Schwannomas and Their Pathogenesis

Schwannomas may occur spontaneously, or in the context of a familial tumor syndrome such as neurofibromatosis type 2 (NF2), schwannomatosis and Carney's complex. Schwannomas have a variety of morphological appearances, but they behave as World Health Organization (WHO) grade I tumors, and only very rarely undergo malignant transformation. Central to the pathogenesis of these tumors is loss of function of merlin, either by direct genetic change involving the NF2 gene on chromosome 22 or secondarily to merlin inactivation. The genetic pathways and morphological features of schwannomas associated with different genetic syndromes will be discussed. Merlin has multiple functions, including within the nucleus and at the cell membrane, and this review summarizes our current understanding of the mechanisms by which merlin loss is involved in schwannoma pathogenesis, highlighting potential areas for therapeutic intervention.     

改良颈腮腺入路切除咽旁间隙高位良性肿瘤

目的 探讨改良颈腮腺入路在高位咽旁间隙良性肿瘤手术中的应用疗效。 方法 7例咽旁间隙良性肿瘤患者,术前影像学检查提示为高位、肿瘤巨大、哑铃型且边界欠清,采用改良颈腮腺入路术式,解剖辨认面神经总干及颞面干后,于外耳道软骨前方、腮腺的后缘以及颞面干的上方间隙向深部分离至肿瘤上极,剥离子分离并下压肿瘤与下方常规颈部自下而上肿瘤游离会师后,从颌下区取出肿瘤。腮腺浅叶不切除,面神经分支不做过多解剖。 结果 所有患者均一次性完整切除肿瘤;术后病理示多形性腺瘤6例,神经鞘瘤1例;术中出血均少于300 mL;1例患者出现术后同侧眼睑轻度闭合障碍,两周后完全正常;所有患者面容美学保存理想。 结论 对于高位咽旁间隙良性肿瘤采用改良颈腮腺入路术式,不仅可以安全完整地切除肿瘤,同时由于减少了面神经及腮腺浅叶的处置,术后相关神经并发症及腮腺区凹陷性改变的发生率下降。     

Where are our patients? Retrospective cohort study of acute medical unit admissions during and prior to the COVID‐19 pandemic

Admissions to hospital have declined markedly during the COVID‐19 pandemic in Australia. This may be due to patients not presenting with acute illness or managing their chronic illness at home. We reviewed a cohort admitted to the Acute Medical Unit of the Royal Melbourne Hospital during and before the pandemic and found admissions were more acutely unwell and more comorbid. This may lead to worse outcomes for those not presenting, as well as those presenting late. We recommend a public health campaign to encourage Australians to present to hospital if unwell.     

Low rate of active treatment of patients with hilar cholangiocarcinoma

Introduction

The results of surgical resection and palliative chemotherapy use in hilar cholangiocarcinoma (HC) have been well publicised but the proportion of patients able to undergo these treatments and the comparative outcomes in a population of patients with HC are less well known.

Methods

Patients with HC were identified by review of all patients undergoing percutaneous cholangiography over a nine-year period (2002–2010) in a tertiary facility. The treatment undertaken and outcomes were recorded.

Results

Overall, 68 patients were identified (37 female) with a median age of 70 years. Forty-five (66%) were treated solely by insertion of a metal stent (median survival 4.73 months) and nine (13%) also received palliative chemotherapy (median survival 13.7 months). Persisting jaundice after stent insertion was noted in 18 of 35 patients (51%) tested within one month of death. Fourteen patients (21%) underwent surgical resection (median survival 20.2 months).

Conclusions

Patients undergoing surgical resection had significantly longer survival than those receiving only a palliative stent but not compared with those also receiving palliative chemotherapy, with short-term follow-up. Only a third of patients, however, receive active treatment (surgery or chemotherapy) and improvements in long-term biliary palliation are needed.     

The Evaluation of Leukocytes in Response to the In Vitro Testing of Ventricular Assist Devices

Infection is a clinically relevant adverse event in patients with ventricular assist device (VAD) support. The risk of infection could be linked to a reduced immune response resulting from damage to leukocytes during VAD support. The purpose of this study was to develop an understanding of leukocyte responses during the in vitro testing of VADs by analyzing the changes to their morphology and biochemistry. The VentrAssist implantable rotary blood pump (IRBP) and RotaFlow centrifugal pump (CP) were tested in vitro under constant hemodynamic conditions. Automated hematology analysis of samples collected regularly over 25‐h tests was undertaken. A new flow cytometric assay was employed to measure biochemical alteration, necrosis (7‐AAD) and morphological alteration (CD45 expression) of the circulating leukocytes during the pumping process. The results of hematology analysis show the total leukocyte number and subset counts decreased over the period of in vitro tests dependent on different blood pumps. The percentage of leukocytes damaged during 6‐h tests was 40.8 ± 5.7% for the VentrAssist IRBP, 17.6 ± 5.4% for the RotaFlow CP, and 2.7 ± 1.8% for the static control (all n = 5). Flow cytometric monitoring of CD45 expression and forward/side scatter characteristics revealed leukocytes that were fragmented into smaller pieces (microparticles). Scanning electron microscopy and imaging flow cytometry were used to confirm this. Device developers could use these robust cellular assays to gain a better understanding of leukocyte‐specific VAD performance.