Localized peripancreatic plasma cell Castleman disease

Castleman disease (CD) is a rare, benign, and usually systemic lymphoproliferative disorder. Unicentric Castleman disease of the pancreas is extremely rare, with only less than 10 cases described in the literature. We describe a case of an isolated peripancreatic localization of a plasma cell-type Castleman disease, its clinical presentation, the diagnostic evaluation, and the cure of disease by surgical excision.     

Surgical outcome of superficial and deep Castleman disease

BACKGROUND: Castleman disease is a rare lymphoproliferative disease of low malignant potential occurring in two forms, unicentric and multicentric. Surgery, chemotherapy, immunotherapy and radiation therapy have all been used to manage the disease. In this study, we evaluate whether the site of the lesions, that is, superficial or deep, influences the surgical outcome. METHODS: We retrospectively reviewed the records of 20 patients operated on for Castleman disease from 1994 to 2003, of whom 11 patients had superficial disease and 9 had deep lesions. The end-points of this study were survival and recurrence. RESULTS: Of the 20 patients, 19 had unicentric (cervical in 8, mediastinal in 5, retroperitoneal in 2, axillary in 2, hepatic in 1, and mesenteric in 1) and 1 had multicentric Castleman disease. Among 19 patients who had complete resection (18 with unicentric and 1 with multicentric disease), there has been no evidence of recurrence. CONCLUSION: Whether Castleman disease is superficial or deep has no effect on surgical outcome as long as resection is complete.     

Preoperative embolization as an adjunct to the operative management of mediastinal Castleman disease

Castleman disease usually presents in children as a localized mass with prominent feeding vessels. The mainstay of treatment of Castleman disease is surgical resection; historically, resection is associated with excessive blood loss. These tumors are well known to have large feeding vessels and, thus, are amenable to preoperative arteriography with embolization. The authors present a case of Castleman disease treated with preoperative embolization as an adjunct to operative management.     

Castleman disease of the pararenal retroperitoneum: Report of a case

(Received for publication on July 10, 1997; accepted on May 15, 1998)     

腹部Castleman病的临床诊断和治疗

目的 总结腹部Castleman病的诊断和治疗经验.方法 回顾性分析1985年6月至2009年3月北京协和医院收治的17例腹部Castleman病患者的临床资料.11例患者无任何症状,2例表现为腹部包块,2例表现为口腔溃疡、皮疹,1例表现为浮肿、气短,1例表现为上腹部隐痛、恶心、呕吐.2例行X线检查,17例行超声检查,13例行CT检查.结果 4例术前经CT检查诊断为Castleman病,其余13例均未明确诊断.14例局灶型患者经手术完整切除肿瘤,3例多中心型患者行部分切除或活组织检查.所有患者经病理检查确诊,其中14例透明血管型患者的免疫组织化学检查结果为CD3、CD20、CD21、CD34阳性;3例浆细胞型患者的免疫组织化学检查结果为CD3、CD68、PCNA阳性.17例患者中1例失访,16例随访3～12个月无转移和复发.结论 腹部Castleman病临床表现无特异性,术前诊断困难.局灶型患者应尽早行手术完整切除,预后良好;多中心型难以完整切除,可于术后进行化疗,预后较好.     

Mesenteric Castleman disease

Castleman disease is a rare benign lymph node hyperplasia usually presenting as an asymptomatic mediastinal mass in children. It can present at any extrathoracic site where there is lymphoid tissue. The sites include retroperitoneum, mesentery, axilla, and pelvis. Mesenteric Castleman disease is very rare in children.     

Castleman病肾损害的临床病理分析

目的 探讨Castleman病肾损害的临床病理特点。 方法 对10例Castleman病合并肾损害患者的临床病理资料进行分析。全部病例均接受了淋巴结和肾组织的病理检查,肾活检组织分别进行了光镜、免疫荧光和电镜检查。 结果 10例均为男性,平均年龄（49±14）岁。多数患者有水肿;全部患者均有蛋白尿,尿蛋白量（24 h）为（2.79±3.56）g,其中1例为肾病综合征（NS）;8例有血尿;6例并发急性肾功能不全;4例有高血压。多数有发热、乏力、纳差、体质量下降等。其他异常有贫血、血小板减少、浆膜腔积液、红细胞沉降率增块、高γ球蛋白血症、补体降低、C反应蛋白（CRP）升高、肝脾肿大、甲状腺功能低下等。2例符合POEMS综合征,1例有干燥综合征。10例均表现全身多处淋巴结肿大,以颈部、腋窝及腹股沟淋巴结肿大最常见。淋巴结活检病理类型分别为浆细胞型4例,透明血管型3例,混合型3例。肾活检病理诊断分别为血栓性微血管病5例、新月体性肾小球肾炎2例、肾淀粉样变1例、肾小球微小病变1例、慢性肾小管间质肾病1例。所有病例经过免疫抑制剂或COP方案治疗,病情均明显缓解,淋巴结缩小,蛋白尿减少或转阴,大部分病例肾功能恢复正常。 结论 Castleman病肾损害的临床和病理表现具有多样性,急性肾功能不全发生率较高,常并发全身多系统损害,肾脏病理以血栓性微血管病较多见。对于伴有全身多系统异常的肾脏病患者,有必要进行淋巴结影像学检查和淋巴结活检。     

Complete resection of unicentric Castleman disease in the superior mediastinum: A case report

IntroductionCastleman disease (CD) is a rare benign lymphoproliferative disorder characterized by benign lymph node hyperplasia in a single site (unicentric CD [UCD]) or in multiple sites (multicentric CD [MCD]). Patients with UCD are usually asymptomatic; however, those with MCD usually develop fever, weight loss, and peripheral lymphadenopathy.Case presentationWe describe a case of surgically resected UCD in the superior mediastinum in which the involved lymph node was surrounded by important vessels and trachea, necessitating a median sternotomy for complete resection of the tumor.DiscussionPreoperative diagnosis of UCD in the thorax is very difficult, and surgical resection or excision is necessary. Complete resection is recommended for UCD because it is curative and has a 5-year survival rate of 100%.ConcludionUCD should be included in the differential diagnosis of asymptomatic mediastinal tumors. Surgical resection or excision is preferred to ensure an accurate diagnosis and appropriate treatment if malignant disease cannot otherwise be ruled out.     

Castleman disease: an unusual diagnosis of a portal mass in an 8-year-old boy

An 8-year-old boy presented with a mass located in the portal hilum and hepatosplenomegaly, and the presumed initial diagnosis was lymphoma. The pathology result was Castleman disease of hyaline vascular type. Castleman disease is an unusual diagnosis that should be kept in mind in the differential diagnosis of portal masses. In the case of solitary lesions, total excision can be curative.     

肾上腺区Castleman肿瘤1例报告及文献复习

目的:探讨肾上腺区Castleman肿瘤的临床特点及诊治方法.方法:分析肾上腺区域Castleman肿瘤患者的临床资料,临床表现为口腔和会阴溃疡、全身皮疹,皮肤活检病理示副肿瘤性天疱疮(PNP).CT发现右肾上腺区包膜完整的肿物大小约10 cm×9 cm,行肿瘤切除术.并结合文献复习讨论15例Castleman肿瘤患者的临床资料.结果:术中探查见右肾上腺区域肿瘤,病理诊断右肾上腺区域巨大淋巴结增生,透明血管型,伴局灶钙化.术后随访半年,恢复良好,未见肿瘤复发,口腔及外生殖器溃疡好转,皮疹消退.天疱疮抗体滴度下降.结论:Castleman病临床上少见,表现为不明原因的淋巴结肿大.肿块可发生在淋巴组织的任何部位,肾上腺区域罕见.手术切除肿瘤为首选治疗.     

Localized pancreatic Castleman disease presenting with extrahepatic dilatation of bile ducts: A case report and review of published cases

Introduction: Castleman disease (CD) is a rare polyclonal lymphoproliferative disorder of unknown etiology, which usually develops in the mediastinum. It can also occur in the cervical, retroperitoneal and axillary regions. Localized pancreatic CD is quite rare [1].Presentation of case: The authors herein present a case of a 34 years old female that was diagnosed during a symptomatic cholelithiasis evaluation. During the evaluation, an abdominal ultrasonography revealed a tumor at the head of the pancreas, which went on to generate a dilatation of the extrahepatic bile ducts. This finding was confirmed by abdominal magnetic resonance imaging (MRI). Subsequently, the patient underwent a laparotomy, where a capsulated tumor was found at the head of the pancreas with well-defined margins. The decision was made for tumor excision. The histopathology and immunohistochemistry established CD, hyaline vascular variation.Discussion: The authors of the present paper also performed a literature review concerning Pancreatic CD, where there were found only 33 cases until the time of the writing of this paper, and we have subsequently carried out a retrospective analysis of all cases.In a patient with atypical images, there might be a benefit from a preoperative diagnosis of CD, by using immunohistochemistry analysis in an image guided biopsy. Thus, avoiding unnecessary procedures and surgeries.Conclusion: Localized pancreatic CD is a very rare condition with good prognosis, but it can mimic many common diseases, such as gastrointestinal stromal tumor (GIST), pancreatic neuroendocrine tumor or pancreatic adenocarcinoma.     

腹膜后Castleman病（附二例报告及文献复习）

目的　分析和归纳腹膜后Castleman病的临床特征 ,提高对该病的认识。方法　报告 2例本院收治并经术后病理证实的后腹膜Castleman病的临床资料 ,结合有关文献进行综合分析。结果　 1例单中心Castleman病经完整手术切除治愈 ;另 1例多中心Castleman病经部分手术切除随访至今无异常。结论　本病病因不明 ,可能与感染和自身免疫等有关 ,对单中心Castleman病应力争完整手术切除 ,而对多中心Castleman病则因采取包括手术在内的综合治疗     

Characteristics and survival for HIV-associated multicentric Castleman disease in Malawi

Introduction

Clinical reports of multicentric Castleman disease (MCD) from sub-Saharan Africa (SSA) are scarce despite high prevalence of HIV and Kaposi sarcoma-associated herpesvirus (KSHV). Our objective is to describe characteristics and survival for HIV-associated MCD patients in Malawi. To our knowledge, this is the first HIV-associated MCD case series from the region.

Methods

We describe HIV-positive patients with MCD in Lilongwe, and compare them to HIV-associated lymph node Kaposi sarcoma (KS) and non-Hodgkin lymphoma (NHL) patients treated at our centre. All patients were enrolled into a prospective longitudinal cohort study at a national teaching hospital and cancer referral centre serving half of Malawi''s 16 million people. We included adult patients≥18 years of age with HIV-associated MCD (n=6), lymph node KS (n=5) or NHL (n=31) enrolled between 1 June 2013 and 31 January 2015.

Results and discussion

MCD patients had a median age of 42.4 years (range 37.2–51.8). All had diffuse lymphadenopathy and five had hepatosplenomegaly. Concurrent KS was present for one MCD patient, and four had performance status ≥3. MCD patients had lower median haemoglobin (6.4 g/dL, range 3.6–9.3) than KS (11.0 g/dL, range 9.1–12.0, p=0.011) or NHL (11.2 g/dL, range 4.5–15.1, p=0.0007). Median serum albumin was also lower for MCD (2.1 g/dL, range 1.7–3.2) than KS (3.7 g/dL, range 3.2–3.9, p=0.013) or NHL (3.4 g/dL, range 1.8–4.8, p=0.003). All six MCD patients were on antiretroviral therapy (ART) with median CD4 count 208 cells/µL (range 108–1146), and all with HIV RNA <400 copies/mL. Most KS and NHL patients were also on ART, although ART duration was longer for MCD (56.4 months, range 18.2–105.3) than KS (14.2 months, range 6.8–21.9, p=0.039) or NHL (13.8 months, range 0.2–98.8, p=0.017). Survival was poorer for MCD patients than lymph node KS or NHL.

Conclusions

HIV-associated MCD occurs in Malawi, is diagnosed late and is associated with high mortality. Improvements in awareness, diagnostic facilities, treatment and supportive care are needed to address this likely under-recognized public health problem in SSA.     

Castleman病65例诊治分析

目的 总结Castleman病的临床特点和治疗手段.方法 回顾2005-2012年连续收治的65例Castleman病患者的临床资料,总结分析局灶型Castleman病和多中心型Castleman病在临床表现、发病部位、实验室检查及治疗上的不同.结果 本组42例局灶型Castleman病多见于中青年,常为单发肿瘤,表现为无症状的局部淋巴结增大,病理分型以透明血管型(33/42,78.6％)为主,本组42例患者发现44枚肿瘤,以腹膜后腔(10/44,22.7％)、腹腔(10/44,22.7％)等深部多见,通过手术治疗可治愈.本组23例多中心型Castleman病多见于中年,表现为多发淋巴结肿大,无局部淋巴结疼痛及表面破溃,常伴有发热(9/23,39.1％)、贫血(9/23,39.1％)、脾大,实验室检查多见异常,病理分型主要为浆细胞型(14/23,60.9％)和昆合型(5/23,21.7％),本组23例患者发现66枚肿瘤,以颈部(16/66,24.2％)和股沟区(12/66,l8.2％)等浅表部位多见,主要采用CHOP化疗方案或手术联合CHOP化疗方案,本组21例患者获得完全或部分缓解,2例因化疗引起肺部感染死亡,能否耐受长期化疗是影响预后的重要因素.结论 局灶型和多中心型Castleman病临床特点不同,治疗方法也有差异.局灶型Castleman病通过手术可达到治愈目的;多中心型Castleman病则主要采用CHOP化疗方案,多数预后较好.     

Diabetic kidney disease in pediatric patients: A current review

In the last decades, a significant increase in the incidence of diabetic kidney disease (DKD) was observed concomitant with rising diabetes mellitus (DM) incidence. Kidney disease associated with DM in children and adolescents is represented by persistent albuminuria, arterial hypertension, progressive decline in estimated glomerular filtration rate to end-stage renal disease and increased cardiovascular and all-cause morbidity and mortality of these conditions. In medical practice, the common and still the “gold standard” marker for prediction and detection of diabetic kidney involvement in pediatric diabetes is represented by microalbuminuria screening even if it has low specificity to detect early stages of DKD. There are some known limitations in albuminuria value as a predictor biomarker for DKD, as not all diabetic children with microalbuminuria or macroalbuminuria will develop end-stage renal disease. As tubular damage occurs before the glomerular injury, tubular biomarkers are superior to the glomerular ones. Therefore, they may serve for early detection of DKD in both type 1 DM and type 2 DM. Conventional and new biomarkers to identify diabetic children and adolescents at risk of renal complications at an early stage as well as renoprotective strategies are necessary to delay the progression of kidney disease to end-stage kidney disease. New biomarkers and therapeutic strategies are discussed as timely diagnosis and therapy are critical in the pediatric diabetic population.     

Chronic kidney disease and inflammation in pediatric patients: from bench to playground

Signs of an activated immune system can be observed already in the early stages of chronic kidney disease (CKD). Markers of a chronically activated immune system are closely linked to several complications of CKD, such as accelerated atherosclerosis, vascular calcification, insulin resistance, increased muscle catabolism, loss of appetite, bone remodeling, and increased peritoneal permeability. Interestingly, all the aforementioned pathological states resemble a state of accelerated ageing and are strongly associated with increased morbidity and mortality in CKD patients. In recent studies, signs of inflammation have been shown as predictors for mortality in dialysis patients, and the role of inflammation as a risk factor for complications of CKD in children has emerged. Although preliminary findings suggest that inflammation is highly prevalent in the pediatric population with CKD, information related pathogenic links and to clinical outcomes is lacking. For the future, it is crucial for investigations to address the mechanisms and complications of inflammation that are manifested in pediatric patients with CKD in all stages. Since early identification and intervention may generate the most efficient strategies for prevention and treatment of cardiovascular disease in CKD patients, the pediatric population deserves special attention in future studies. In this review, we discuss the mechanisms involved in the inflammatory activation and the main causes and consequences of the inflammatory state observed in the CKD patient, with special emphasis on the pediatric population.     

超声诊断肾盂输尿管Castleman病1例

患者男,56岁,因"体检发现右肾盂占位10天"入院。查体:血压110mmHg/79 mmHg,双肾区无叩痛,无肉眼血尿等。超声:右肾集合系统分离约8.6cm×5.3cm,右侧输尿管上段管壁增厚,盂管交界部见3.3cm×0.9cm斑片状中等回声,边     

Cardiovascular complications of pediatric chronic kidney disease

Cardiovascular disease (CVD) mortality is a leading cause of death in adult chronic kidney disease (CKD), with exceptionally high rates in young adults, according to the Task Force on Cardiovascular Disease. Recent data indicate that cardiovascular complications are already present in children with CKD. This review summarizes the current literature on cardiac risk factors, mortality and morbidity in children with CKD.