膀胱副神经节瘤的诊治分析(附1 例病例报告并文献复习)

目的:总结膀胱副神经节瘤的诊治方法及体会,提高膀胱副神经节瘤的诊断和治疗水平。方法:报告1例膀胱嗜铬细胞瘤的临床资料并结合文献复习分析总结膀胱副神经节瘤的诊断治疗方法。结果:患者行腹腔镜下膀胱部分切除术,手术成功,术后顺利出院,随访至今无转移。结论:膀胱副神经节瘤的诊断主要依靠临床表现和实验室检查,血尿、高血压和排尿时典型发作三联征为膀胱副神经节瘤的主要症状,对于瘤体较小的患者要警惕CT检查漏诊的可能性,可行活组织检查进行确诊。腹腔镜下切除是目前治疗膀胱副神经节瘤最有效、创伤最小的方法,但术后要严格随访。     

乳腺浸润性微乳头状癌临床病理及免疫组织化学分析

目的探讨乳腺浸润性微乳头状癌的临床病理特征、诊断与鉴别诊断要点。方法对乳腺浸润性微乳头状癌进行临床病理分析、组织形态学及免疫组化染色观察,结合文献对其临床表现、病理形态特点及鉴别诊断进行探讨。结果浸润性微乳头状癌光镜下特征性表现为癌巢由呈桑椹状或(和)腺管型的微乳头状癌巢组成,与网状间质间形成明显的空隙(主间质分离)。瘤细胞ER、PR、C-erbB-2、CD44V6阳性;Actin(sm)阴性;EMA在瘤细胞簇外周细胞膜和中间腔缘呈阳性表达。结论乳腺浸润性微乳头状癌可在病理诊断时作为一种单独的类型提出。     

副神经节瘤伴腰椎转移一例报道并文献复习

目的:探讨腹膜后非嗜铬副神经节瘤(副节瘤)的诊断与治疗方法.方法:诊断为肾上腺癌并腰椎转移的患者,女,38岁;手术采用经胸腹联合切口,切除第9肋,打开膈肌,由于肾脏和肿瘤粘连紧密,行腹膜后肿瘤及左肾联合切除术.结果:肿瘤完整切除,肿瘤10cm× 9cm× 7cm.病理诊断为倾向来源于肾上腺的副神经节瘤.腰椎手术后,病检证实是副神经节瘤转移.结论:恶性副神经节瘤对化疗和放疗不敏感,手术是唯一有效的治疗方法.     

原发性腹膜后副神经节瘤临床诊疗分析

目的:探讨原发性腹膜后副神经节瘤的手术治疗效果,复发及死亡的影响因素。方法:回顾性分析1985年1月至2015年1月30年间于我院治疗的91例原发性腹膜后副神经节瘤的手术患者的临床资料,统计学方法分析腹原发性腹膜后副神经节瘤手术的治疗效果,预后及复发和死亡的相关因素。结果:77例原发性腹膜后良性副神经节瘤患者,l、3、5年总生存率均为98.5%,复发率分别为2%,4.5%,16%。手术根治度与复发相关(X~2=10.368,P=0.01),手术未完整切除复发率高。性别、年龄、肿瘤部位、肿瘤直径与复发无关(P0.05)。14例原发性腹膜后恶性副神经节瘤患者,1、3、5年总生存率分别为78.5%、50%,41.5%。14例恶性副神经节瘤复发率分别为71%,85%,100%。肿瘤级别与腹膜后恶性副神经节瘤预后(X~2=5.536,P=0.019)和复发相关(X~2=5.734,P=0.017),肿瘤级别低,死亡率高,复发率高;远处转移与复发相关(X~2=4.067,P=0.044),远处转移患者复发率高;性别、年龄、肿瘤部位、肿瘤直径、手术根治度与预后及复发无关(P0.05)。结论:良性腹膜后副神经节瘤患者预后较好,手术根治度与复发相关,根治性手术是其主要治疗方式;恶性副神经节瘤预后差,肿瘤分级与其复发及死亡相关,肿瘤分级低复发及死亡率高,远处转移与复发相关,远处转移患者复发率高。     

50例胃肠道间质瘤的临床病理分析

目的：探讨胃肠道间质瘤（GIST）的组织形态学特征及对该类肿瘤的诊断及预后问题。方法：对发生于2001年4月至2010年4月间的50例GIST进行临床病理及免疫组化分析。结果：50例GIST免疫组化检查结果,肿瘤均不表达CK而表达为Vimentin;CD117阳性表达43例,CD34阳性表达38例;S100均有不同程度表达,SMA阳性表达22例,Desmin阳性表达15例;Ki-67均有表达,其阳性度从＋．＋＋不等。结论：本组肿瘤中所有病例均不同程度表达Ki-67和PCNA,高危险性的阳性表达强、低危险性的阳性表达弱。这说明GIST的预后除与肿瘤的大小、组织形态学的改变及肿瘤的发生部位有关外,还与Ki-67和PCNA在肿瘤中的表达程度有关。     

肺淋巴管平滑肌瘤病临床病理分析及文献复习

目的：探讨肺淋巴管平滑肌瘤病（PLAM）的临床和病理特征,提高对该病的认识。方法：对1例PLAM患者的临床特点、HE及免疫组化染色结果进行分析,并结合文献进行复习。结果：PLAM是一种持续发展的弥漫性肺疾病,几乎发生于育龄期妇女,主要临床症状为进行性呼吸困难、反复气胸及乳糜胸。病理特征显示未成熟平滑肌样细胞在细支气管壁、肺泡壁、淋巴管壁和血管壁周围增生。免疫组化染色显示增生PLAM细胞Desmin、SMA、Vimentin、HMB45均呈阳性表达,部分细胞ER及PR阳性。结论：PLAM在影像学与病理组织学上有特征性表现,免疫组化HMB-45阳性具特异性。由于肺部广泛病变,预后较差．对PLAM的发病机制和治疗的研究已有了一定的进展,但今后尚需更深入地研究。     

肝脏假性淋巴瘤的临床病理学研究及分析

目的:探讨肝脏假性淋巴瘤的临床病理特征、免疫表型及鉴别诊断。方法:在光学显微镜下对肝脏假性淋巴瘤进行组织学形态观察,并借助免疫组化进一步对其形态进行分析。由于原发于肝脏的假性淋巴瘤极为罕见,故本文将报道一例发生于肝脏的假性淋巴瘤,结合文献探讨其临床病理特点,以提高诊断及鉴别诊断水平。结果:大体上为切面可见灰白结节,结节切面灰白色,质地中等,与周围分界清,周围肝组织灰红质软。显微镜下组织学表现为肝周边淋巴结淋巴组织增生,细胞大小较一致,核圆形,细胞无异型,未见明显核分裂,其中见嗜酸性白细胞浸润及少数异型细胞。免疫组化显示肿瘤细胞表达CD3、CD20及CD30。结论:肝脏假性淋巴瘤为罕见的、良性淋巴组织增生性病变,形态学特征、免疫组化染色在肝假性淋巴瘤诊断中具有重要价值。在临床病理实践中,必须首先与常见的发生于该部位恶性肿瘤如霍奇金淋巴瘤肿瘤等鉴别。     

干扰OVA66基因表达对移植瘤生物学功能的影响

目的探讨干扰OVA66基因表达对移植瘤细胞生物学功能的影响。方法采用脂质体法,以重组的pSUPER-shRNA-OVA66和空载体转染HeLa细胞获得稳定表达细胞株,经RT-PCR和Western blot鉴定OVA66基因的表达。将两组细胞接种于BALB／cnu／nu裸鼠的前肢腋下,连续观察干扰OVA66表达对肿瘤的生长影响。并于接种4周后,应用RT-PCR,免疫组化、免疫荧光等方法检测移植瘤细胞中OVA66的蛋白表达。组织病理分析肿瘤细胞生长和转移的特征。结果经检测pSUPER-shRNA-OVA66能有效抑制目的基因的表达。比较两组荷瘤小鼠中肿瘤的生长曲线发现,OVA66实验组的肿瘤生长速度明显低于对照组,并且肿瘤细胞体内转移和浸润能力显著降低。结论干扰OVA66基因和蛋白表达,可以抑制肿瘤细胞的生长,浸润和侵袭能力。     

PML（NLS^-）蛋白在NB4细胞及其裸鼠移植瘤中定位的验证

验证中性粒细胞弹性蛋白酶（neutrophil elastase,NE）切割PML—RARa后,PML（NLS-）蛋白的存在和定位。将质粒pCMV-HA—NE电转染NB4细胞,用Westemblot法验证质粒转染成功;提取电转染质粒成功64／NB4细胞的胞浆蛋白,用Westem blot法检测NB4细胞中PML（NLS^-）蛋白的表达;免疫荧光法和激光共聚焦检测电转染质粒成功的NB4细胞中PML（NLS^-）蛋白的表达及定位;同时,建立NB4细胞、K562细胞和电转染质粒成功的NB4细胞裸鼠皮下瘤模型,用Westem blot、免疫组化法检测PML（NLS^-）蛋白在移植瘤组织细胞中的表达与定位。结果表明,westemblot检测电转染质粒pCMV-HA-NE的NB4细胞成功表达NE蛋白：NE酶成功切割PML-RARα,Western blot检测到电转染质粒pCMV-HA-NE的NB4细胞表达PML（NLS^-）蛋白;免疫荧光和激光共聚焦均可检测到电转染质粒成功的NB4细胞中PML（NLS^-）蛋白定位于细胞胞浆：Westem blot和免疫组化法检测到电转染质粒成功的NB4细胞裸鼠移植瘤中的PML（NLS^-）蛋白的表达且定位于细胞胞浆,而NB4和K562细胞裸鼠皮下瘤中PML蛋白主要定位于胞核。综上所述,该文成功将质粒pCMV-HA—NE电转染NB4细胞并用Western blot、免疫荧光、激光共聚焦、免疫组化验证PML（NLS-）蛋白存在于NB4细胞胞浆,这一现象可以为急性早幼粒细胞白血病的临床早期诊断与治疗提供新的依据。     

睾丸间质细胞瘤3例报告及文献复习

目的：探讨睾丸间质细胞瘤的临床病理特点及诊疗方法。方法：分析并总结3例睾丸间质细胞瘤患者的,J盏床病理资料并文献复习。结果：1例术中冰冻切片诊断为睾丸间质细胞瘤,2例术前细针穿刺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。2例患者行单侧睾丸切除,1例行睾丸肿瘤剜除术,术后分别随访24、15、10个月未见复发。结论：睾丸间质细胞瘤发病率低,临床表现缺乏特异性,易误诊,确诊需依赖病理组织学检查,细针穿刺病理可明确诊断并有助于手术的选择及手术范围的确定。     

Spontaneous neurite outgrowth and vasoactive intestinal peptide-Iike immunoreactivity of cultures of human paraganglioma cells from the glomus jugulare

Summary The chief cells of paraganglionic tissues have morphological and functional similarities to adrenal chromaffin cells, and both cell types are derived from the neural crest. In the present investigation cells from two glomus jugulare paragangliomas were studied in culture. Approximately 50% of the cells from one tumor, and 7% from the other spontaneously formed neurite-like processes. Numerous granular and agranular synaptic-like vesicles also appeared in the process-forming cells. In contrast to findings with normal and neoplastic adrenal chromaffin cells, addition of nerve growth factor (NGF) to the culture medium had no major effects on proportion of cells with processes. Dexamethasone caused only a small decrease in process length. Culturing of the tumors also appeared to promote production of material with VIP-like immunoreactivity. It is concluded that the phenotype of paraganglioma as well as pheochromocytoma cells may be altered in vitro. Responsiveness to specific factors such as NGF or steroids, however, may vary for related tumor cell types in different anatomic locations.     

Immunocytochemical study on the localization of neuron-specific enolase and S-100 protein in the carotid body of rats

Light- and electron-immunocytochemical investigation with the peroxidase-antiperoxidase (PAP) procedure revealed neuron-specific enolase and S-100 protein-like immunoreactivities specifically localized in the chief cells and the sustentacular cells of the rat carotid body, respectively. This finding suggests a neuron-like nature of the chief cells and a glia-like nature of the sustentacular cells on both embryological and functional bases.     

Pigmented paraganglioma of the kidney: a case report

ABSTRACT: Paragangliomas are rare neoplasms arising from undifferentiated cells of the primitive neural crest. We report a case of a 57-year-old patient with renal pigmented paraganglioma that was an incidental finding. Histopathological examination showed typical morphology of paraganglioma, as well as the unusual feature of large amounts of pigment in the cytoplasm of the tumor cells which was confirmed by bleached Fontana-Masson. Electron microscopy showed abundant, pleomorphic electron-dense granules consistent with neuromelanin. The tumor cells were positive for CD56 and chromogranin A, negative for HMB-45. The unique morphologic appearance represents divergent differentiation from neural crest. To our knowledge, the present case represents the first example of pigmented paraganglioma of the kidney. Virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2017147293711495.     

Immunohistochemical identification and comparison of glial cell lineage in foetal, neonatal, adult and neoplastic human adrenal medulla

The differentiation of glial cells in developing, neonatal, adult and neoplastic human adrenal medulla has been studied immunohistochemically. From 8 to 28 weeks' gestational age, S-100 protein and its β-subunit revealed two different glial cell populations in adrenal glands, namely Schwann-like and sustentacular cells. Schwann-like cells were spindle-shaped cells forming a continuous layer around groups of sympathetic neuroblasts, often in contact with Schwann cells of nerve fibres entering neuroblastic groups. Sustentacular cells were round or oval cells with dendritic cytoplasmic processes; they were not associated with nerve fibres and mingled both with sympathetic neuroblasts and differentiating chromaffin cells. The developmental fate of Schwann-like cells was different from that of sustentacular cells. Schwann-like cells disappeared from the 28th week of gestational age, in association with the disappearance of sympathetic neuroblastic groups, and they were rarely found in neonatal and adult adrenal medulla. In contrast, sustentacular cells persisted between medullary chromaffin cells, and their number and dendritic cytoplasmic processes progressively increased from foetus to adult. In eight cases of primitive adrenal neuroblastic tumours of neonatal age (five undifferentiated neuroblastomas and three ganglioneuroblastomas), Schwann-like cells were found at the periphery of tumoral nests with a lobular growth pattern, while rare sustentacular cells were associated with neuroblasts. In two cases of adult phaeochromocytomas, only sustentacular cells were detected between chromaffin tumoral cells. Our findings suggest that the glial cell types and their distribution in primitive adrenal medulla tumours closely resemble those observed during development in the groups of adrenal sympathetic neuroblasts and in the clusters of chromaffin cells     

Hypoglycemia due to ectopic release of insulin from a paraganglioma

Insulin-secreting pancreatic tumors and insulin-like growth hormone-secreting non-islet cell tumors can cause hypoglycemia. However, insulin-releasing paraganglioma or pheochromocytoma has almost never been reported. A 67-year-old female patient was admitted to our hospital because of headache, palpitation, perspiration, faintness, frequent sense of hunger and absent-mindedness. These intermittent symptoms had begun approximately a year before admission. On physical examination, she had high blood pressure of 150/90 mm Hg. Hormonal studies demonstrated increased urinary norepinephrine levels, and hyperinsulinemic hypoglycemia was confirmed while the patient was symptomatic. Abdominal MRI revealed a retroperitoneal mass measuring 4.5 cm in the pancreatic region. She was treated with an alpha-blocking agent to control blood pressure preceding the removal of the mass. Histopathological diagnosis was paraganglioma, and immunohistochemically insulin staining in the neoplastic cells was demonstrated. Her blood pressure normalized and hypoglycemia relieved after the operation. The patient did not have recurrence of hypoglycemia after a year of follow-up. Paraganglioma is a rare tumor of the neural crest, and co-secretion of insulin and catecholamines has been reported only by a single case report in the literature. The present patient is another case with this co-secretion.     

Immunohistochemical identification and comparison of glial cell lineage in foetal, neonatal, adult and neoplastic human adrenal medulla

The differentiation of glial cells in developing, neonatal, adult and neoplastic human adrenal medulla has been studied immunohistochemically. From 8 to 28 weeks' gestational age, S-100 protein and its β-subunit revealed two different glial cell populations in adrenal glands, namely Schwann-like and sustentacular cells. Schwann-like cells were spindle-shaped cells forming a continuous layer around groups of sympathetic neuroblasts, often in contact with Schwann cells of nerve fibres entering neuroblastic groups. Sustentacular cells were round or oval cells with dendritic cytoplasmic processes; they were not associated with nerve fibres and mingled both with sympathetic neuroblasts and differentiating chromaffin cells. The developmental fate of Schwann-like cells was different from that of sustentacular cells. Schwann-like cells disappeared from the 28th week of gestational age, in association with the disappearance of sympathetic neuroblastic groups, and they were rarely found in neonatal and adult adrenal medulla. In contrast, sustentacular cells persisted between medullary chromaffin cells, and their number and dendritic cytoplasmic processes progressively increased from foetus to adult. In eight cases of primitive adrenal neuroblastic tumours of neonatal age (five undifferentiated neuroblastomas and three ganglioneuroblastomas), Schwann-like cells were found at the periphery of tumoral nests with a lobular growth pattern, while rare sustentacular cells were associated with neuroblasts. In two cases of adult phaeochromocytomas, only sustentacular cells were detected between chromaffin tumoral cells. Our findings suggest that the glial cell types and their distribution in primitive adrenal medulla tumours closely resemble those observed during development in the groups of adrenal sympathetic neuroblasts and in the clusters of chromaffin cells     

Ultrastructural studies of vagal paraganglia in Syrian hamsters

Summary Typical vagal paraganglia of Syrian hamsters are encapsulated in connective tissue and consist of groups of epithelial cells. Ganglion cells, a few fenestrated capillaries, and bundles of unmyelinated nerve fibers are intermingled among the parenchymal cells. The parenchymal cells are of two types: chief or paraganglion and sustentacular or supporting cells. The processes of the supporting cells partly or completely surround the paraganglion cells. In addition to the nucleus, Golgi complex, mitochondria, parallel-arrayed granular endoplasmic reticulum, and lipofuscin pigment, the chief cells are characterized by the presence of numerous membrane-bound, electron opaque granules. After an injection of ³H-dopa, labelings were concentrated over the chief cells and were associated predominantly with the granules. Following glutaraldehyde-dichromate treatment the granules gave a positive reaction for unsubstituted amines. These results suggest that the chief cells contain catecholamines in the electron opaque granules.Research supported by USPHS Grants NS 05665, 00690 and HE 12751. A preliminary report of this research was presented before the American Society for Cell Biology, 1969.Sponsored by National Council on Science Development, Republic of China.Recipient of Career Research Development Award 1 K3 GM 28064.     

Locally advanced duodenal gangliocytic paraganglioma treated with adjuvant radiation therapy: case report and review of the literature

BACKGROUND: Gangliocytic paraganglioma are rare neoplasms that predominantly arise in periampulary region. Though considered benign the disease can spread to regional lymphatics. CASE PRESENTATION: A 49 year old woman presented with melena and was found to have a periampullary mass. Endoscopic evaluation and biopsy demonstrated a periampullary paraganglioma. The tumor was resected with pylorus-preserving pancreaticoduodenectomy and was found to represent a gangliocytic paraganglioma associated with nodal metastases. In a controversial decision, the patient was treated with adjuvant external beam radiation therapy. She is alive and well one year following resection. The authors have reviewed the current literature pertaining to this entity and have discussed the biologic behavior of the tumor as well as the rationale for treatment strategies employed. CONCLUSION: Paraganglioma is a rare tumor that typically resides in the gastrointestinal tract and demonstrates low malignant potential. Due to rarity of the disease there is no consensus on the adjuvant treatment even though nearly 5% of the lesions demonstrate the malignant potential.