Pelvic Castleman's Disease Presenting as an Adnexal Mass in an Adolescent

BackgroundCastleman disease (CD) is a rare lymphoproliferative disorder that might present as an adnexal mass. We report a case of pelvic CD in an adolescent girl who presented with abdominal pain.CaseA 13-year-old girl presented with severe abdominal pain, nausea, and vomiting, and was found to have a solid adnexal mass. Repeat imaging revealed the mass to be retroperitoneal and in the left pelvic side wall. She underwent surgical removal via an open retroperitoneal approach, and pathology revealed CD, hyaline vascular variant subtype.Summary and ConclusionPelvic CD should be considered in the differential diagnosis for an adnexal mass in a young woman. Surgical planning is critical because of the possibility of extension and mass effect. Most pelvic CD is unicentric, hyaline vascular variant subtype, and does not recur after surgical removal.     

Castleman病研究进展

Castleman病(CD)是一组罕见的淋巴细胞增殖性疾病,其症状、体征和病理特点均与恶性淋巴瘤相似。由于CD发病率极低,缺乏大样本的临床病例、实验室检查、治疗方案及预后因素之间的比较,使CD的临床诊疗遇到了极大的困难。第60届美国血液学会(ASH)年会关于CD的研究有了最新进展,基础研究方面,主要探究了血清蛋白质组学分型与治疗效果的关系、疾病的基因表达和细胞驱动过程、高细胞因子产生的来源等;临床治疗方面,针对疾病活动状态及具体分型进行的个性化治疗为CD患者提供了新的希望。     

Castleman病6例临床病理分析及文献复习

目的 提高对Castleman病的诊断和治疗水平。方法 报告6例Castleman病,进行分析并复习相关文献。结果 男性4例,女性2例,年龄8岁～54岁。临床分型：局灶型2例,多中心型4例。病理学分型：透明血管型5例,浆细胞型1例。局灶型经手术或术后放疗均治愈。多中心型中3例经干扰素或化疗联合激素治疗后病情稳定,1例出现复发。结论 该病诊断主要依靠病理学确定。手术、化疗、抗病毒治疗可控制其发展。     

Castleman病8例临床病理分析

目的探讨Castleman病的临床病理特点。方法通过常规组织病理和免疫组织化学等方法观察8例Castleman病并结合相关文献进行分析。结果 8例Castleman病中男3例,女5例,其中局限型6例,均为玻璃样血管型,多中心型2例,玻璃样血管型及浆细胞型各1例。结论 Castleman病是一种特殊类型的淋巴组织增生性疾病,可发生于任何年龄,该病的诊断和分型主要依靠组织病理学;手术治疗是首选,化疗、放疗及生物治疗等多种方法联合应用,有助于控制本病发展。     

腹膜后局限型Castleman病的CT表现(附2例报道)

目的探讨腹膜后局限型Castleman病的CT表现,进一步提高临床及影像对该病的认识。方法分析该院收治的2例腹膜后局限型Castleman病的CT特点,并结合有关文献进行分析。结果 2例腹膜后局限型Castleman病均为透明血管型,CT平扫与肌肉相比呈等密度;动态增强CT扫描,多数肿块动脉期明显强化,在延迟期均表现为持续强化,强化程度邻近动脉相仿。结论腹膜后Castleman病较为少见,但CT表现具有一定特征性,应提高对该病的认识,避免误诊。     

腹膜后Castleman瘤伴副肿瘤性天疱疮2例

副肿瘤性天疱疮(paraneoplastic pemphigus,PNP)是以黏膜损害(尤其是口腔黏膜广泛糜烂),皮肤出现多形性皮损,且并发肿瘤为特征。Castleman瘤(又称血管滤泡型淋巴组织增生或巨大淋巴结增生症)伴发PNP少见,我院近期收治2例。报告如下。     

4例面颈部Castleman病的临床病理分析

Castleman病（CD）是一种少见的特殊类型的交界性淋巴组织增生性疾病。本文报道了4例面颈部CD,其临 床分型均为局灶型,组织学分型为透明血管型,手术切除后预后较好,同时结合文献对其临床表现、病因、病理、诊断及治疗等进行了文献复习。     

Renal failure in pediatric Castleman disease: Four French cases with thrombotic microangiopathy

Pediatric Castleman disease (CD) is an uncommon and poorly understood disorder of the lymph nodes. Renal failure has not been described in pediatric multicentric CD (MCD). We report four cases, who presented with polyadenopathy, organomegaly, edema and fluid accumulations, high blood pressure, and acute renal failure. In all cases, renal biopsy confirmed diffuse thrombotic microangiopathy. Definitive diagnosis of MCD was made by a biopsy of an affected lymph node located by computer tomography before initiation of corticosteroid therapy. Treatment of CD with corticosteroid therapy and rituximab was rapidly effective without relapse to date.