断及外科治疗方法的研究进展做一综述。     

骶骨骨巨细胞瘤的外科治疗

目的 探讨骶骨骨巨细胞瘤外科切除边界与局部复发率及骶神经保留节段与神经功能的关系.方法 对1996年8月至2008年8月行肿瘤切除的48例骶骨骨巨细胞瘤患者进行回顾性分析,男20例,女28例;年龄19～74岁,平均34.7岁.肿瘤累及S1～S5 4例,S1～S4 7例,S1～S3 15例,S1,2 12例,S2～S5 8例,S3～S5 2例.采用单纯后路29例,前后联合入路19例.整块切除2例,边缘切除12例,边缘切除加刮除25例,刮除9例.术后定期随访,观察局部复发情况与骶神经功能.结果 41例随访18～115个月,平均43.5个月.术中出血量550～12000 ml,平均3560 ml.1例发生良性肺转移,2例发生肉瘤变后死亡.15例肿瘤原位复发,其中整块切除复发率0(0/2)、边缘切除18.2%(2/11)、边缘切除加刮除40.9%(9/22)、单纯刮除66.7%(4/6).边缘切除局部复发率低于单纯刮除,差异有统计学意义.保留双侧S3神经根者大小便功能障碍发生率7.4%(2/27),保留单侧S3神经根者33.3%(4/12),差异有统计学意义.结论 外科切除边界与骶骨骨巨细胞瘤局部复发率相关,在注意保留骶神经根的前提下应以边缘切除为目标;保留双侧S3神经根可使绝大部分患者的括约肌功能得以恢复.

Abstract：

Objective To discuss the relations between optimal surgical margin and local recurrence and the impact of preserving segment of sacral nerve root on neural functions based on the clinical and pathological features of giant cell tumor(GCT).Methods From August 1996 to August 2008,48 patients with sacral GCT undergoing tumor resection were respectively analyzed,including 20 males and 28 females with an average of 34.7 years(range,19-74).The tumors were located in S1-S5 in 4 patients,S1-S4 in 7,S1-S3 in 15,S1,2 in 12,S2-S5 in 8,and S3-S5 in 2.Surgical methods included single posterior approach in 29 cases,combined anterior-posterior approach in 19.The surgical margins adopted were en-bloc in 2 patients,marginal in 15,marginal and curettage in 25,and curettage in 9.Results Forty-one of 48 cases were successfully followed up,the average time was 43.5 months(range,18-115).The average blood loss during surgery was 3560 ml(range,550-12 000).Benign lung metastasis occurred in one case 6 years after operation,2 patients died of malignant transformation.Local recurrence occurred in 15 cases.The recurrence rates in patients with en-bloc resection,marginal resection,marginal resection combined with curettage,and curettage were 0,18.2%,40.9%,66.7%,respectively.The recurrence rate of marginal group was significantly lower than that of the curettage group.Of 27 cases with bilateral S3 nerve root preservation,2 sufiered from urine or fetal dysfunction.with an incidence rate of 7.4%.While 4 of 12 patients with unilateral S3 nerve root preservation suffered from sphincter disturbance,with an incidence rate of 33.3%.The significant difference between groups in nerve root preservation was confirmed.Conclusion Optimal surgical margin for sacral GCT is of great importance to local control of tumor recurrence,the surgical procedure of sacral GCT should aim at the marginal resection on the basis of rational sacral nerve roots preservation;preservation of bilateral S3 nerve roots contributes to the recovery of sphincteral function in most patients.     

Leydig cell tumor in grey zone: A case report

IntroductionLeydig cell tumor constitutes only about 1–3% of testicular neoplasms. There is apparently increased incidence in the last few years; one possible explanation for this phenomenon is the widespread use of ultrasound technology and the subsequent increased early detection of smaller lesions that have not been found in historical series.Case presentationWe report a case of Leydig cell tumor of testis in a patient presenting with painless long standing slowly growing left scrotal mass who found to have intrapulmonary nodule and multiple enlarged retroperitoneal lymph nodes on staging work up. The mass was managed by radical orchiectomy. Pathological diagnosis was Leydig cell tumor.DiscussionOrchiectomy is the accepted mode of treatment but follow-up every 3–6 months with physical examination, hormone assays, scrotal and abdominal ultrasonography, chest radiography, and CT scans is essential in such a case with a potential for malignant behavior.ConclusionInguinal orchiectomy is the therapeutic decision of choice and long-term follow-up is necessary to exclude recurrence or metastasis. Cases which fall in the grey zone like ours need to be followed up carefully for metastasis instead of rushing into an early retroperitoneal lymph node dissection, with its potential risks and complications.     

罕见6岁女童卵巢Leydig细胞瘤:临床特点、免疫组织化学和电镜研究

目的对1例罕见6岁女童卵巢Leydig细胞瘤患者的临床特点、肿瘤组织的免疫组织化学和电镜结果、治疗及随访情况进行总结分析,提高对此疾病的认识。方法收集患者的临床资料、生化检查、免疫组织化学和电镜结果。结果 (1)雄激素增多引起男性化表现,外院误诊为"先天性肾上腺皮质增生症",予氢化可的松治疗6个月,睾酮水平未降低。(2)生化检查:血FSH 1.3 U/L,LH 1.1 U/L,雌二醇(E2)246pmol/L(N55pmol/L),孕酮(P)22.1nmol/L(N 1.3~5.7nmol/L),睾酮(T)16.1nmol/L(N 0.3~1.6nmol/L),硫酸脱氢表雄酮(DHEAS)2.34μmol/L(N2.46μmol/L),17-羟孕酮(17-OHP)169.5nmol/L(N9nmol/L)。(3)影像学:右附件区可见约4cm囊实性包块。骨龄9~10岁。(4)病理和免疫组织化学:a-抑制素(inhibin)(+),Calretinin(+),符合Leydig细胞瘤。(5)电镜结果:可见同心圆样异常形态线粒体。(6)随诊:术后T和P下降到正常水平,痤疮消失。结论 P和17-OHP水平升高并非先天性肾上腺皮质增生症的特异性改变,卵巢Leydig细胞瘤也可分泌性激素的中间产物。同心圆样异常形态线粒体可能是Leydig细胞瘤的特异性电镜下改变。本例国内年龄最小的卵巢Leydig细胞瘤,拓宽了我们对此疾病发病年龄的认识。     

Ca2+和CaN凋亡信号通路参与皮质酮诱导的Leydig细胞凋亡中FasL的表达

目的观察在皮质酮诱导的大鼠Leydig细胞凋亡中,Ca2 和钙调神经磷酸酶(CaN)依赖的信号通路是否参与FasL表达的调控。方法利用钙定性探针Fluo-3/AM检测皮质酮作用下的Leydig细胞中Ca2 浓度变化。通过酶底物法测定CaN活性。以Westernblot检测FasL表达。用Annexin-Ⅴ-FITC和PI双标评价Leydig细胞凋亡率。结果经超生理剂量皮质酮处理的Leydig细胞中出现Ca2 浓度升高,CaN活性增加及FasL表达增加。环孢菌素A可抑制CaN活性,使FasL表达下调,细胞凋亡率下降。结论Ca2 和CaN依赖的信号通路参与了皮质酮诱导的大鼠Leydig细胞凋亡;CaN介导了由Ca2 引发的FasL表达,Ca2 和CaN在大鼠Leydig细胞凋亡过程中起重要作用。     

循环肿瘤细胞检测在转移性肾细胞癌中的应用

目的:探讨循环肿瘤细胞(circulating tumor cells,CTC)检测在转移性肾细胞癌临床诊断中的应用价值。方法:2013年3月~2015年5月收治的肾癌患者52例,所有患者在治疗前取外周静脉血标本7.5ml,用细胞搜索系统(cell search system,CSS)对患者外周血进行CTC定量检测,其中男41例,女11例。结果:CTC检测总体阳性率为48.1%(25/52),存在淋巴结转移及远处转移的患者,其CTC阳性率明显高于无淋巴转移及远处转移的患者,中晚期患者(Ⅲ期+Ⅳ期)其阳性率明显高于早期患者(Ⅰ期+Ⅱ期)。结论:CTC检测结果和传统上用来评价肾癌患者的预后的指标其结果一致,CTC的检测可以用来评估肾癌患者的预后,为肾癌患者术后是否应早期进行干预提供依据。