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.     

鼓室副神经节瘤的诊断与治疗

目的：探讨鼓室副神经节瘤的临床和病理特征。方法：复习临床病历资料,采用HE和免疫组化方法研究鼓室副神经节瘤的病理特征。结果：肿瘤实质由上皮样主细胞和短梭形支持细胞组成。主细胞中CgA、NSE和Syn呈阳性表达,支持细胞中S-100蛋白阳性表达。病理诊断为鼓室副神经节瘤,随访2年未见复发征象。结论：鼓室副神经节瘤十分罕见,临床和病理诊断困难,常需借助免疫组化协助诊断。治疗以手术切除为主。     

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。     

Synthesis of novel benzothiazole amides: Evaluation of PPAR activity and anti-proliferative effects in paraganglioma,pancreatic and colorectal cancer cell lines

The reduced activation of PPARs has a positive impact on cancer cell growth and viability in multiple preclinical tumor models, suggesting a new therapeutic potential for PPAR antagonists. In the present study, the benzothiazole amides 2a-g were synthesized and their activities on PPARs were investigated. Transactivation assay showed a moderate activity of the novel compounds as PPARα antagonists. Notably, in cellular assays they exhibited cytotoxicity in pancreatic, colorectal and paraganglioma cancer cells overexpressing PPARα. In particular, compound 2b showed the most remarkable inhibition of viability (greater than 90%) in two paraganglioma cell lines, with IC₅₀ values in the low micromolar range. In addition, 2b markedly impaired colony formation capacity in the same cells. Taken together, these results show a relevant anti-proliferative potential of compound 2b, which appears particularly effective in paraganglioma, a rare tumor poorly responsive to chemotherapy.     

Performance de la 18FDG-TEP dans le dépistage de paragangliome chez des patients porteurs d’une mutation SDHx

ObjectiveTo evaluate the performance of ¹⁸FDG-PET/CT for detecting infra-clinic paraganglioma (PGL) in SDHx mutation carriers (relatives).Patients and methodsSixty-six patients, from 13 distinct families underwent a genetic testing on the SHD genes between 2003 and 2012. Among the 45 patients with a mutation, 30 with a ¹⁸FDG-PET performed at initial work-up were included in this retrospective study. A gadolinium-enhanced magnetic resonance angiography of the neck (angio-MR) was performed in all cases, a thoracoabdominal-pelvic contrast-enhanced computed tomography (TAP-CT) in 25 cases, a TAP-MR in 20 cases, a ¹²³I-metaiodo-benzylguanidine scintigraphy (¹²³I-MIBG) in 20 cases and a somatostatin receptor scintigraphy (SRS) in 20 cases. Gold standard was histologic or composite (confirmation by another imaging method and follow-up).ResultsA tumor was found in five subjects: 2 abdominal PGL, 1 pheochromocytoma and 2 PGL of the neck. The sensitivity of ¹⁸FDG-PET was 100 %, of SRS was 80 %, of ¹²³I-MIBG was 60 % and of anatomical imaging (association between angio-MR of the neck and TAP-CT and/or TAP-MR) was 100 %. Three false positive lesions were described: 2 with the ¹⁸FDG-PET imaging and 1 with the TAP-MR technique.Conclusion¹⁸FDG-PET/CT is an excellent tool for screening SDHx relatives and should be completed by an angio-MR of the neck if suspicion of abnormality. Association of angio-MR of the neck and TAP-MR has the advantage of being a non-irradiating imaging method but with limited access in some countries.