A rare presentation of unicentric Castleman's disease in the thigh:A case report and review of literature

BACKGROUND Castleman's disease(CD)is a rare lymphoproliferative,emulating both benign and malignant diseases.The diagnosis of CD is formulated upon the combination of clinical and laboratory criteria and ultimately confirmed by histopathological assessment.Due to its rarity,CD presents a challenge in treatment selection,with available options encompassing surgery,chemotherapy,and autologous stem cell transplantation.However,studies suggest that surgical resection of the lesion is the most effective treatment modality,especially for unicentric CD(UCD).CASE SUMMARY Here,we describe the case of a 25-year-old woman who presented with painless left thigh swelling for 10 wk.She had been following a low-fat diet to lose weight and had normal laboratory results.Magnetic resonance imaging revealed a wellcircumscribed,demarcated cystic lesion located in the left inguinal region with eccentrically positioned signal void vascular structures,measuring 4.3 cm×3 cm×3.2 cm,likely of lymphoid origin.The patient underwent surgical resection,and the final histopathology showed a vascular proliferation and hyalinization of the vessel walls,along with atretic germinal centers traversed by penetrating vessels,consistent with CD.The patient was discharged home one day after the procedure in good condition,with a follow-up appointment scheduled in our outpatient clinic.CONCLUSION Although surgical resection is the mainstay for UCD,a multidisciplinary approach is needed due the lack of specific diagnostic features and treatments.

Castleman Disease with Retroperitoneal Invasion of Iliac Vascular Zone: A Case Report of Unicentric Type & Review of the Literature

Castleman Disease is a rare nonneoplastic lymphoproliferative disorder that can be found in any lymph node station with unknown etiology. The current cumulative number of reported cases is minimal. We report a case of a 44-year-old woman with a hard mass in the pelvic retroperitoneal that has been gradually increasing in size for many years. Abdominopelvic MRI scan showed a left retroperitoneal mass and visible calcifications. The patient underwent resection of the left retroperitoneal mass and the pathological diagnosis was Castleman disease of hyaline vascular type.

Multicentric hyaline-vascular Castleman's disease in the retroperitoneum

BACKGROUND:Castleman’s disease is a rare lymphoproliferative disease of unknown cause.Most multicentric cases described have been of the plasma-cell variety.This article presents a case of multicentric hyaline-vascular Castleman’s disease in the retroperitoneum with the ultrasonographic and computed tomography(CT)imaging manifestations. METHODS:During routine physical examination,a mass was detected in the left abdomen of a 53-year-old man with no signs or symptoms.The patient underwent ultrasoundguided aspiration biopsy and operative excision after laboratory examination,ultrasonography,and CT. RESULTS:Ultrasonography demonstrated a dominant hypoechogenic mass with hypervascularity in the retroperitoneum.CT detected a relatively homogenous enhanced lesion and several satellite nodules.After the mass and several lymph nodes were resected, histopathologic examination demonstrated a lymphocytepredominant infiltrate surrounding the germinal centres and extensive capillary proliferation,consistent with the hyaline-vascular type of Castleman’s disease.The patient received postoperative chemotherapy and remained free of recurrence 3 months later. CONCLUSION:Ultrasonography and contrast-enhanced CT can provide a positive differential diagnosis of hyalinevascular Castleman’s disease which is a kind of giant lymph node hyperplasia with hypervascularity.

Idiopathic multicentric Castleman disease with pulmonary and cutaneous lesions treated with tocilizumab:A case report

BACKGROUND Human herpes virus-8(HHV-8)-negative,idiopathic multicentric Castleman disease(iMCD)is a rare and life-threatening disorder driven by proinflammatory cytokines,which is still poorly understood.Pulmonary parenchyma lesion is a rare condition in iMCD,which mainly manifests as lymphocytic interstitial pneumonia and is an indicator of severe iMCD.Cutaneous lesion is also very rare and mainly occurs in Asians.There have been few reports of iMCD patients with both skin and lung parenchyma involvement.CASE SUMMARY We present a Chinese man who complained about a 3-year history of intermittent dry cough and a 2-year history of diffuse reddish-brown maculopapules.Laboratory examination revealed polyclonal hypergammaglobulinemia and hypercytokinemia including interleukin 6.Chest computed tomography revealed small patchy shadows with ground-glass nodules scattered in two lobes and mediastinal lymphadenopathy.The pathological result of the lymph node was consistent with the plasma cell type of Castleman disease.As serum human immunodeficiency virus test and HHV-8 staining of the lymph node were negative,the patient was finally diagnosed with HHV-8 negative i MCD.He was treated with tocilizumab at an intravenous(i.v.)dose of 8 mg/kg every 2 wk combined with methylprednisolone at an i.v.dose of 80 mg/d initially with gradual dose tapering.Partial remission was achieved 9 mo later.CONCLUSION i MCD with lung parenchyma and skin involvement is a rare condition that requires clinicians'attention and awareness for early diagnosis.

特发性多中心型Castleman病伴腹腔积液一例并文献学习

Castleman病是一种以淋巴结肿大为特征的慢性淋巴细胞增殖性疾病,病因和病理机制尚未完全明确,临床表现及影像学特征缺乏特异性,依据肿大淋巴结分布部位和器官受累的严重情况可分为单中心型Castleman病(UCD)与多中心型Castleman病(MCD)。UCD首选外科手术治疗,多数可达到治愈,预后好。MCD治疗难度大、预后差,目前还没有最佳治疗方案。Castleman病合并腹水的治疗目前尚无临床数据可以借鉴,腹腔热灌注化疗(HIPEC)是目前用于治疗恶性胸腹腔积液疾病的有效方法之一。本文报告一例特发性MCD伴有腹腔积液患者进行探索性HIPEC后取得了短期的疗效,并通过文献复习总结该病的诊断及治疗现状。

Endoscopic ultrasonography-guided trucut biopsy for the preoperative diagnosis of peripancreatic castleman's disease: A case report

Castleman＇s disease （CD） of the pancreas/peripancreas is extremely rare. The recently introduced, endoscopic ultrasonography （EUS）-guided trucut biopsy （TCB） is a useful diagnostic rnodality for obtaining tissue samples from peripancreatic lesions. However, its role in diagnosing CD remains unknown. We report a case of localized, peripancreatic, hyaline-vascular CD biopsied using EUS. The pathology results were initially interpreted as an extranodal, marginal-zone B-cell lyrnphoma. However, polyrnerase chain reaction （PCR） study for the IgH gene rearrangement revealed a polyclonal pattern. We also reviewed the relevant literature. To our knowledge, this is the first illustrated report on EUS-TCB findings of CD with its pathology results of EUS-TCB mimicked a B-cell lymphoma.

Retrospective Study of Castleman's Disease:A Report of Fourteen Cases and Review of the Literature

OBJECTIVE To enhance the understanding of Castleman’s disease (CD), and to improve its diagnosis and management. METHODS Clinical features and related information on diagnosis and treatment of 14 cases of CD were retrospectively analyzed and the literature reviewed. RESULTS Based on the clinical classification, localized CD was found in 8 of the 14 cases. Both the results of lymph node biopsy and histopathology indicated they were a hyaline-vascular type. The multicentric type CD was detected in 6 cases, among which 4 were plasma cell type and 2 mixed type based on histopathologic examination. There were a variety of clinical situa-tions in the 14 cases, with a lack of specificity. They were previously misdiag-nosed as other diseases, and final diagnosis depended on a histopathologic examination. The 8 patients with localized CD underwent excision, without recurrence up to now. The 6 patients with multicentric-type CD were treated with glucocorticoids or combined chemotherapy, and all achieved remission. CONCLUSIONS CD has complicated clinical manifestations and is difficult to diagnose. Lymph node biopsy is important for early diagnosis. An optimal curative effect can be achieved with a suitable therapeutic option, based on histopathology and clinical classification.

Unicentric Castleman disease was misdiagnosed as pancreatic mass:A case report

BACKGROUND Castleman’s disease(CD)is a lymphatic proliferative disorder of unknown cause and is rarely seen clinically.It has been divided into unicentric and multicentric types.Unicentric CD(UCD)occurs as a solitary enlarged mass and mediastinal lymph nodes are the most common site.Surgical excision has proven to be curative for UCD.Multicentric CD(MCD)appears as a systemic disease with peripheral lymphadenopathy.MCD had a poor response to surgery and monoclonal antibodies with rituximab have become a research hotspot.CASE SUMMARY A 44-year-old woman presented with a pancreatic mass during routine physical examination.She had no obvious symptoms,such as fever,abdominal pain,abdominal distension,or jaundice.Ultrasound examination indicated a hypoechoic mass between the body of the pancreas,left lobe of the liver and stomach.It had a clear boundary,irregular shape,uneven echo,and no obvious blood flow signals.To clarify the diagnosis,contrast-enhanced ultrasound examination was performed,which showed a benign pancreatic lesion.Neuroendocrine or solid pseudopapillary tumor was a possible diagnosis.The patient underwent further contrast-enhanced computed tomography and contrastenhanced magnetic resonance imaging,which were suggestive of solid pseudopapillary tumor or neuroendocrine tumor.All the examinations failed to give a definitive diagnosis,and the patient underwent surgery.The final pathological and immunohistochemical results showed that the mass was CD.CONCLUSION This case highlights when lymphadenopathy is encountered clinically,CD should be considered and a biopsy should be performed.

Castleman's disease of the spleen

Castleman's disease(CD) is a rare lymphoproliferative disorder of unknown etiology.Clinically,it occurs as a localized(unicentric) disease or as a systemic(multicentric) disease.Unicentric Castleman's disease(UCD) presents as a solitary mass and primarily affects the mediastinal,retroperitoneal,and cervical lymph nodes.In contrast to multicentric CD,which involves peripheral lymphadenopathy and numerous systemic symptoms,UCD is not typically associated withgeneralized symptoms.Three main distinct histologic variants are recognized:hyaline-vascular type,plasma cell type,and mixed type.Extranodal CD is rare.Specifically,UCD exclusively in the spleen is extremely rare,with only 2 cases described in the literature to date.Here,we describe an asymptomatic 75-yearold man with a 5.7 cm × 4.5 cm sized heterogenous enhanced mass located in the spleen.He underwent surgical resection for diagnosis and treatment.A pathologic examination indicated the hyaline-vascular type of CD.In this patient,the preoperative diagnosis was difficult to determine,and therefore,invasive procedures were required.

Unicentric Castleman's disease of the pancreas with massive central calcification

Unicentric Castleman＇s disease of the pancreas is extremely rare, with only six cases described in the worldwide literature. An asymptomatic case of unicentric, hyaline, vascular-type Castleman＇s disease （UCD） localized to the tail of bhe pancreas with central calcification imitating a primary neoplasm of the pancreas is presented. This is the first description of endosonographic and endoscopic retrograde pancreatographic findings of pancreatic UCD. Additionally, computed tomography, histological and serologic findings are reported.

Glucocorticoids combined with tofacitinib in the treatment of Castleman’s disease:A case report

BACKGROUND Castleman’s disease(CD),also known as vascular follicular lymphadenopathy is a rare proliferative disease of lymphoid tissue of unknown etiology that is clinically classified as unicentric CD(UCD) or multicentric CD(MCD) depending on lymph node involvement.At present,idiopathic MCD(iMCD) is treated with interleukin-6 inhibitors,but some patients have poor clinical outcomes.This paper reports on a case of iMCD that achieved a good therapeutic effect after treatment with glucocorticoids combined with tofacitinib.The relevant data are summarized and reported below.CASE SUMMARY This paper reports on a case of MCD in a 49-year-old female with persistent peritoneal effusion as the first manifestation and combined with multiple lymphadenopathies.Lymph node biopsy showed Castleman’s disease-like changes.The ascites subsided after treatment with glucocorticoids and tofacitinib,indicating that the treatment was effective.CONCLUSION The combination of glucocorticoids with tofacitinib is an effective regimen for the treatment of CD.

Castleman病患者的临床特征及预后分析

目的:分析Castleman病的临床特征及预后,以提高Castleman病的诊治水平。方法:回顾性分析甘肃省人民医院自2009年1月至2020年11月经病理活检诊断为Castleman病的29例患者的临床资料。根据临床分型分为两组:单中心型组(UCD,n=20)和多中心型组(MCD,n=9),对其临床表现、实验室检查、治疗方案、病理学检查和随访资料进行统计学分析。结果:UCD组和MCD组平均年龄、男女比例的差异无统计学意义。UCD患者中透明血管型占比80.0%,浆细胞型占比20.0%;MCD患者中透明血管型占比33.3%;浆细胞型占比55.6%,混合型占比11.1%,两组病理分型差异有统计学意义(P=0.039)。UCD患者常表现为无症状的单个淋巴结肿大,临床症状轻微;MCD患者表现为全身多发浅表及深部淋巴结肿大。MCD组乏力、脾大、浆膜腔积液发生率均高于UCD组(P<0.05),同时,贫血、低蛋白血症、血沉升高、血清球蛋白升高、β2-微球蛋白升高发生率均明显高于UCD组(P<0.05)。而两组白细胞计数和血小板计数异常、乳酸脱氢酶升高发生率相比较,差异均没有统计学意义(P>0.05)。20例UCD患者中,13例达CR,1例PR。9例MCD患者中,3例获得CR,4例获得PR。结论:Castleman病需病理检查确诊,UCD患者临床症状轻微,手术治疗效果良好且预后较好;MCD患者临床表现多样化,预后相对较差,需综合治疗。

Liver cirrhosis in a child associated with Castleman's disease: A case report

BACKGROUND Castleman's disease(CD)is a lymphoproliferative disorder.TAFRO syndrome is classified as a variant of CD based on its key clinical manifestations of thrombocytopenia,anasarca(generalized edema and pleural effusion),fever(pyrexia),reticulin fibrosis in the bone marrow and the proliferation of megakaryocytes,and organomegaly(such as hepatosplenomegaly and multiple lymphadenopathies);TAFRO syndrome is mainly reported in Japanese patients.To our knowledge,this is the first pediatric case report detailing a CD-associated disorder progressing to cirrhosis.CASE SUMMARY A 10-year old male patient presented with fever and anemia.Six months before hospitalization,he had remarkable abdominal distention.Subsequently,he visited a clinic for a fever that lasted 5 d.The physical findings were marked hepatosplenomegaly and cervical lymphadenopathy.A blood test revealed leukocytosis,microcytic anemia,aspartate aminotransferase-dominant transaminase elevation,high levels of C-reactive protein,polyclonal hypergammaglobulinemia,and high levels of interleukin-6 and vascular endothelial growth factor.Abdominal contrast computed tomography and magnetic resonance imaging suggested cirrhosis,which was confirmed by liver histology.Histological findings in the enlarged hepatic lymph nodes revealed both hyperplasia and atrophy of lymphoid follicles with some vascular hyperplasia and moderate plasmacytosis between the lymphoid follicles,which is compatible with lymph node histology in TAFRO syndrome.Prednisolone was not effective in reducing the patient’s symptoms;therefore,the patient was prescribed tocilizumab.To date,the patient remains free of fever and continues to receive tocilizumab.CONCLUSION We described the clinicopathological features of TAFRO syndrome to highlight the clinical presentation of this rare disease in a pediatric case.

Unicentric Castleman's disease associated with end stage renal disease caused by amyloidosis

Castleman's disease(CD), also known as angiofolicular lymph node hyperplasia, is a rare heterogenous group of lymphoproliferative disorders. Histologically, it can be classified as hyaline vascular type, plasma cell type, or mixed type. Clinically two different subtypes of the CD are present: Unicentric and multicentric. Unicentric CD is generally asymptomatic and associated with hyaline vascular type, and its diagnoses depend on the localized lymphadenopathy on examination or imaging studies. However, multicentric CD presents with generalized lymphadenopathy and systemic symptoms including malaise, fever, night sweats, weight loss, and it is associated with the plasma cell type and mix type. Herein, we report a patient with unicentric CD of the plasma cell type without systemic symptoms, who developed end stage renal failure caused by amyloidosis 6 years after onset of CD.

Unicentric Castleman's Disease with Cardiovascular Involvement

CASTLEMAN＇S disease（CD）,a rare lymphoproliferative disorder of unknown etiology, was first described in 1956 as a benign mass in the mediastinum. Although CD can present anywhere in the body, 70% of the cases are in the chest along the tracheobronchial tree or hilum of the lung in the middle mediastinum; however, they can also occur in the anterior or posterior compartments. CD is classi-fied as unicentric （UCD） or multicentric （MCD） based on the anatomical distribution, and histologically as hya-line-vascular, plasma cell, or mixed subtypes.1 Although MCD is less common than UCD, it can be rapidly pro-gressive and often fatal.

Cervical Castleman's disease mimicking lymph node metastasis of esophageal carcinoma

Castleman's disease(CD) is an uncommon benign lymphoproliferative disorder of unknown etiology. A rare case of cervical CD diagnosed at lymph node dissection for esophageal carcinoma is reported. An esophageal tumor was identified in a 67-year-old man during a follow-up examination after surgery for oral carcinoma. Esophagoscopy revealed a type 1 tumor in the cervical esophagus. Histology of esophagoscopic biopsies indicated squamous cell carcinoma. Contrastenhanced computed tomography revealed swollen lymph nodes of the right cervical region. No distant metastasis was detected. Esophageal carcinoma, T2N2M0, Stage ⅢA was diagnosed. Neoadjuvant chemotherapy was recommended, but the patient rejected the chemotherapy. The patient underwent laparoscopic-assisted transhiatal esophagectomy. The histopathological diagnosis was moderately differentiated squamous cell carcinoma with pT1bN0M0, Stage ⅠA. On histology, the swollen lymph nodes of the right cervical region revealed CD. The patient's postoperative course was relatively good.

Castleman’s Disease of the Pleura: Case Presentation and Review of Literature

Purpose. Castleman’s Disease is a very rare medical disease and the presence of this condition in the pleura is even rarer and there are only few reported cases. The presence of symptoms is also unusual and has been mostly described in multicentric cases of castleman’s disease instead of solitary lesions (unicentric castleman). We report a case of unicentric pleural castlema’s disease with presence of constitutional symptoms. Methods. A 36-year-old male without any significant past medical history who presented to the emergency due to an incidental large pleural effusion seen in chest radiograph, but referring chronic constitutional symptoms consistent of significant weight loss and chronic cough. Results. Chest computed tomography revealed a mass and several attemps with minimally invase approach failed to identify the lesion. The mass was excised through VATS but due to its significant vascularity, it had to be converted to open thoracotomy. Conclusion. The treatment of unicentric lesions is mainly complete surgical excision and the prognosis is excellent with some chances of recurrence. There are less than one dozen of reported pleural castleman reported in the literature. This case evidence than unicentric lesion may exhibit constitutional symptoms and confirmed than lesions above 5 cm can be removed by VATS but the chances for conversion to an open thoracotomy are high.

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.

CASTLEMAN＇S DISEASE IN THE RETROPERITONEAL AREA：REPORT OF 5 CASES AND REVIEW OF THE LITERATURE

Objective To investigate the diagnostic and treatment methods of Castleman's disease(CD).Methods Five CD cases were treated in our department,we presented its clinical characteristics and had a comprehensive review of different reports from other centers.Results We presented five patients who had retroperitoneal mass and postoperative pathological results confirmed all of them had hyaline vascular(HV)type CD.We also discussed CD by reviewing different literatures on its diagnosis,treatment and outcome.Conclusion CD is an atypical lymphoproliferative disorder and a heterogeneous entity that can be either localized or systematic.The etiology and pathogenesis of this entity is still unclear.The imaging findings though are not specific,still can help make differential diagnosis.The hyaline vascular type frequently appears as a benign isolated mediastinal or rarely retroperitoneal mass,which does not recur after curative surgical excision.

CASTLEMAN＇S DISEASE OF THE ABDOMINAL REGION IN ADOLESCENT

Objective To describe the CT features of abdominal Castleman's disease(CD)in adolescent.Methods Abdomen CT of four adolescents ranging in age from 7 years to 17 years diagnosed as abdominal region CD were reviewed.One case was retroperitoneal CD;one case was lesser omentum CD;and the other two cases were mesenteric CD.Three of four cases underwent post enhancement,and one case was only done no contrast CT as the reason of media hypersusceptibility.One case underwent needle biopsy.Three cases were removed by surgical resection.Results Isolated or multiple well-defined homogenous masses with homogenously enhanced after underwent enhancement was the common finding in these four cases.Conclusion Recognition of the CT features and highlights the histological specimen of CD is helpful to diagnose CD.