睾丸富于细胞纤维瘤临床病理观察

目的:探讨睾丸富于细胞纤维瘤的临床病理特征、鉴别诊断和预后。方法:对1例睾丸富于细胞纤维瘤的临床表现、组织形态和免疫组化进行综合分析,并结合文献讨论其病理特点及鉴别诊断。结果:30岁男性患者,右侧睾丸不适并无痛性肿大为主诉。睾丸纤维瘤实性边界清楚,包膜亦厚亦薄,切面灰白色,镜下梭型瘤细胞排列成席纹状、鱼骨状或束状,细胞丰富,细胞核无多形性,核分裂像为0～1个/10HP。免疫组化显示肿瘤细胞波形蛋白(+)、S-100局灶(+)、平滑肌蛋白局灶(+)、肌纤维蛋白(-)、α-抑制素(-)、CD34(-)、CD99(-)、Ki-67阳性率不足1%。结论:睾丸富于细胞纤维瘤是一种罕见的睾丸性索间质肿瘤,与卵巢同源性肿瘤病理特征相同;容易与睾丸的其它梭型细胞肿瘤混淆,形态学结合免疫组化可资鉴别;手术切除预后良好。     

单侧大细胞钙化型支持细胞瘤合并弱精子症1例报道并文献复习

大细胞钙化型支持细胞瘤属于睾丸非生殖细胞瘤分类中十分罕见的性索间质肿瘤,目前仍未见合并弱精子症的报道,本文以我院收治1例大细胞钙化型支持细胞瘤合并弱精子症患者临床资料为基础,查阅相关资料对其进行回顾性文献复习并探讨大细胞钙化型支持细胞瘤的临床诊治策略及该病与弱精子症的联系。     

睾丸原发性神经内分泌肿瘤临床病理研究(附7例报告)

目的:观察睾丸原发性神经内分泌肿瘤的组织学、免疫表型,探讨其临床病理特征、诊断、鉴别诊断及治疗预后。方法:运用组织学、免疫组化技术对7例睾丸原发性神经内分泌肿瘤进行光镜观察及免疫标记,并结合相关文献对其临床表现、组织形态和免疫组化特点及治疗和预后等进行综合分析。结果:7例男性患者均以睾丸无痛性肿块就诊,平均40.6岁。均不伴有类癌综合症。组织学上,肿瘤排列呈岛状、梁状,可见实性片状及大的不规则腺管样结构,瘤细胞大小较一致,呈圆形、多角形,胞质略嗜酸,胞界不清。核单一,圆形,染色质细颗粒状,核分裂象计数2个/10HPF。免疫表型,瘤细胞均弥漫性表达Syn、CgA、NSE和CK,Ki-67阳性细胞数2%。结论:原发于睾丸的神经内分泌肿瘤非常罕见,通常不伴有类癌综合症,早期手术切除预后较好。免疫组化染色有助于该肿瘤诊断,鉴别诊断包括睾丸畸胎瘤合并类癌、转移性神经内分泌肿瘤、精原细胞瘤、支持细胞瘤等。     

睾丸支持细胞瘤1例报告并文献复习

目的探讨睾丸支持细胞瘤患者的临床诊治特点及预后。方法回顾性分析1例睾丸支持细胞瘤患者临床资料,并结合文献进行讨论。结果该患者术后病理诊断普通型睾丸支持细胞瘤,未行放、化疗治疗。随访6月无肿瘤复发转移。结论睾丸支持细胞瘤是一种少见的性索间质肿瘤,恶性程度低,预后较好,术后需定期随访。     

睾丸恶性间质细胞瘤1例报告

目的:提高对睾丸恶性间质细胞瘤(TMLCT)临床病理特征的认识及无创诊断水平。方法:回顾性分析1例患者(年龄47岁)TMLCT的临床病理资料,检测患者外周血循环肿瘤细胞(CTC)并进行文献复习。结果:全麻下行根治性右睾丸肿瘤切除术。术后病理示:TMLCT,以间质细胞为主,可疑脉管癌栓。免疫组化染色示:α-抑制素(+),波形蛋白部分(+),EMA(+),PLAP(+),CD30(+),Ki67约5%(+),CK(-),CK7(-),S100(-),CD10(-),SMA(-),Des(-),AFP(-),h CG(-),CEA(-),CK19(-),CD117(-),Oct-4(-),LCA(-),CD20(-),Pax-5(-),CD3(-),CD43(-)。患者外周血中共检测到2个CTCs。术后复查因多发淋巴结转移行化疗3个疗程,随后复查CT见腹膜后淋巴结转移无明显缩小,予行腹膜后肿物切除+冷冻消融术。随访8个月复查CT见腹膜后转移淋巴结较前增大并肾上腺转移。结论:TMLCT是极罕见的性索间质肿瘤,恶性度高,预后差,而CTC有望用于其早期诊断及预后预测。     

原发性肾小细胞神经内分泌癌1例报道并文献复习

目的 探讨肾脏小细胞神经内分泌癌的起源、临床病理特征、诊断、治疗方法,以提高对该病的认识.方法 分析我科收治的1例肾脏小细胞神经内分泌癌患者的临床资料,结合国内外文献对该病进行复习总结.结果 患者无明显症状及阳性体征,因体检发现左肾占位入院.术后标本大体观:左肾上中极见一肿物,大小9 cm×8 cm×8 cm,切面灰白,质软呈实性,肾盂、肾盏结构破坏.镜下观:肿瘤细胞为小圆形或梭形,小梁状、巢状或片状排列,核分裂易见.免疫组化:CD10(-)、CD117(+)、CD56(+)、CD99(+)、CgA(-)、CK(部分+)、CK7(-)、CK8(+)、Ki-67(≥25%)、Syn(+)、Vim(+).结论 肾脏小细胞神经内分泌癌临床罕见,临床表现缺乏特异性,确诊主要依靠组织学特点和免疫组织化学检查,治疗以根治性手术为主,预后较差.     

胰腺实性及囊性肿瘤与无功能性胰岛细胞瘤

胰腺实性及囊性肿瘤(SCT)的临床特点及病理学特征已日趋明了,目前已作为一非胰岛细胞起源的独立肿瘤为广大病理及临床医生所认识。作者对该科室过去诊断为非功能性胰岛细胞瘤的12例手术标本重新进行了病理组织学研究,其中包括HE、PAS、Grimelius染色并似Insulin、Glucagon、Somatostatin以及α_1-antitrypsin抗体进行了免疫组织化学研究。符合以下标准者,诊断为SCT:(1)内眼观,肿瘤为结缔组织包膜包绕,中心因变性坏死而形成囊肿,(2)组织学特征:肿瘤细胞呈乳头状增生并以血管为中心形成玫     

睾丸混合性非精原细胞性生殖细胞癌1例并文献复习

目的:睾丸混合性非精原细胞性生殖细胞癌的文献报道极少,本文旨在探讨睾丸混合性非精原细胞性生殖细胞癌的临床症状、病理特点及诊疗方法。方法:分析1例睾丸混合性非精原细胞性生殖细胞癌的临床资料,并运用组织学、细胞化学和免疫组化技术对该例睾丸混合性非精原细胞性生殖细胞癌进行光镜观察和免疫标记并结合文献就该类肿瘤的临床特征进行探讨。结果:患者以睾丸无痛性肿大3年就诊,病理组织学表现为肿瘤排列结构多样,有乳头状结构、裂隙或腺样结构,细胞大,呈多角形或柱状,核不规则呈泡状,一个或多个核仁,核膜清楚,胞质嗜碱或嗜酸,间质少量淋巴细胞浸润。免疫组化显示:白细胞分化抗原(CD117)(-)、细胞角蛋白(CK8-18)(++)、CD30(++)、CK(+++)、波形纤维蛋白(Vimentin)(-)、胎盘型碱性磷酸酶(PLAP)(±)、抑癌基因产物(P53)(+)、甲胎蛋白(AFP)(+)和上皮膜抗原(EMA)(++)。病理诊断为睾丸畸胎胚胎癌,行手术根治术,按混合性非精原细胞性生殖细胞癌行术后辅助化疗。随访时间l年,健康生存。结论:睾丸混合性非精原细胞性生殖细胞癌是一种罕见的恶性肿瘤,多数临床症状不明显。诊断主要依靠体格检查、B超、CT、血清肿瘤标记物测定等,确诊需要病理学检查,手术切除是其首选的治疗方法。     

9例散发性甲状腺髓样癌临床病理特征分析

目的:探讨散发性甲状腺髓样癌(MTC)临床特点、术中及术后病理特征、治疗及预后。方法:收集9例MTC患者临床资料,重新阅读冷冻切片及石蜡切片,完善免疫组化及特殊染色,分析临床病理特征并结合文献复习。结果:患者年龄45～70岁,中位年龄57岁,女性7例。8例接受冷冻切片病理检查,3例术中诊断为MTC,5例诊断甲状腺癌(乳头状癌或组织学分型不明确)。术后病理示肿瘤最长径0.3～6.9 cm,镜下见淀粉样沉积物将肿瘤组织分隔成实性巢团状或条带状,细胞呈多边形、圆形、高柱状、短梭形,胞浆颗粒感,核分裂象低。肿瘤组织内血管丰富,钙化常见。免疫组化:肿瘤细胞降钙素、CEA、Syn、CgA几乎均阳性。其中有1例为MTC(最大径1.3 cm)和甲状腺乳头状癌(最大径0.6 cm)合并发生。结论:散发性MTC术中冷冻切片较难明确诊断,石蜡切片需要警惕并发甲状腺乳头状癌,当肿瘤内无明显淀粉样蛋白沉积时,掌握组织学其他特点,辅以免疫组化染色利于确诊。     

肾脏神经内分泌癌1例报告并文献复习

目的 通过分析肾脏神经内分泌癌(neuroendocrine carcinoma,NEC)的临床病理特征、免疫表型、治疗方式及预后,提高对该类肿瘤的认识和诊疗水平. 方法 分析我科1例肾脏NEC的临床表现、病理特点及治疗方法并结合文献复习. 结果 患者表现为肉眼血尿伴腰痛.病理:肿瘤细胞中等大小,细胞核呈圆形、卵圆形,染色质为细颗粒状,肿瘤细胞排列呈小梁状、条索状,见菊形团样结构;免疫组化:CK(+)、EMA(+)、CD56(+)、Ki-67(80％)、CgA(-)、Syn(-)、CD99(-)、WT-1(-)、Vimentin(-)、NSE(-). 结论 肾脏NEC十分罕见,且临床表现与肾盂癌较为相似,其诊断主要根据病理学检查和免疫组化结果来做出判断.     

Testicular-sparing surgery for the prepubertal testicular tumor. Experience of two cases with large cell calcifying Sertoli cell tumors.

PURPOSE: We review prepubertal germ cell tumors of testis in our institute and the Japanese registry and present 2 cases with a large cell calcifying Sertoli cell tumor (LCCSCT) and discuss the possibility of testis-sparing surgery. MATERIALS AND METHODS: Incidence, age, pathology and clinical stages of prepubertal germ cell tumors are surveyed for 30 years at our department and 10 years of the malignant tumor registry of the Japanese Society of Pediatric Surgery. Two representative prepubertal boys with LCCSCT are presented. One of them was treated by partial orchiectomy. RESULTS: The majority of testicular germ cell tumors in the prepubertal age were composed of embryonal cell carcinoma/yolk sac tumors or teratoma, occurred in preschool age, were limited to clinical stage I and did not metastasize irrespective of histology. Benign behavior which included recovery from hormonal derangement, no tumor recurrence and negative antisperm antigen was observed in 2 cases with LCCSCT who underwent either radical orchiectomy or partial orchiectomy. CONCLUSION: Partial orchiectomy should be considered as a standard option in prepubertal schoolboys with a testicular mass if surgically feasible. This surgical treatment is safe and preserves fertility and is psychologically advantageous. It is not recommended for yolk sac tumors that may recur, however they are rare in prepubertal boys and can be differentiated preoperatively by prudent evaluation.     

Organ-sparing surgery of the bilateral testicular large cell calcifying sertoli cell tumor in patient with atypical Peutz–Jeghers syndrome

Large cell calcifying sertoli cell tumor (LCCSCT) is an exceptionally rare neoplasm originating from sperm cord cells. The tumors have relatively low malignant potential and unlikely proceed to metastasis formation. The lesions may occur in an isolated form or in ca. 40% of cases may be associated with genetic abnormalities, by and large Peutz-Jeghers syndrome and Carney complex. At presentation, 20% of LCCSCT cases are bilateral and/or multifocal. Owning to characteristic skin lesions and particular hyperechoic ultrasound image of the tumor, preliminary diagnosis of the syndromic LCCSCT is possible in the preoperative period. Consequently, testicle organ-sparing procedure can be attempted, which is especially justified in bilateral lesions. Here, we report a case of a bilateral LCCSCT in a 20-year-old man with atypical Peutz-Jeghers syndrome due to amplification of the exon 1 of STK11 gene who was successfully treated with bilateral testicle-sparing tumorectomies.     

Sertoli cell proliferations of the infantile testis: an intratubular form of Sertoli cell tumor?

We report on six boys with intratubular Sertoli cell proliferations (ISCPs), studied by routine histologic methods, electron microscopy, and immunohistochemistry of anti-müllerian hormone (AMH), inhibin alpha-subunit, 3beta-hydroxysteroid dehydrogenase (3beta-HSD), proliferative cellular nuclear antigen, and p53, and carefully followed for extended periods with periodic clinical examinations, testicular ultrasonographies, and determinations of serum levels of AMH and inhibin B. Peutz-Jeghers syndrome was found in four of six patients, and gynecomastia occurred in five of six patients. One boy had isosexual pseudoprecocity. ISCPs were observed as multiple foci of seminiferous tubules with large and proliferated Sertoli cells replacing germ cells and limited by the basement membrane. Mitotic figures, atypia, and/or interstitial invasion were not observed. Bilateral ISCPs were the only pathologic finding in three patients (patient nos. 1-3) and were associated with a microscopic tumor that resembled a large-cell calcifying Sertoli cell tumor (LCCSCT) in a fourth patient (patient no. 4). In the two remaining patients (patient nos. 5 and 6) ISCPs and LCCSCT were found in both testes. Ultrastructural examination showed large Sertoli cells, with round nuclei, sparse organelles, and some glycogen. Inhibin alpha-subunit immunolocalization was positive in the five patients in whom it was determined (patient nos. 2-6), AMH was positive in those ISCPs associated with tumors (patient nos. 4-6) and negative in isolated ISCPs (patient nos. 2 and 3); 3beta-HSD and PCNA were variable, and p53 was negative in all ISCPs. Patient nos. 1-4 have been followed for 2-19 years. One of them is currently entering puberty, the other two have already completed puberty and have testes of normal size, and the remaining one is an adult with clinically normal testes and sperm production. None of these patients had evidence of tumor development during follow-up as shown by serial ultrasonographies and serum levels of AMH and inhibin B. Patient nos. 5 and 6 who had bilateral ISCPs and LCCSCT were orchidectomized and evolved for 2-10 years after surgery without tumor recurrence. The prognostic significance of ISCPs, particularly when they are the only pathologic finding in a testicular biopsy, is a matter of controversy. Based on the long normal evolution, we recommend a conservative approach to therapy. The bilateral and multicentric character of ISCPs and their association with Sertoli tumors and Peutz-Jeghers syndrome suggest that they represent either proliferative lesions with tumorigenic potential or the intraepithelial stage in the evolution of some testicular Sertoli cell tumors.     

Conservative surgical therapy for leydig cell tumor

PURPOSE: We performed a long-term evaluation of conservative surgical treatment of benign Leydig cell tumor. MATERIALS AND METHODS: A multicenter retrospective clinical study was performed at 6 European centers. Case files of all patients diagnosed with Leydig cell tumor and treated with conservative surgery were examined. Patients underwent physical examination, hormone and tumor marker assays, scrotal and abdominal ultrasound, chest x-ray, and an endocrinological examination. RESULTS: From 1987 to 2006, 22 patients with Leydig cell tumor underwent conservative surgery. Mean patient age was 35 years (range 5 to 61). Mean followup was 47 months (range 1 to 230). No local recurrence or metastasis was observed. Patients presented with a palpable testicular nodule (3 patients, 13.7%) or a nodule diagnosed by ultrasound (15 patients, 68.2%), gynecomastia (2 patients, 9.1%), precocious pseudopuberty (1 patient, 4.5%) or scrotal pain (1 patient, 4.5%). Three patients were monorchid after contralateral orchiectomy for inguinal hernia repair (1 patient, 28 years before surgery) and nonseminomatous germ cell tumor (2 patients, 1 month and 6 years before surgery). Diagnosis after frozen section examination was Leydig cell tumor in 20 of 22 cases (91.0%). Mean histological size of the nodule was 1.11 cm (range 0.5 to 2.5). Preoperative FSH and LH levels were high in 4 patients. Tumor markers were normal before and after surgery. Followup was conducted for all patients every 3 to 6 months with physical examination, tumor markers, scrotal and abdominal ultrasound, chest x-ray. Six patients (27.3%) underwent abdominal computerized tomography. CONCLUSIONS: When diagnosed early Leydig cell tumors present a favorable followup. In select cases with motivated patients, conservative surgery proved to be a feasible and safe choice.     

Renal cell carcinoma with non-islet cell tumor hypoglycemia

We report a case of an elderly gentleman with renal cell carcinoma presenting with the rare entity of non-islet cell tumor hypoglycemia (NICTH). Non-islet cell tumor hypoglycemia syndrome is caused by the tumor producing insulin-like growth factor II, causing hypoglycemia. The syndrome is most commonly associated with very large fibromas or fibrosarcomas.     

Progression of prostate cancer to neuroendocrine cell tumor

BACKGROUND: The progression to endocrine therapy-resistant prostate cancer is partly due to clonal change to neuroendocrine cell tumor. To elucidate this pathologic process, the clinical courses of four cases of neuroendocrine cell tumor that were found at autopsy are reported. METHODS: Between 1995 and 1999, autopsies were performed for 20 cases of prostate cancer. Lesions predominantly composed of a neuroendocrine cell tumor (small cell carcinoma) were found in four men. The clinical courses of these cases were compared to 16 other non-neuroendocrine cell tumors (adenocarcinomas). RESULTS: The outstanding features of the neuroendocrine cell tumors were: (i) survival was brief after relapse, although the duration of control by employing endocrine therapy varied; (ii) the prostate-specific antigen level did not increase after relapse; and (iii) the sites of metastasis were similar to those of common adenocarcinomas. CONCLUSION: The progression to a neuroendocrine cell tumor indicated a poor prognosis and slight (if any) changes in the serum prostate-specific antigen level. This tumor might not appear to respond to any therapeutic attempt.     

Malignant islet cell tumor projecting into the main pancreatic duct

We report herein a rare case of islet cell tumor showing a unique growth pattern in a patient who developed repeated acute pancreatitis as the tumor's initial symptom. Preoperative imaging examinations showed dilatation of the main pancreatic duct (MPD) and cysts around the pancreatic tail. A distal pancreatectomy with splenectomy was performed because the pancreatitis was localized in the distal pancreas and was not controlled by various drug therapies. Grossly, the tumor consisted of two component parts: a markedly infiltrative part in the pancreatic parenchyma, and a papillary elevated part in the MPD. The MPD was obstructed by the tumor spreading widely along the distal MPD. Microscopically, the tumor was composed entirely of islet cell tumors (nonfunctioning), with several foci of venous and lymphatic involvement. Based on its growth behavior, we assumed that the tumor may have arisen from the MPD or from islet cells closely adjacent to the MPD. The patient's postoperative course was uneventful and he is doing well 2 years after the operation. We discuss the growth pattern of the tumor and the cause of the pancreatitis. Received: March 30, 2000 / Accepted: July 12, 2000     

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.     

一期经口前路肿瘤切除和前后路椎体重建治疗枢椎骨巨细胞瘤

目的：探讨枢椎肿瘤切除和椎体重建的新方法.方法：采用经口咽入路枢椎体肿瘤切除,改良异形钛笼重建椎体,同期后路寰椎椎弓根和第三颈椎侧块钉棒固定植骨融合治疗枢椎体骨巨细胞瘤,并辅以放疗.结果：术后患者临床症状立即消失,行走正常,影像学示肿瘤切除干净,脊椎重建固定稳妥.结论：一期经口前路联合后路行枢椎肿瘤切除椎体重建是治疗枢椎肿瘤的可行方法,但远期疗效有待观察.     

Granular cell tumor of the urethra

A 38-year-old woman was referred for urethral tumor treatment. Needle biopsy of the tumor was performed. Histologically, the tumor was composed of large polygonal cells with eosinophilic and periodic acid-Schiff (PAS)-positive granular cytoplasm, indistinct cell margins and small round central nuclei. Immunohistochemical staining for S100 protein, vimentin, and neuron-specific enolase (NSE) was positive, indicating that it was a granular cell tumor. Excision of the tumor including the urethra and appendicovesicostomy using the Mitrofanoff principle were performed. There has been no tumor recurrence in the 8 months after the operation.