前列腺副神经节瘤(附二例报告)

目的 总结前列腺副神经节瘤的临床、病理特征及诊治方法。方法 报告前列腺副神经节瘤2例，肿瘤组织均行神经元特异性烯醇化酶(NSE)、嗜铬A和PSA免疫组化染色。结合文献复习，总结诊断和治疗情况。结果 2例前列腺副神经节瘤均为无功能性。病理检查符合副节瘤组织学特征，NSE和嗜铬A阳性，PSA阴性。1例发生局部转移，行销内动脉栓塞加化疗术，肿瘤萎缩，现病情稳定，另1例伴发良性前列腺增生，行经尿道前列腺电切术，疗效满意。结论 前列腺副神经节瘤是极罕见的前列腺肿瘤，其临床和病理特征具有特殊性治疗宜采用手术切除肿瘤。     

前列腺副神经节瘤1例报道并文献复习

目的:探讨前列腺副神经节瘤的临床表现、病理特征、治疗和预后。方法:报道前列腺副神经节瘤1例,患者男,39岁,主因"反复血精1年余"入院,误诊为前列腺癌后,行耻骨上前列腺根治性切除术,完整切除肿瘤。结果:术后病理确诊为前列腺副神经节瘤,本例前列腺副神经节瘤为无功能性,免疫组化:NSE(+)、CGA(+)、S100(+)、CK(-)、Desmin(-)。术后血压平稳,2周后拔尿管出院。随访48个月至今未复发。结论:前列腺副神经节瘤缺乏特异的临床特征表现,易误诊,只有靠切除术后病理和免疫组化才能确诊。由于其非常罕见,相关治疗缺乏成熟的经验,故需要进一步研究。     

膀胱非功能性副节瘤一例报告并文献复习

目的提高对膀胱副神经节瘤的临床病理特征、诊断及治疗的认识。方法回顾性分析我科收治的1例膀胱非功能性副节瘤患者的临床资料,结合文献复习并讨论膀胱副节瘤的诊断和治疗特点。结果膀胱副神经节瘤好发于年轻女性,常表现为血尿,免疫组化示CgA、NSE和S-100蛋白阳性,CK阴性。CT及MRI在鉴别诊断上意义不大。经尿道肿瘤切除及膀胱部分切除术治愈该患者,术后密切随访3月无复发。结论膀胱副神经节瘤目前治疗上以膀胱部分切除为主。诊断需结合临床表现、病理及免疫组化结果。因其为潜在恶性肿瘤,术后应长期随访。     

伴空肠转移恶性嗜铬细胞瘤1例及文献复习——免疫组织化学及电镜观察

目的:探讨伴空肠转移恶性嗜铬细胞瘤的临床病理特点,提高鉴别诊断良恶性嗜铬细胞瘤的水平.方法:观察1例伴空肠转移恶性嗜铬细胞瘤的临床病理,结合免疫组化染色及电镜结果,并复习相关文献.结果:患者于行右肾上腺肿瘤切除术半年后发现空肠、肝脏及左锁骨上淋巴结转移,初诊9个月后患者死亡.肉眼观:肾上腺肿瘤大小7 cm×5 cm×3 cm,空肠黏膜下肿块大小9 cm×6 cm×6 cm.镜检:瘤组织呈弥漫性、实性、巢状结构,血窦丰富,核分裂计数＞3个/10 HPF,伴有融合性坏死,见肿瘤侵犯血管、包膜及肾上腺皮质,空肠肠系膜淋巴结见转移.免疫组化均表达CgA、Syn及NSE,S-100散在阳性.超微结构见高电子密度的神经内分泌颗粒.结论:肾上腺恶性嗜铬细胞瘤转移至空肠极罕见,对恶性嗜铬细胞瘤的诊断应结合临床、手术及病理.     

膀胱副神经节瘤5例报告及文献复习

目的:探讨膀胱副神经节瘤的疾病特点及诊疗方法。方法:回顾性分析2012年3月～2019年1月在我院诊治的5例术后病理诊断为膀胱副神经节瘤患者的临床资料和随访结果。结果:5例患者均顺利完成手术治疗,术后病理组织表现为免疫组化不同程度的表达[嗜铬粒蛋白A(CgA)、突触素(Syn)、CD56等染色标记为阳性]。术后随访6～40个月,平均26.5个月,术前有血尿、头痛、心悸及排尿后血压升高等症状的患者于术后症状消失或逐渐缓解,5例患者术后均无复发或转移。结论:膀胱副神经节瘤在临床上非常罕见,且易误诊漏诊,需根据术后免疫组化进行明确诊断。其可分为功能性和非功能性,主要依据临床特征及内分泌检查进行鉴别。术前应进行定性和定位的辅助检查,目前膀胱副神经节瘤的治疗方案多样,手术治疗仍是首选方案,治疗后应长期规律随访。     

胰腺实性假乳头状瘤的病理特点及诊治体会

目的总结胰腺实性假乳头状瘤临床病理特点及诊治经验。方法回顾性分析1996年6月至2005年9月收治的13例胰腺实性假乳头状瘤患者的临床病理资料。结果所有患者均为女性,平均年龄29.2岁。上腹疼痛10例,腹部肿块9例。影像学检查均能发现腹部肿块但未能明确诊断。肿块平均直径10.3cm,包膜完整者9例,包膜不完整4例,质地呈实性5例,呈囊实性8例。术中快速冰冻病理6例,3例获得确诊。病理特征为肿瘤细胞围绕纤细血管轴心形成特征性的假乳头状结构,细胞形态一致,异型不明显。2例证实有胰腺和血管浸润。8例免疫组化结果均为波形蛋白(Vimentin)、神经元特异性烯醇化酶(NSE)、α1抗胰蛋白酶(α1-AT)、突触素(Syn)、孕激素(PR)阳性,上皮膜抗原(EMA)、雌激素(ER)、嗜铬颗粒素A(CgA)、S-100蛋白阴性。4例行胰十二指肠切除术,4例行肿瘤局部切除术。4例行胰体尾加脾切除术,1例胰体部肿瘤行胰腺节段切除术。12例获随访,中位随访时间41(2～103)个月,肿瘤无复发,除1例出现营养不良外,余患者生存满意。结论胰腺实性假乳头状瘤为低度恶性肿瘤,确诊需依赖病理组织学检查。手术切除是治疗本病的良好方法。     

膀胱副神经节瘤的诊治（附3例报告）

目的：提高对膀胱副神经节瘤的诊断和治疗水平。方法：回顾性分析3例膀胱副神经节瘤患者的临床和病理资料：3例患者均有高血压病史,其中2例表现为排尿时阵发性高血压,1例表现为间歇性肉眼血尿。肿瘤直径1．5～3．0cm。2例行膀胱部分切除术,1例行TURBT术。结果：病理检查组织细胞多数为多边形,有的为梭形,富于嗜伊红细颗粒状胞浆,核分裂少见,间质富含毛细血管,个别细胞核有非典型性及多核瘤巨细胞。免疫组化染色CgA、NSE、Syn阳性,病理诊断为副神经节瘤。3例患者随访16～35个月,所有患者术后未见复发及转移,高血压治愈2例,明显改善1例。结论：膀胱副神经节瘤的典型表现为与排尿有关的阵发性头痛、头晕等高血压发作症状,少数可表现为肉眼血尿;应用B超、CT、膀胱镜可做定位诊断,尿VMA和儿茶酚胺可作定性诊断;由于可能诱发高血压危象,术前不主张活检。手术切除是首选治疗方法,术后应严密随访。     

1例未预计的前列腺副神经节瘤手术的麻醉处理

文章报道了1例未预计的前列腺副神经瘤的麻醉处理过程.患者在麻醉诱导和手术内镜操作时出现高血压危象,BP控制后继续完成前列腺剜除手术,术中高血压危象发生时留取血样的儿茶酚胺检测结果、术后组织病理及免疫组化结果证实该例患者为前列腺副神经节瘤.患者预后良好.术中未预计的嗜铬细胞瘤或副神经节瘤是麻醉医师围手术期面临的重大挑战,为临床提供借鉴.     

双侧肾上腺嗜铬细胞瘤并腹膜后副神经节瘤1例报告并文献复习

目的:探讨嗜铬细胞瘤及副神经节瘤的诊断和治疗。方法:诊治1例双侧肾上腺嗜铬细胞瘤并腹膜后副神经节瘤的患者。该患者同时患有高血压、Ⅱ型糖尿病,术前血浆及尿游离儿茶酚胺水平明显高于正常,CT检查提示双侧肾上腺富血供占位病变,腹膜后占位病变。结果:术前口服酚苄明降压及充分扩容后,全麻下行开放手术切除右侧肾上腺肿瘤,3个月后腹腔镜下切除左肾上腺肿瘤及腹膜后肿瘤。病理检查诊断为双侧肾上腺嗜铬细胞瘤、腹膜后副神经节瘤。患者术后半月余血糖恢复正常,血压较术前无明显变化,随访6个月无肾上腺皮质功能减退表现,未见肿瘤复发和转移。结论:提高认识、选择适当检查是诊断嗜铬细胞瘤和副神经节瘤的关键,进行充分的术前准备后手术切除肿瘤病灶是其首选治疗方法,术后应严格随访。     

前列腺异位嗜铬细胞瘤1例报告并文献复习

目的报告一例异位在前列腺的嗜铬细胞瘤,探讨其发生机制及诊疗。方法回顾性分析我院收治的1例前列腺异位嗜铬细胞瘤患者的临床资料,总结归纳其临床特征及影像学表现,并结合文献进行分析。结果患者24h尿香草扁桃酸(VMA)等检查基本正常,CT及MRI检查示膀胱外侧,前列腺左侧叶凸出一大小约4.0cm×3.1cm×3.0cm肿块。予以行腹腔镜下前列腺肿瘤切除术,术后标本示肿物剖面呈金黄色,大小为4.0cm×2.8cm×2.0cm,有完整包膜,免疫组化结果:CgA(+),NSE(+),CD56(+),S100(-),Ki67(阳性率1%),病理诊断为副节瘤。结论异位嗜铬细胞瘤较少见,异位在前列腺上嗜铬细胞瘤极为罕见,CT、磁共振成像(MRI)及~(131)I-间碘苄胍(~(131)I-MIBG)用于定位诊断,CT平扫+增强有其特异表现,腹腔镜手术可完整切除,确诊依据病理。     

An extremely large solitary primary paraganglioma of the lung: Report of a case

We present herein the case of a 38-year-old woman found to have an extremely large solitary primary paraganglioma of the lung. The patient presented with chest pain on exertion and a mass was discovered in the left lower lobe of the lung by chest X-rays and computed tomography (CT). As no other neoplasms were detected elsewhere, a left lower lobectomy was performed. The patient has remained well without any evidence of recurrence for 5 years since her operation. The tumor, measuring 13×12×7 cm, was composed of ovoid cells (Zellballen), which were positive for Fontana-Masson and Grimelius stains, and sustentacular cells. Immunohistochemically, the ovoid cells were positive for neuron-specific enolase, S-100, CAM5.2, Leu7, and chromogranin A, and negative for carcinoembryonic antigen and epithelial membrane antigen. The sustentacular cells were positive for S-100 protein and CAM5.2, and negative for glial fibrillary acid protein. Therefore, the tumor was diagnosed as a paraganglioma. The tumor from our patient is the largest of the 17 solitary primary pulmonary paragangliomas reported thus far in the English-language literature.     

Diagnostic immunohistochemistry of neuroblastic tumors.

Eighteen commercially available antibodies were applied to formalin-fixed, paraffin-embedded neuroblastomas (NBLs, n = 20), ganglioneuroblastomas (GNBLs, n = 7), and ganglioneuromas (GNs, n = 7) to assess their reliability as markers for neuroendocrine differentiation and degree of tumor cell maturation. Incubations with a monoclonal antibody to neuron-specific enolase resulted in positive reactions in all tumors, with consistently strong staining intensities in moderate and well-differentiated NBLs, GNBLs, and GNs. Antibodies to dopamine beta-hydroxylase and protein gene product (PGP) 9.5 reacted with all tumors except two NBLs. Among the antibodies directed to chromogranins and related proteins, HISL19 was most reliable (33/34) followed by endocrine granule constituent (EGC) (30/34), chromogranin A (LK2H10) (21/34), and chromogranin A + B (CGA + B) (19/34), in proving the existence of endocrine granules in tumor cells and Neurofilament (70 + 200 kD) immunoreactivity was demonstrated in all tumors except two undifferentiated NBLs. S-100 protein-immunoreactive cells were visualized with increasing frequency in highly differentiated GNBLs and GNs, whereas Leu 7 immunoreactivity was restricted to ganglioneuromas. We conclude that antibodies directed to neuron-specific enolase, HISL19, dopamine beta-hydroxylase, neurofilaments, EGC, LK2H10, and leucocyte common antigen represent markers that might be useful in the discrimination of GNBLs from non-neuroendocrine round and small cell tumors in routinely processed tissue. Antibodies to neuron-specific enolase, PGP 9.5, different chromogranins, neurofilaments, vasoactive intestinal peptide (VIP), and S-100 protein may help to determine the grade of tumor cell maturation.     

Primary paraganglioma strictly confined to the liver and mimicking hepatocellular carcinoma: an immunohistochemical and in situ hybridization study

We describe a case of primary nonfunctioning paraganglioma that, unlike any other previously reported case, was strictly confined to the liver and must therefore have arisen on liver parenchyma. An asymptomatic 46-year-old man was referred to us for laparotomy and a right hemihepatectomy after a preoperative diagnosis of fibrolamellar hepatocellular carcinoma, based on a fine-needle biopsy. An 8-cm resiliently firm, pale gray nodule with a large central area of fibrosis and a thin fibrous capsule was resected. The polygonal eosinophilic tumor cells containing round nuclei lacking nucleoli were arranged in small nests set in a vascularly rich stroma. At immunohistochemistry neoplastic cells were strongly positive for chromogranin A, neuron-specific enolase, synaptophysin, and IGF-II protein; they were negative for keratin, S-100 protein, CD10, vimentin, and smooth muscle actin. In situ hybridization confirmed that, as in other sites, liver paraganglioma can express IGF-II gene. Conversely (and unlike hepatocellular carcinomas), the neoplastic cells did not express albumin mRNA, which was detected only in surrounding hepatocytes. The clinical course was benign and the patient is well and free of neoplastic disease 9 years after surgery. Knowledge of the entity should avoid possible confusion with hepatocellular carcinoma, especially of the fibrolamellar variety.     

Unusual sinonasal small-cell neoplasms following radiotherapy for bilateral retinoblastomas

Two patients developed sinonasal small-cell neoplasms that arose 22 years and 37 years, respectively, following radiotherapy for bilateral retinoblastomas. The tumors were composed of small cells with scant cytoplasm and had a few scattered Homer-Wright rosettes. Immunohistochemically, one tumor was positive for keratin (CAM 5.2 and AE1/AE3), epithelial membrane antigen, and neuron-specific enolase. The other neoplasm was immunoreactive for keratin (CAM 5.2 only) and neuron-specific enolase; it also had focal immunopositivity for S-100 protein, desmin, and muscle-specific actin. Both were negative for CEA, vimentin, melanocyte-specific antigen (HMB45), chromogranin A, synaptophysin, Leu-7, 200 kd neurofilament, and retinal S-antigen. Despite aggressive multimodal therapy, the patients died of metastatic tumor 7 months and 10 months following their initial diagnosis, respectively. Although osteosarcoma is the most frequent second cancer following bilateral retinoblastomas, some patients develop clinically aggressive sinonasal small-cell tumors that are difficult to place into conventional classifications. Both of our cases showed evidence of multidirectional differentiation; one tumor labeled with epithelial and neural markers, and the other expressed epithelial, neural, and myogenous antigens.     

Pulmonary gangliocytic paraganglioma: case report and comparative immunohistochemical study of related neuroendocrine neoplasms

The authors report a case of gangliocytic paraganglioma of the lung, which has not yet been described in a pulmonary neoplasm. A 75-year-old man underwent right middle and lower lobe lobectomy. A slightly yellowish mass was located at the bifurcation between the lower and middle lobe bronchus, protruding into the truncus intermedius. The neoplastic cells were composed of three cellular elements: uniform endocrine cells in a Zellballen arrangement, large ganglion-like cells within the nests of endocrine cells, and spindle-shaped cells arranged in streams to surround the nests. Each component exhibited the characteristic immunohistochemical properties, which were similar to those of the corresponding neuroendocrine neoplasms: Endocrine cells were positive for CAM 5.2, chromogranin A, and synaptophysin, like carcinoid tumor; ganglion-like cells were positive only for neurofilament, like ganglioneuroma; and spindle-shaped cells were positive for neurofilament and S-100 protein, like paraganglioma. These results agreed with those in gangliocytic paraganglioma of the duodenum. Pulmonary gangliocytic paraganglioma is similar to that in the duodenum, and is a hamartomatous proliferation of epithelial endocrine and neuronal cells of the bronchus.     

Metastatic prostate cancer with normal level of serum prostate-specific antigen

The clinical and pathological features of metastatic prostate cancer with normal level of serum prostate-specific antigen (PSA) were investigated. Four patients with metastatic prostate cancer had serum PSA within the normal range at the diagnosis. All tumors were poorly-differentiated adenocarcinoma. Endocrine therapy was performed as the initial therapy in all patients. Despite subsequently treatment, all cases died of prostate cancer at 2, 8, 9 and 38 months. During disease progression, 3 of 4 patients had elevated serum markers such as carcinoembryonic antigen (CEA), CA19-9, CA15-3, CA125, neuron-specific enolase and pro-gastrin releasing peptide. Immunohistochemical examination of the initial biopsy specimens revealed that 4 and 3 cases were positive for CEA and chromogranin A, respectively. In advanced prostate cancer patients with low PSA level, those markers may aid in the follow up of disease.     

Cushing's syndrome in prostate cancer. An aggressive course of prostatic malignancy

We report a case with an initial diagnosis of adenocarcinoma of the prostate in whom Cushing's syndrome developed. The disease did not respond to estrogen treatment and the patient died of severe septicemia. Histopathologic examination of the autopsy specimens revealed a small cell carcinoma intermingled with a moderately differentiated adenocarcinoma in the prostate and widespread metastases of small cell carcinoma. Immunoreactivity for neuroendocrine differentiation was found only in the small cell carcinoma. Determination of different tumor markers in plasma samples showed markedly elevated levels of prostate-specific antigen as well as carcinoembryonic antigen prior to treatment, with no significant changes after treatment. The concentration of the neuroendocrine marker chromogranin A was initially within the normal range, but increased during estrogen treatment, whilst neuron-specific enolase was moderately elevated throughout the observation period. Copyright Copyright 1999 S. Karger AG, Basel     

Poorly differentiated neuroendocrine carcinoma of the seminal vesicle

We describe an extremely rare case of poorly differentiated neuroendocrine carcinoma arising from the seminal vesicle. A 67-year-old man presented with a left humeral bone tumor resulting in a pathological fracture. Positron emission tomography scan disclosed a large pelvic tumor mimicking prostatic cancer invading into the seminal vesicle. Laboratory data showed an elevation of neuron-specific enolase, despite the normal prostate-specific antigen. Transrectal needle biopsy showed a poorly differentiated carcinoma of the right seminal vesicle and the metastasis of the pelvic lymph node. Immunohistochemical results were compatible with the features of neuroendocrine carcinoma; synaptophysin, chromogranin A and CD 56 were positive. The previously biopsied bone tumor was finally diagnosed as a metastasis. A systemic chemotherapy using etoposide and cisplatin failed. The patient died of cancer one-and-a-half years later.