Unicentric Castleman Disease Research Articles

Castleman Disease In Children: Diagnosis and Treatment

Background: We describe the experiences in diagnosis and results of treatment in pediatric Castleman disease. Method: Serial case reports. Result: From 2016 to 2019, we had 7 cases of pediatric Castleman disease: 3 boys and 4 girl. The median age at diagnosis was 147 months (121-173 months). Clinical manifestations were found in five cases. They were all unicentric Castleman disease (6 abdominal mass, one left infraclavicular mass). All patients were operated with postoperative period uneventful. The median time of postoperative follow up was 22.7 months (11-53 months) with no signs of relapse. Conclusions: Pediatric Castleman disease is a rare benign lymph node hyperplasia, it can be localised or disseminated. Operation is the treatment of choice for localised Castleman disease.

Analysis of the clinicopathologic features as well as diagnosis and treatment of 59 patients with Castleman disease

目的探讨Castleman病的临床表现、病理特点、治疗及预后。方法回顾性分析2011年10月至2019年10月河南省肿瘤医院确诊的59例Castleman病患者的临床与病理资料，根据临床分型分为两组，单中心型组（47例）和多中心型组（12例），对其临床特点、实验室检查结果、治疗方法及预后进行统计学分析。结果单中心型组和多中心型组平均年龄、男女比例的差异无统计学意义，单中心型患者多表现为无症状的单个淋巴结肿大，病理类型以透明血管型为主（83.0%），其中44例接受局灶切除手术，预后良好。多中心型患者多表现为全身多发浅表和（或）深部淋巴结肿大，更易出现B症状、乏力、肝脾肿大、贫血、血清球蛋白升高、低蛋白血症，以浆细胞型为主（50.0%），采用单纯化疗、手术联合化疗或化疗联合放疗等方案，7例患者治疗有效，有效率为58.3%。结论单中心型Castleman病多表现为无症状的单个淋巴结肿大，手术切除效果好，预后佳；多中心型Castleman病临床症状相对复杂，预后较差，最佳治疗方案仍需继续探索。

Unicentric castleman's disease of interpectoral (rotter's) lymph nodes mimicking a breast tumor.

Unicentric castleman's disease of interpectoral (rotter's) lymph nodes mimicking a breast tumor.

Retrospective analysis of 45 cases of localized retroperitoneal Castleman disease from a single center.

Castleman disease (CD) is a rare lymphoproliferative disease characterized by high heterogeneity in clinical manifestation and prognosis. This study aimed to summarize clinical features of localized retroperitoneal CD and our experiences to improve the diagnosis and treatment of this disease. Clinical data of 45 patients with localized retroperitoneal CD were retrospectively analyzed. The differences in clinical features between groups with and without paraneoplastic pemphigus (PNP) were compared. Survival was analyzed between groups depending on whether complicating with PNP, bronchiolitis obliterans (BO), gender, age and uni-centric CD (UCD)/multi-centric CD (MCD), respectively. Significant differences were observed between patient groups in the prevalence of retroperitoneal CD with PNP complicated with BO (P=0.010), the constituent ratios of initial symptoms (P<0.001) and the duration from appearance of the initial symptoms to being diagnosed (P=0.009). Among 45 cases, 43 tumors had clear margins and intact envelops and were completely resected, 40 patients were cured or significantly relieved, 3 patients were not significantly relieved, 2 patients received palliative surgical therapy and eventually relapsed and died after surgery. There were significant differences in the survival rate between groups depending on complication with BO, gender and age (≤40 and >40 years) (all P<0.05). Prompt and complete removal of the retroperitoneal CD tumor is critical to the management of this disease, as palliative resection tends to cause relapse and lead to a poor prognosis. Retroperitoneal CD patients with PNP may develop complications from BO leading to death. Complication with PNP, complication with BO, male gender and age ≥40 years were identified as prognostic risk factors for patients with localized retroperitoneal CD.

Castleman disease spectrum.

8548 Background: Castleman disease (CD) describes a group of lymphoproliferative disorders that share characteristic histopathology. Unicentric CD (UCD) and idiopathic multicentric CD (iMCD) are differentiated by the number of enlarged lymph node (LN) regions: UCD involves 1 region and iMCD involves &gt; 1 region. UCD typically has mild or no symptoms whereas iMCD requires abnormal labs and symptoms for diagnosis and can progress to life-threatening multi-organ failure. Review of an international natural history registry of CD revealed patients across a broad spectrum with regards to number of enlarged LN regions and disease severity. We hypothesize that there is a positive correlation between disease activity and the number of enlarged LNs and that the spectrum of CD is more complex than a binary UCD-iMCD dichotomy. Methods: Herein, enrolled UCD and iMCD patients whose diagnosis was confirmed by an expert-panel were selected for analysis (N = 81). A standardized disease activity score (scale 0-1) was computed for each patient using available diagnostic values of C-reactive protein, hemoglobin, and albumin (CHA score). Results: We looked at the association between number of enlarged LNs and CHA and found a significant positive correlation (R = 0.65, p &lt; 0.0001). Given this, we divided the cohort into groups of mild, moderate, and extensive lymphadenopathy according to the number of regions of enlarged LNs at the time of diagnosis: group 1 (1 enlarged LN region); group 2 (2-4 enlarged LN regions); and group 3 (≥5 enlarged LN regions). We identified 20 patients in group 1, 19 in group 2, and 42 in group 3 with no statistical differences in sex, race, or age at diagnosis. Histopathological subtype differed significantly among groups. Group 1 was 89% hyaline vascular (HV)/ hypervascular (HpV) and 11% mixed (Mx); group 2 was 74% HV/HpV, 21% Mx, and 5% plasmacytic (Pl); and group 3 was 64% HV/HpV, 32% Mx, and 5% Pl. We then looked at CHA score in these groups and found that group 3 patients have a significantly greater CHA score (median [IQR]: 0.46 [0.49]) than both group 2 (0.08 [0.14]) and group 1 (0.0 [0.10]) (adjusted p &lt; 0.001 for both) while there was no difference between groups 1 and 2. Conclusions: These results suggest that disease severity is positively associated with the number of enlarged LNs. The different proportions of histopathological subtypes between the three groups could indicate different pathologic mechanisms are involved. Further work is needed to determine if patients with a few enlarged LNs exhibit disease more closely to UCD or iMCD and to understand long-term outcomes for these patients.

Ultrasound and radiological features of abdominal unicentric castleman's disease: A case series study.

This study aimed to improve the diagnostic accuracy of abdominal unicentric Castleman's disease (UCD) by retrospectively summarizes the relatively specific imaging features of UCD.This study retrospectively collected fifteen patients with abdominal UCD confirmed by pathology. All patients were underwent ultrasound (US), computed tomography (CT), or magnetic resonance imaging (MRI) examination. The imaging findings of UCDs were analyzed by senior radiologists.Fifteen patients included 7 males and 8 females, aged 30 to 68 years old, with an average age of 51.73 ± 13.57. In the 15 cases, 7 were located around the mesentery, 4 were located in the retroperitoneal space, and 4 in the liver. Fifteen cases contained solid masses, of which 13 had clear margins and 2 had blurred margins. The size of the mass ranged from 1.5 to 14.2 cm, with an average of 6.49 ± 4.16 cm. US showed that 9 lesions were presented with hypo-echogenicity while 5 lesions presented with hyper-echogenicity spots. Unenhanced CT showed that the lesions were comprised of soft tissue while calcified lesions were found in 10 of the cases (66.67%, 10/15). T1-weighted imaging (T1WI) suggested the lesions as iso/hypo-signal, and mildly hyper-signal on T2-weighted imaging (T2WI). Diffusion-weighted imaging (DWI) showed different degrees of hyper-signal. Contrast-enhanced US and CT/MRI showed obvious enhancement at the arterial phase in 12 cases (85.71%, 12/14), most of which (50%, 7/14) showed continuous enhancement at the delayed phase. Feeding vessel could be seen within, or around the lesion in 5 cases (35.71%, 5/14).The study suggests that abdominal UCD commonly manifests as well-defined, homogeneous, solid, and hypervascular masses. Calcification and the presence of feeding vessel in the tumors are relatively specific features of abdominal UCD.

Unicentric Castleman disease and lymphoepithelial cyst of the parotid: A case report and review of the literature

AbstractObjectivesPresent the first reported case of unicentric Castleman disease and lymphoepithelial cyst of the parotid in an HIV‐negative adult.MethodsRetrospective case presentation.ResultsCastleman disease is an uncommon disorder of benign hypervascular lymphoid hyperplasia with an estimated incidence rate of 15.9‐19.1 cases per million person‐years (Leukemia &amp; Lymphoma, 56, 2015: 1252). Parotid involvement is extremely rare, with less than 30 cases reported in the literature (Case Reports in Otolaryngology, 2015, 2015: 1). Benign lymphoepithelial cysts (BLECs) are another rare parotid lesion presenting most commonly in HIV‐positive patients. Here we present the case of a 42‐year‐old HIV‐negative male who presented with 3 cm parotid mass. The patient underwent superficial parotidectomy with final pathology demonstrating BLEC with the hyaline‐vascular subtype of Castleman disease. The patient has had no recurrence of symptoms in a 3‐month follow‐up period.ConclusionsThe practitioner should consider unicentric Castleman disease and BLEC in HIV‐negative patients presenting with a new head and neck mass.

Idiopathic multicentric Castleman disease: bringing order to chaos

Idiopathic multicentric Castleman disease is a rare lymphoproliferative disorder characterised by lymphadenopathy and systemic symptoms, in some cases leading to organ failure. The condition must be distinguished from unicentric Castleman disease, which is generally curable with local therapy, and HHV-8-associated Castleman disease, for which an effective treatment approach has been defined. 1 Fajgenbaum DC Uldrick TS Bagg A et al. International, evidence-based consensus diagnostic criteria for HHV-8-negative/idiopathic multicentric Castleman disease. Blood. 2017; 129: 1646-1657 Crossref PubMed Scopus (171) Google Scholar , 2 Bower M How I treat HIV-associated multicentric Castleman disease. Blood. 2010; 116: 4415-4421 Crossref PubMed Scopus (87) Google Scholar , 3 Fajgenbaum DC Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease. Blood. 2018; 132: 2323-2330 Crossref PubMed Scopus (26) Google Scholar A hallmark of idiopathic multicentric Castleman disease is hypersecretion of cytokines, particularly IL6, the triggering factors for which might be multiple, leading to a common pathway of cytokine derangement. 3 Fajgenbaum DC Novel insights and therapeutic approaches in idiopathic multicentric Castleman disease. Blood. 2018; 132: 2323-2330 Crossref PubMed Scopus (26) Google Scholar Although lymphocytes are non-clonal, the clinical spectrum ranges from non-severe to life-threatening. Long-term safety of siltuximab in patients with idiopathic multicentric Castleman disease: a prespecified, open-label, extension analysis of two trialsThese results show that siltuximab is well tolerated long term and provides important evidence for the feasibility of the life-long use required by patients with idiopathic multicentric Castleman disease. Full-Text PDF

Clinical analysis of 30 cases of Castleman disease with different types of thoracic involvement

目的总结不同类型胸部受累Castleman病（CD）的临床、影像学、病理及诊治特征，以提高临床医师对CD的认识。方法回顾性分析广州医科大学附属第一医院自2009年6月至2019年5月收治入院的胸部受累CD患者30例，将其分为闭塞性细支气管炎（BO）组、不伴BO的单中心型Castleman病（UCD）组及不伴BO的多中心型Castleman病（MCD）组，分析其临床资料并总结其特征。结果30例患者中，5例（16.7%）纳入BO组，18例（60.0%）纳入不伴BO的UCD组，7例（23.3%）纳入不伴BO的MCD组。不伴BO的MCD组中位年龄明显大于BO组[（49.29±5.39）岁对（27.20±3.76）岁，P=0.005]和不伴BO的UCD组[（49.29±5.39）岁对（37.17±2.87）岁，P=0.034]。胸部症状在BO组（100%）和不伴BO的MCD组（71.4%）多见，而不伴BO的UCD组无胸部症状。贫血及IgG增高仅在不伴BO的MCD组出现（发生率均为57.1%），红细胞沉降率增快及低氧血症在BO组（发生率分别为40.0%和60.0%）及不伴BO的MCD组（发生率分别为57.1%和28.6%）均可出现。BO组患者的肺功能均表现为极重度混合性通气功能障碍。胸部CT所示肺实质受累率：BO组为100%；不伴BO的MCD组为57.1%，表现为双肺弥漫性病变；不伴BO的UCD组为11.1%，表现为孤立性肺结节。不伴BO的MCD组的淋巴结短径明显小于BO组[（1.83±0.51）cm对（4.73±1.63）cm，P=0.006]和不伴BO的UCD组[（1.83±0.51）cm对（3.62±0.26）cm，P=0.011]。BO组病理类型均为透明血管型（100%），不伴BO的UCD组88.9%为透明血管型，而不伴BO的MCD组以浆细胞型为主（57.1%）。BO组患者均出现口腔溃疡，在肿物切除术后予免疫调节剂治疗，溃疡症状缓解，但肺部症状仍进行性加重。不伴BO的UCD组主要治疗方式为胸腔镜下肿物切除，不伴BO的MCD组主要治疗方式为化疗、免疫调节剂、靶向治疗等。结论三组患者的年龄、临床症状、实验室检查、肺功能、影像学表现、病理类型、治疗及预后均有所不同，此分类能促进临床医师对本病的认识。

Castleman Disease in Children from Histopathology to Therapy

Background: Castleman disease (CD) describes a group of rare lymphoproliferative disorder with characteristic histopathology. It presents with heterogeneous clinical features whether unicentric (UC) or multicentric disease (MCD). The aim of the work was to describe clinic-pathological characteristics, management and outcome of different types of castleman disease in CCHE. It is a retrospective study, all children with (CD) treated from July 2007 till end of 2017 were analyzed as regard diagnosis, management and outcome. The results showed that, twelve patients with a median age of 11.5 years (ranging from 4 - to 17 years) were enrolled. Eight of them (66.6%) were males and 4 (33.3%) were females (33.3%). Histopathology was either hyaline vascular in 8 patients (66.6%) or plasma cell variant in 4 patients (33.3%). Nine patients (75%) had uni-centric disease (UC) with lymphadenopathy. Three patients (25%) were multi-centric disease (MCD); out of them one patient had immune bi-cytopenia, small intestinal thickening and splenomegaly and another one had extensive pulmonary involvement and respiratory distress, while the third patient had pleural effusion and ascites. HIV antibody was negative for all patients. Regarding the three MCD patients, IL-6 was normal in one patient, and extremely high in second one, while it hasn’t been done for the third patient. All UC patients underwent surgical excision and are alive with no active disease till now. All MCD patients received systemic steroids as initial therapy, followed by R- CHOP (Rituximab-cyclophosphamide-doxorubicin-vincristine-prednisone) as salvage therapy, except for one patient with pulmonary involvement who didn’t achieved clinical response and received single agent Rituximab with Anti IL-6. Unfortunately this patient is still having uncontrolled systemic manifestation, while the other two patients showed partial response. In conclusion, Uni-centric castleman disease is a localized surgically cured disease. MCD treatment remains challenging, and the outcome is controversial, so Uniform treatment guidelines are mandatory.

Unicentric castleman disease in the differential diagnosis of a paravesical mass: A case report

Castleman disease is a rare condition with variable forms of clinical presentation. Diagnosis is therefore difficult and the ideal management of the disease has yet to be defined. Prognosis varies as a function of histologic type. Treatment can range from curative surgery for the unicentric form of the disease to a combination of treatments for the multicentric form. This paper describes an unusual case of unicentric Castleman disease in which the differential diagnosis included several gynaecological malignancies. The lesion was resected, with histopathology and immunohistochemistry confirming the diagnosis. The surgical procedure was completed without complications and follow-up of the patient over one year has shown no signs of recurrence. A literature review was performed, and the possible conditions to be included within the differential diagnoses are discussed.

A Case of Unicentric Castleman Disease Diagnosed by FDG-PET/CT

A Case of Unicentric Castleman Disease Diagnosed by FDG-PET/CT

Unicentric Castleman’s Disease Presenting as Retroperitoneal Pelvic Lymph Node in Young Female—Laparoscopic Excision: A Case Report

Unicentric Castleman’s disease (UCD) is localized lymphoproliferative disease and has favourable prognosis. Surgery offers complete cure of unicentric Castleman disease. Radiotherapy offers either complete response or variable clinical response and cure in selected patients. The present case report is of a young unmarried obese girl presented with pain in right iliac fossa for 2 months. On pelvic ultrasound and MRI performed for diagnosis only single enlarged right external iliac lymph node was the positive finding. The CT scan guided biopsy was performed and the histopathological finding was lymphoid lesion-reactive lymphoid hyperplasia is favoured over Hodgkin’s lymphoma. Successful laparoscopic complete excisional surgery was performed for this single enlarged external iliac lymph node. The final histopathological report of the lymph node removed was unicentric Castleman’s disease, hyaline vascular type. This is the 16th such reported case of pelvic retroperitoneal UCD of hyaline vascular type treated by surgical excision, and 3rd case treated by Laparoscopic excisional surgery. By now at the time of reporting this case 4 months of follow up has been completed and patient does not have any symptom nor not show any sign of residual disease locally on transabdominal ultrasound examination and whole-body PET CT scan is also normal.

A Meta-Analysis of Genetic Abnormalities and Next Generation Sequencing of Primary Cases of Castleman Disease

Castleman disease is a rare lymphoproliferative disorder known to represent at least four distinct clinico-pathologic sub-types. Large advancements in our clinical and histopathologic description of these diverse diseases have been made, resulting in subtyping based on degree of nodal involvement (unicentric versus multicentric) and according to viral infection (human herpes virus 8 - HHV8 and Human Immunodeficiency Virus - HIV). However, we continue to lack a foundational understanding of the biologic mechanisms driving at least a subset of patients with these diseases. In recent years, significant molecular and genetic abnormalities that may be associated with this disease have been described; yet we are still unclear as to a unified vision of what loci and genes are involved in at least a subset of cases of Castleman disease. In our study we performed a meta-analysis of all cases (unicentric and multicentric) of Castleman disease described in literature to date, and correlate cytogenetic, molecular and genetic abnormalities with disease subtypes, phenotypes and clinical outcomes. We further performed targeted DNA deep sequencing and RNA-sequencing on a cohort of Castleman Disease cases. Our results demonstrate that genetic abnormalities in the MAPK-pathways, PI3K-pathway and Interleukin signaling are frequent in unicentric Castleman disease, whereas abnormalities in DNA methylation are seen more commonly in idiopathic multicentric Castleman disease, along with abnormalities in MAPK-pathways. We conclude that UCD and iMCD may share similar pathways of MAPK pathogenesis but can also be distinguished by cytokine and other DNA modification pathways that differ. Disclosures Ohgami: Agilent technologies: Other: received support/funding.

Clinicopathological comparison and therapeutic approach to Castleman disease-a case-based review.

Castleman disease (CD) is a rare, B-cell lymphoproliferative disorder affecting lymph nodes and extranodal anatomical locations. Four types of clinical presentations can be distinguished after exclusion of mimics. The first division is into unicentric CD (UCD) and multicentric CD (MCD). MCD is classified further as HHV-8-negative (idiopathic), MCD associated with HHV-8 infection, and POEMS associated MCD. From the histological standpoint, UCD and MCD can be classified as hyaline-vascular (HV), plasma cell (PC), or mixed cellularity (MC) type, with a spectrum of histopathological manifestations. We present clinical and histopathological features and grading of 25 cases of CD classified according to CDCN histological criteria and according to this clinical algorithm, along with outcomes. Here we provide a fine-resolution description of the histological features of CD. We review and discuss the current diagnostic algorithm, grading system, and recently recommended treatment options. In the presented group of 25 patients with CD there were 14 women and 11 men in the age range 15-79 years. UCD was identified in 15 patients and it was most often located in mediastinum. MCD most frequently occurred as generalized lymphadenopathy. The most common type of CD was HV. All patients with UCD underwent complete surgical resection with a positive outcome. Patients with MCD had diagnostic partial surgical excision of the lesions, later followed by different types of treatment (corticosteroids, chemotherapy, radiotherapy, immunomodulatory agents) or 'watch and wait'. In four cases CD was associated with other malignancies (laryngeal cancer, small lymphocytic lymphoma, gallbladder cancer with hepatic metastases, primary squamous cell lung cancer). The accuracy of histopathological examination is essential and re-evaluation has to be performed in case of relapse or unexpected course of CD. Treatment tailored to fit the disease type and severity should follow the novel recommendations, including anti-IL-6 treatment in the case of MCD.

Fiebre de origen desconocido y linfadenopatia en contexto de lupus: presentación de un caso clínico de enfermedad de Castleman

La linfadenopatía ocurre en aproximadamente el 60% de los pacientes con lupus eritematoso sistémico (LES) durante la evolución de la enfermedad. Estas lesiones de ganglios linfáticos son caracterizadas por hiperplasia folicular, necrosis coagulativa, cuerpos de hematoxilina y deposición de ADN dentro de los vasos. Como consecuencia, se subestima al examen histopatológico de los ganglios linfáticos en LES1. Se han notificado casos de linfadenopatía de LES conmorfología de enfermedad de Castleman (EC), que se descubre con el fin de excluir el linfoma maligno. La EC es un trastorno de linfoproliferación atípica que puede presentarse con o sin síntomas sistémicos2-3. Histológicamente, la EC se clasifica como variante hialino-vascular, de células plasmáticas o mixtas. La histología hialino-vascular representa la mayoría de casos de EC unicéntrico (ECU) y el tipo de célula plasmática caracteriza más a la EC multricéntrico (ECM). Se ha demostrado que la ECM tiene asociación con varias enfermedades autoinmunes4-5. En este informe, presentamos una paciente con LES y enfermedad de Crohn con fiebre de origen desconocido (FOD) y linfadenopatías con EMC asociado a infección por virus herpes 8 (HHV-8).

Autoimmunity Due to Unicentric Castleman Disease Cured by Resection of a Hepatic Mass.

Autoimmunity Due to Unicentric Castleman Disease Cured by Resection of a Hepatic Mass.

2571 Unicentric Castleman Disease as a Cause of Ileal Perforation: A Case Report

INTRODUCTION: Castleman disease is describes a group of heterogenous lymphoproliferative disorders that share histopathologic features. It is divided into Unicentric castleman disease (UCD) and Multicentric Castleman disease. We present a case of a patient with one year history of recurrent small bowel obstruction (SBO), which was found to be due to UCD affecting the mesentery. CASE DESCRIPTION/METHODS: A 75 years old gentleman initially presented one year ago with an episode of SBO. He had no history of abdominal or pelvic surgery. CT scan showed a transition point near the terminal ileum and mesenteric/retroperitoneal lymphadenopathy. Patient underwent a colonoscopy which showed a stenotic ileocecal valve with active colitis in the terminal ileum and ascending colon suggestive of resolving intestinal ischemia. This time, patient was admitted with decreased oral intake due to SBO. He was found to have a low albumin of 0.8 and an AKI on CKD. He also had bilateral pleural effusions and new ascites. He was treated conservatively initially, but he developed new abdominal pain with X ray showing free air under the diaphragm concerning for intestinal perforation. Patient underwent emergent laparotomy, where he had a perforated terminal ileum and multiple large fibrotic lymph nodes in the associated mesentery. Pathology showed proliferation of atretic follicles. There were occasional penetrating capillaries and the mantle lymphocytes have an onion-skin pattern. The interfollicular zones showed increased plasma cells, but not prominent vascular proliferation. CD3 and CD20 stains showed an intact immune-architecture. The overall findings showed a mixed pattern of Castleman lymphadenopathy, with some features of both the hyaline vascular and plasma cell variants. He had IgA of 510 mg/dl and an abnormal SPEP with IgG lambda paraprotein of 0.39 g/dl. His hospital course was complicated by septic shock due to bacterial and fungal peritonitis. Patient eventually went into cardiac arrest and died. DISCUSSION: Mixed variant CD is mostly multicentric, however, some patients with UCD reveal features of both hyaline vascular type and plasma cell type. In this case, apart from abdominal pain and recurrent SBO episodes, there were no other complaints. Acute phase reactants, CBC, and serological testing for HIV, HHV-8, Hepatitis B, Hepatitis C, RPR and PPD were all unremarkable. UCD is usually asymptomatic and generally curable using surgical resection but in this case led to severe complication ending in patient's demise.

Clinicopathological Profile of Castleman's Disease in Indian Population: Experience From a Tertiary Care Center.

Castleman's disease (CD), also known as angiofollicular lymph node hyperplasia, a rare, non-malignant chronic lymphoproliferative disease characterized by, uni or multicentric lymphadenopathy. There is limited information about the clinicopathological variations and associations of this entity. A total of 50 cases of CD were retrieved from the archives, between the years 2005-2017. The cases were divided into pediatric (0-18years), young adult (age ≤ 40years) and older adult groups (age > 40years respectively). Detailed clinicopathological correlation was done. The age range was 6-74years. There was a male predominance (M: F-1.6:1). The majority (72%; 36/50) of the patients were adults; 46% young adult and 28% older adult, while only 28% (14/50) were of pediatric. Majority (78%) showed features of unicentric Castleman disease (UCD) while rest 22% presented with multi centric Castleman disease (MCD). Systemic symptoms were more frequent in MCD as compared to UCD cases (p = 0.06). The majority of the cases (40/50; 80%) were of the hyaline vascular type. Two of the cases showed mixed histological feature. Out of 50 patients 29 patient's treatment details are available. Majority unicentric cases were cases surgically excised 14/17 (82.3%). Three patients 3/17 (17.6%) were treated with chemotherapy. In multicentric Castleman's disease group, six of the 12 cases with MCD were managed by chemotherapy. One patient died of progressive disease. Castleman disease has varied clinical presentation and is often associated with other diseases. A high degree of suspicion and careful histological examination is required in order not to miss this entity.

A 62-Year-Old African American Woman With Stable Lymphadenopathy Over 5 Years

Abstract The patient is a 62-year-old African American female with medical history of Plummer Vinson syndrome and CT scan in 2014 showing lymphadenopathy of the porta hepatis. Needle core biopsy of enlarged lymph nodes revealed sheets of polytypic plasma cells with no aberrant B or T cells or granulomas or hyaline-like depositions identified. The findings were suspicious for unicentric Castleman disease. In 2019, the patient presented with chronic fatigue without other systemic symptoms. Contrast CT revealed persistent retroperitoneal lymphadenopathy and calcified granulomas in the liver, spleen, and lung. Excision of portal and celiac nodes showed preserved nodal architecture with mild mantle zone expansion and marked hyaline-like deposition in the follicles. Sheets of mature plasma cells and focally increased vasculatures were seen in the interfollicular areas. Focally frequent nonnecrotizing granulomas were noted. Occasional giant cells and rare asteroid bodies were identified in the granulomas. Immunohistochemistry stain workup revealed normal distribution of the B and T lymphocytes. CD138 stain highlighted marked increased plasma cells and HHV8 was negative. Immunofluorescence microscopy for kappa and lambda did not show monoclonality; IgA was positive in &lt;10% of plasma cells; special stain for Congo red and serum amyloid associated protein (SSA) IHC stain were strongly positive in the follicles. The overall findings are consistent with reactive lymphadenopathy with interfollicular polytypic plasmacytosis, noncaseating granuloma formation, and reactive amyloidosis. The features of mantle zone hyperplasia and polytypic plasmacytosis are suggestive of HHV8-negative/idiopathic unicentric Castleman disease. This case possibly represents an example of concurrent amyloidosis, sarcoidosis, and idiopathic unicentric Castleman disease. Given that SSA was recently reported to have a pathogenic role in granulomatous inflammation of sarcoidosis through sustained inflammatory reaction, the possibility of increased SSA triggering sarcoidoisis in this patient can be considered. However, the exact pathogenesis of these concurrent findings needs further investigation and clarification.