Cellular Receptors Involved in KSHV Infection

The process of Kaposi’s Sarcoma Herpes Virus’ (KSHV) entry into target cells is complex and engages several viral glycoproteins which bind to a large range of host cell surface molecules. Receptors for KSHV include heparan sulphate proteoglycans (HSPGs), several integrins and Eph receptors, cystine/glutamate antiporter (xCT) and Dendritic Cell-Specific Intercellular adhesion molecule-3-grabbing non-integrin (DC-SIGN). This diverse range of potential binding and entry sites allows KSHV to have a broad cell tropism, and entry into specific cells is dependent on the available receptor repertoire. Several molecules involved in KSHV entry have been well characterized, particularly those postulated to be associated with KSHV-associated pathologies such as Kaposi’s Sarcoma (KS). In this review, KSHV infection of specific cell types pertinent to its pathogenesis will be comprehensively summarized with a focus on the specific cell surface binding and entry receptors KSHV exploits to gain access to a variety of cell types. Gaps in the current literature regarding understanding interactions between KSHV glycoproteins and cellular receptors in virus infection are identified which will lead to the development of virus infection intervention strategies.     

Unresolved pulmonary embolism leading to a diagnosis of pulmonary artery sarcoma

Pulmonary artery sarcomas (PAS) are rare tumors with a poor prognosis. They are often misdiagnosed as pulmonary embolism (PE) leading to futile anticoagulation treatment and delay in proper diagnosis. We present a case of a patient who was initially misdiagnosed and anticoagulated for presumed pulmonary embolism. Progressive symptoms and additional imaging led to the diagnosis of intimal pulmonary artery sarcoma for which he underwent surgical resection. This case serves as a reminder to consider pulmonary artery sarcoma in the differential diagnosis of patients with dyspnea and filling defects on CT pulmonary angiogram offering the potential for resection prior to metastasis.     

新疆地区70例艾滋病相关型卡波西肉瘤患者临床特征及预后分析

目的 分析新疆艾滋病相关型卡波西肉瘤(AIDS-KS)患者的临床特征,探讨抗反转录病毒治疗(HAART)及系统性化疗等对患者预后的影响.方法 连续收集2008年1月至2013年6月新疆维吾尔自治区第六人民医院收治的70例AIDS-KS患者的临床资料,采用回顾性研究的方法对人群特点、KS进展情况、病变部位及范围、治疗及预后等进行总结分析,并采用t检验和x2检验比较不同治疗组的疗效及预后.结果 70例AIDS-KS患者的平均年龄为(40±10)岁,男女比例2.5∶1,其中66例(94.29％)为维吾尔族,23例(32.9％)疾病进展评分为快速.KS临床症状主要有斑片、结节等,且头颈、躯干、四肢和内脏均有累及.治疗组总病死率为7.7％ (4/52),而未治疗组的12例患者中4例死亡,与治疗组比较差异有统计学意义(x2=5.861,P＜0.05).单纯HAART组和HAART序贯化疗组治疗半年后CD4+T淋巴细胞计数较治疗前明显上升(t=35.258和42.528,P＜0.01),而采用其他治疗方案的患者和未治疗的患者CD4+T淋巴细胞计数较治疗前无显著变化或下降.HAART序贯化疗组、化疗序贯HAART组和单纯化疗组中的所有患者对KS治疗均有效,而单纯HAART组的治疗有效率为86.4％ (19/22),且45.5％ (10/22)的患者在HAART后出现免疫重建炎症反应综合征(IRIS),KS症状加重.结论 AIDS-KS多发生于中青年,以维吾尔族男性多见,病灶部位多样化,部分呈快速进展.HAART联合系统性化疗疗效较好,且可降低IRIS的发生率,值得临床推广应用.     

Malignant fibrous histiocytoma presenting with complete opacification of the hemithorax: A case report

BACKGROUND

Malignant fibrous histiocytoma, a subtype of primary lung sarcoma is a very rare disease. It usually presents as a lung nodules and the final diagnosis is made by immunohistochemical studies.

METHODS

A 45-year-old patient presented with progressive dyspnea, dry cough and right shoulder pain. Chest X-ray revealed complete opacification of the right hemithorax. Chest computed tomography confirmed the presence of a heterogeneous lesion occupying the whole right hemithorax causing a mass effect on the trachea. Ultrasound guided biopsy was done and final pathology was suggestive of malignant fibrous histiocytoma.

CONCLUSION

Progressive dyspnea in young otherwise healthy patients should be investigated early on. In our case the presence of right shoulder pain indicates advance disease illustrated by the singular imaging findings.     

Malignant primary intraosseus synovial sarcoma – a rare case report

Primary intraosseous synovial sarcoma is an extremely rare malignancy that occurs primarily in young adults. We present a case of a primary intraosseous synovial sarcoma of the right distal ulna in a 19-year-old female. It has a propensity to mimic other radiologic and pathologic diagnosis. Histopathology after a surgical excisional biopsy with a wide margin plus adjunct radio and chemotherapy are necessary to improve prognosis.     

艾灸通过成纤维细胞生长因子受体1和血管内皮细胞生长因子受体2抑制肉瘤微环境血管生成

目的:观察艾灸对肉瘤微环境中成纤维细胞生长因子受体1(FGFR1)和血管内皮细胞生长因子受体2(VEGFR2)的影响,探讨艾灸防治肉瘤的作用机制.方法:将接种S180肉瘤细胞形成移植瘤的C57BL/6J小鼠分为模型组、顺铂组和艾灸组,每组10只.艾灸组直接灸移植瘤,距离3 cm,10 min/次,每日1次,治疗14 d...     

Systematic analysis of post-treatment soft-tissue edema and seroma on MRI in 177 sarcoma patients

PurposeTo assess post-treatment subcutaneous edema, muscle edema, and seroma in MRI after soft-tissue sarcoma (STS) resection with regard to muscle involvement of STS and therapy.MethodsIn all, 177 patients were included and received 1.5-T MRI follow-up examinations after treatment. Post-treatment changes were classified according to type of therapy (therapy 1-surgery; therapy 2-surgery with radiation therapy) and primary tumor localization in soft tissue (localization 1, subcutaneous tissue; localization 2, muscle involvement).Subcutaneous and muscle edema were divided into three grades: grade 0, absence of edema; grade 1, low-to-moderate edema; and grade 2, high-grade edema.ResultsThe mean age of the patients was 55.7 ± 18.2 years and the mean volume of the resected primary STS was 321.5 cm³. After therapy 1 of a sarcoma in localization 1, patients significantly more often showed low-grade subcutaneous tissue edema and an absence of muscle edema (p < 0.001) than high-grade edema. The risk for grade 2 subcutaneous tissue and muscle edema significantly increased with a tumor in localization 2 (RR = 2.58, p = 0.016 and RR = 15, p = 0.0065/RR = 2.05 , p = 0.021, respectively) and after therapy 2 (RR = 15, p = 0.0087 and RR = 2.05, p < 0.0001, respectively). Of the patients with sarcoma in localization 2, 88% developed grade 2 muscle edema after therapy 2; 40% of the patients developed post-treatment seroma. The risk for seroma is significantly higher after surgery and radiation therapy than after surgery alone (p < 0.001).ConclusionHigh-grade postoperative subcutaneous and muscle edema are significantly associated with muscle involvement of primary STS both in patients with and without radiation therapy. The risk for seroma is significantly higher after surgery with additional radiation therapy than after surgery alone.     

Malignant peripheral nerve sheath tumors – Outcomes and prognostic factors based on the reference center experience

IntroductionMalignant peripheral nerve sheath tumor (MPNST) accounts for about 5% of soft tissue sarcomas. It can occur as sporadic diseases or can be associated with type 1 neurofibromatosis. MPNST is usually associated with poor prognosis, mostly due to their aggressive behavior, high metastatic potential, and resistance to chemotherapy. Our study aimed to determine treatment outcomes and associated prognostic factors in a large cohort of patients with MPNSTs treated at the reference sarcoma center.Methods239 consecutive patients (114 women and 125 men) diagnosed with MPNST between March 1998 and March 2018 who were treated with surgery with curative intent in the reference sarcoma center were included in the retrospective analysis.ResultsThe mean age at diagnosis was 51 years (range 15–86). 28 (11.7%) patients had neurofibromatosis type 1 associated tumors (NF1 positive). Median OS was 126.5 months and 5-year survival rate was 61.9% in the group treated with curative intent. Median DFS, LRFS and DMFS were 91.6, 126.5 and 126.5 months, respectively. We identified tumor size, high tumor grade and positive surgical margins as independent negative predictors of DFS, LRFS, DMFS and OS.ConclusionsHigh-quality surgery remains a gold standard of MPNST treatment. High grade, size and quality of surgery are significant independent prognostic factors for overall survival. There is an unmet need for improvement, especially regarding the perioperative treatment and treatment of metastatic disease. Future studies on the biology of MPNST would lead to the development of novel treatment options and improvement of treatment outcomes.     

Kaposi肉瘤治疗的研究进展

Kaposi肉瘤的治疗应依据不同临床类型,制定个体化方案.常用治疗方法有电化学疗法、放疗、物理治疗(如硝酸银、液氮冷冻、弹力长筒袜)和皮损内注射等,适用于经典型Kaposi肉瘤及其他类型Kaposi肉瘤的皮肤损害.常用治疗药物有足叶乙甙、紫杉醇、阿霉素、雷帕霉素、吉西他滨等,适用于各型Kaposi肉瘤.Kaposi肉瘤的治疗尚缺乏标准化指南,以上治疗远期疗效欠佳.目前,新治疗靶标的研究主要集中在阻止成瘤过程、炎症调控、阻止病毒复制、细胞周期的调控等方面.     

非白血病性髓系肉瘤1例报告并文献复习

目的：分析非白血病性髓系肉瘤（NMS）的临床特征包括疾病的发病率、诊断、预后和治疗策略。方法：对我院近期确诊的1例牙龈NMS和NMS的文献资料进行复习和分析。结果：淋巴结、皮肤、泌尿生殖系统和消化系统是NMS常见的髓外好发部位,本例发生于牙龈,为少见病例;患者初诊时易被误诊为恶性淋巴瘤。NMS肿瘤细胞形态具有髓系细胞不同阶段分化特点,免疫组化示MPO、CD43和CD68等阳性;治疗首选蒽环类联合阿糖胞苷为基础的化疗。根据髓象与耐受情况加大阿糖胞苷的剂量,取得较好疗效。结论：为减少原发性NMS向ANLL转化并延长生存期,关键在于早期确诊和早期采用ANLL方案全身化疗。