Cerebral venous sinus thrombosis due to external compression of internal jugular vein

Cerebral venous sinus thrombosis (CVST) is a special subtype of stroke that may be life-threatening in severe cases. CVST has distinct risk factors and is frequently overlooked because of its initially nonspecific clinical presentation. We herein describe a 72-year-old man who developed CVST in the right lateral sinus. Despite the absence of common risk factors in this patient, he developed external compression of the bilateral internal jugular veins by a lateral mass of the C1 vertebra and expansion of the carotid artery. Because of his elevated D-dimer and fibrinogen concentrations, which are associated with ongoing activation of the coagulation system, the patient underwent treatment with batroxobin combined with anticoagulation. Recanalization of the sinus was achieved, and his high intracranial pressure and papilledema remarkably decreased. We conclude that external compression of the internal jugular veins, which can be identified with three-dimensional computed tomography venography, may be an important risk factor for CVST.     

Overview Article: Ultrastructural Features of the Common Epithelial Tumors of the Ovary: Part II

The ultrastructural features of benign and malignant serous, mucinous, and endometrial variants of ovarian carcinoma are presented. Clear cell tumors and Brenner tumors are also discussed.

Where possible, specific electron microscopic features are stressed.     

Extrathoracic Mesothelial Proliferations and Their Mimics

Mesothelial proliferations, either reactive or neoplastic in nature, often pose difficult diagnostic dilemmas. Electron microscopy continues to be a gold standard in the identification of mesothelial differentiation. However, it is very common to apply long panels of antibodies for that purpose. In most cases, light microscopy and immunohistochemistry will solve the problem. However, the definitive, specific, and sensitive immunohistochemical marker is still lacking. This is particularly true in peritoneal and testicular mesothelial tumors, in which common embryologic origin with epithelial elements results in overlapping immunohistochemistry and morphology. The particularities of peritoneal and testicular mesothelial proliferations, and the main tumors that may mimic them in these sites, as well as the value and limitations of immunohistochemistry and electron microscopy in their differential diagnosis are the subject of this review.     

White matter tract signatures of the progressive aphasias

The primary progressive aphasias (PPA) are a heterogeneous group of language-led neurodegenerative diseases resulting from large-scale brain network degeneration. White matter (WM) pathways bind networks together, and might therefore hold information about PPA pathogenesis. Here we used diffusion tensor imaging and tract-based spatial statistics to compare WM tract changes between PPA syndromes and with respect to Alzheimer's disease and healthy controls in 33 patients with PPA (13 nonfluent/agrammatic PPA); 10 logopenic variant PPA; and 10 semantic variant PPA. Nonfluent/agrammatic PPA was associated with predominantly left-sided and anterior tract alterations including uncinate fasciculus (UF) and subcortical projections; semantic variant PPA with bilateral alterations in inferior longitudinal fasciculus and UF; and logopenic variant PPA with bilateral but predominantly left-sided alterations in inferior longitudinal fasciculus, UF, superior longitudinal fasciculus, and subcortical projections. Tract alterations were more extensive than gray matter alterations, and the extent of alteration across tracts and PPA syndromes varied between diffusivity metrics. These WM signatures of PPA syndromes illustrate the selective vulnerability of brain language networks in these diseases and might have some pathologic specificity.     

KRAS (but not BRAF) mutations in ovarian serous borderline tumour are associated with recurrent low‐grade serous carcinoma

BRAF and KRAS mutations in ovarian serous borderline tumours (OSBTs) and ovarian low‐grade serous carcinomas (LGSCs) have been previously described. However, whether those OSBTs would progress to LGSCs or whether those LGSCs were developed from OSBT precursors in previous studies is unknown. Therefore, we assessed KRAS and BRAF mutations in tumour samples from 23 recurrent LGSC patients with a known initial diagnosis of OSBT. Paraffin blocks from both OSBT and LGSC samples were available for five patients, and either OSBTs or LGSCs were available for another 18 patients. Tumour cells from paraffin‐embedded tissues were dissected out for mutation analysis by conventional polymerase chain reaction (PCR) and Sanger sequencing. Tumours that appeared to have wild‐type KRAS by conventional PCR–Sanger sequencing were further analysed by full COLD (co‐amplification at lower denaturation temperature)‐PCR and deep sequencing. Full COLD‐PCR was able to enrich the amplification of mutated alleles. Deep sequencing was performed with the Ion Torrent personal genome machine (PGM). By conventional PCR–Sanger sequencing, BRAF mutation was detected only in one patient and KRAS mutations were detected in ten patients. Full COLD‐PCR deep sequencing detected low‐abundance KRAS mutations in eight additional patients. Three of the five patients with both OSBT and LGSC samples available had the same KRAS mutations detected in both OSBT and LGSC samples. The remaining two patients had only KRAS mutations detected in their LGSC samples. For patients with either OSBT or LGSC samples available, KRAS mutations were detected in seven OSBT samples and six LGSC samples. Surprisingly, patients with the KRAS G12V mutation have shorter survival times. In summary, KRAS mutations are very common in recurrent LGSC, while BRAF mutations are rare. The findings indicate that recurrent LGSC can arise from proliferation of OSBT tumour cells with or without detectable KRAS mutations. Copyright © 2013 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.     

IgA class antibodies to 2-oxo-acid dehydrogenase complex are not predictive markers of histopathological progression in primary biliary cirrhosis

Although antimitochondrial antibody (AMA) is the characteristic serological feature of primary biliary cirrhosis (PBC), its pathogenic role remains unclear. In our previous study, we reported a positive correlation between immunoglobulin (Ig) A class anti-2-oxo-acid dehydrogenase complex (2-OADC) and histopathological stage. To determine whether the appearance of IgA class anti-2-OADC by immunoblotting represents an early marker of more aggressive disease or whether it is late finding during the disease course of PBC, we tested not only the entire IgA class but also IgA1, IgA2 and secretory IgA class anti-2-OADC in serial serum samples from 15 patients with PBC. During the median observation period of 51 months, four cases showed histopathological progression (from stage 1 to 2, stage 1 to 3, stage 1 to 4 and stage 2 to 4). There was no statistically significant correlation between the above IgA class anti-2-OADCs and histopathological progression. There was no significant correlation between histopathological stages and IgA2 class anti-2-OADC or secretory IgA class anti-2-OADC by immunoblotting. IgA class anti-2-OADC was more frequent in stages 3–4 than in stages 1–2 (p = 0.0049), but IgA1 class anti-2-OADC was more frequent in stages 1–2 than in stages 3–4 (p = 0.0232). Our present study demonstrated that serum IgA class 2-OADC was not a predictive marker of histopathological progression but was associated with the histopathological stage of PBC. Although the IgA class AMA may have a specific pathogenic role for PBC, the discrepant results between IgA and IgA1 class anti-2-OADC should be further assessed to investigate different functional activities depending on their molecular form.     

原发性三叉神经痛微血管减压术中困难减压的处理

目的 探讨原发性三叉神经痛（PTN）微血管减压术（MVD）出现困难减压的处理方法以及疗效。方法 回顾性分析2013年1月至2019年12月采用MVD治疗的408例PTN的临床资料。术中出现困难减压56例,常规减压352例。对于困难减压病人,综合采取包括安置腰大池引流、神经内镜辅助、扩大松解桥小脑角区蛛网膜粘连、经小脑水平裂-小脑桥脑裂入路解剖显露,以及“架桥”等方法妥善处理。结果 术后随访0.5~5年。常规减压组术后即刻缓解37例,延迟缓解14例,无效5例;困难减压组术后即刻缓解267例,延迟缓解62例,无效23例。常规减压组术后即刻缓解率（75.85%,267/352）与困难减压组（66.07%,37/56）无统计学差异（P>0.05）,常规减压组术后有效率（93.54%,329/352）与困难减压组（91.07%,51/56）无统计学差异（P>0.05）。常规减压组术后总并发症发生率（7.7%,27/352）与困难减压组（10.7%,16/56）无统计学差异（P>0.05）。两组均无手术死亡病例及严重后遗症。结论 PTN病人MVD中出现的困难减压并不是影响MVD疗效的决定性因素,只要术中处理得当,困难减压病人术后也可以取得很好的疗效。术前应熟悉和掌握常见的困难减压的应对方法,并且秉持适可而止的理念,减少手术并发症,可进一步提高手术疗效。     

原发性人脑胶质瘤的预后影响因素:基于SEER数据库的分析

目的 探讨原发性人脑胶质瘤病人预后影响因素。方法 通过SEER*Stat（8.3.8版本）软件搜集SEER数据库2004~2015年原发性人脑胶质瘤病人的临床资料,采用R（4.0.2版本）软件进行单因素与多因素Cox回归分析,通过Kaplan-Meier生存曲线分析不同治疗方式和婚姻状态与病人预后的关系。结果 共纳入符合标准的原发性人脑胶质瘤18 523例。多因素Cox回归分析结果显示,年龄≥30岁、肿瘤直径≥2 cm、病理级别高、肿瘤位于额叶以外部位、离婚、丧偶为原发性胶质瘤预后不良的独立危险因素（P<0.05）,手术治疗、术后放疗、化疗是原发性胶质瘤预后的独立保护因素（P<0.05）。生存曲线分析结果显示,手术治疗（全切除或部分切除肿瘤）、术后放疗、化疗均明显延长原发性胶质瘤病人的生存期（P<0.05）,婚姻状态为结婚的病人预后明显好于丧偶的病人（P<0.05）。结论 对原发性人脑胶质瘤,尽可能手术全切除肿瘤,同时术后辅助放化疗,能够延长病人的生存时间。同时,临床应加强病人心理干预。