Castleman Disease of the Kidney in Computed Tomography Urography.

BACKGROUND: Castleman Disease (CD) of the kidney is extremely rare. In this study, we have presented a case of CD of the left kidney and comprehensively described the findings of computed tomography urography. CASE PRESENTATION: The case involved unusual imaging characteristics of the focal central cystic area. CONCLUSION: The small and regular cyst-like structures and the hyperdense mass relative to the renal parenchyma in plain scans might help distinguish the CD of the kidney from other hypervascular tumors.

Unicentric Castleman's Disease Presenting As Back Pain.

Castleman's disease is a rare disorder caused by a polyclonal proliferation of B lymphocytes and plasma cells. Half of all cases of multicentric Castleman's disease are associated with HIV or Kaposi's Sarcoma. Typically, unicentric Castleman's disease presents as an enlarged thoracic lymph node but can present in multiple other body areas, such as the head and neck. This case report presents a rare large extrathoracic mass causing back pain in a 71-year-old man.

Clinical features and surgical treatment of abdominal unicentric Castleman's disease / 中华普通外科杂志

Objective:To summarize the clinical manifestations, diagnosis and surgical treatment of abdominal unicentric Castleman's disease.Methods:The clinical data of abdominal unicentric Castleman's disease cases admitted to the General Surgery Department of the First Affiliated Hospital,Nanjing Medical University from Jan 2009 to Dec 2019 was retrospectively analyzed.Results:A total of 18 patients were included with definite pathological diagnosis. The main complaint was abdominal pain and discomfort (50%, 6/12), dizziness and fatigue (25%, 3/12), multiple rash with oral ulcer (16.7%, 2/12) and weight loss (8.3%, 1/12). All patients received surgical resection. Postoperative pathology showed clear vascular type in 15 patients and plasma cell type in 3 patients. There were no major complications nor mortality.Conclusion:Abdominal unicentric Castleman's disease has diverse clinical manifestations, which was difficult for preoperative diagnosis, postoperative prognosis is satisfactory.

Dysphagia and Dysphonia, a Pairing of Symptoms Caused by an Unusual Pair of Diseases: Castleman's Disease and Myasthenia Gravis.

OBJECTIVES: To describe a case of coincident Castleman's disease and myasthenia gravis that initially presented as rapidly progressive dysphagia and dysphonia and to review the unique pathophysiology of these two uncommon diagnoses. METHODS: Case report and literature review. RESULTS: Castleman's disease, angiofollicular or giant lymph node hyperplasia, is a rare benign lymphoid proliferation. Traditionally, the disease is classified based on histologic and clinical characteristics. Fewer than 10 cases with concurrent myasthenia gravis have been reported. Myasthenia gravis and thymic epithelial tumors are both associated with acetylcholine receptor antibody. While patients with isolated Castleman's disease are usually asymptomatic, those who have concurrent myasthenia gravis and undergo surgical treatment are at increased risk of postoperative myasthenic crisis. Both pre- and postoperative plasmapheresis are suggested to improve muscle strength and prevent severe postoperative complications. CONCLUSIONS: In the setting of multiple cranial neuropathies including velopalatal insufficiency and bilateral ptosis it is important to consider myasthenia gravis. Castleman's disease occurs rarely in conjunction with myasthenia gravis but may increase the risk of myasthenic crisis.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSIONS: 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.

Excellent Outcomes with Surgery or Radiotherapy in the Management of Castleman Disease Including a Case of Oligocentric Disease.

BACKGROUND: Castleman disease (CD) is a rare polyclonal lymphoproliferative disorder of unclear etiology. Standard therapy for unicentric CD is surgical resection. Radiotherapy can be used; however, its efficacy is poorly characterized. PATIENTS AND METHODS: We reviewed patients with histologically confirmed CD undergoing definitive local therapy at our institution between 1990 and 2017. Overall survival was determined from the date of diagnosis. Local progression-free survival and distant failure-free survival were determined from the date of first definitive therapy. The Kaplan-Meier method was used to analyze survival. RESULTS: Forty-four patients (29 female and 15 male) were identified with a median age at diagnosis of 40 years (range, 14-70 years). Thirty-five (80%) patients received surgery alone, 3 (7%) had surgery followed by radiotherapy, and 6 (14%) had radiotherapy alone. Thirty-nine (89%) patients had a single area of involvement, and 3 (7%) patients had limited regional involvement. Two (5%) patients had multicentric CD and received consolidative radiotherapy. The 3-year overall, local progression-free, and distant failure-free survival were 92%, 100%, and 100%, respectively. No distant failures were observed. The median radiation dose was 3960 cGy (range, 3600-5940 cGy) in 22 fractions (range, 18-33 fractions). CONCLUSIONS: Unicentric CD is readily amenable to cure with local therapy. Surgical excision is preferred, but radiation appears to be an effective alternative for patients when surgery is high risk or not feasible. Patients with oligo- or multi-centric CD may experience prolonged disease-free survival with consolidative radiotherapy after partial response to systemic therapy.

Clinical features and treatment of 7 Chinese TAFRO syndromes from 96 de novo Castleman diseases: a 10-year retrospective study.

BACKGROUND: Castleman disease (CD) is a rare polyclonal lymphoproliferative disorder with unknown etiology. TAFRO syndrome is now regarded as a specific subtype of CD, and is still a huge challenge for clinicians. METHODS: To clarify the clinical features and management of TAFRO syndrome in China, we retrospectively analyzed 96 patients with HIV-negative CD (52 with unicentric CD and 44 with multicentric CD), who were diagnosed and treated at our center between 2008 and 2017. Specially, we systematically reviewed the 7 TAFRO syndrome cases based on the 2015 criteria proposed by Masaki. RESULTS: Among the 7 cases, there were 3 men and 4 women, and the median age was 53 years. The main symptoms included thrombocytopenia (7/7), anasarca (7/7), fever (4/7), renal dysfunction (7/7), and organomegaly (6/7). One patient was treated with corticosteroid monotherapy, one received RD (Rituximab, dexamethasone), and 5 received CHOP/COP like chemotherapy as first-line treatment, 2 of the 5 combined with Rituximab. Four patients needed hemodialysis or CRRT because of progressive renal failure. The outcome for TAFRO syndrome was significantly worse compared to other types of CD. Although 3 patients improved after early treatment, 4 patients died due to disease progression, and only one patient achieved complete resolution of all the symptoms after changing to lenalidomide based regimen. CONCLUSIONS: This study reveals that TAFRO syndrome is more severe and has more systemic symptoms than other iMCD, most cases need active treatment, and their prognoses are poor. Lenalidomide based regimen may be as a promising new therapy for TAFRO syndrome.

Idiopathic multicentric Castleman's disease, infrequent cause of Lupus-like: case report

Castleman's disease (CD) or angiofollicular lymph node hyperplasia includes a heterogeneous mix of reactive lymphoproliferative processes with well-defined histological features. However, they differ in their localization patterns, clinical expression and etiopathogenesis. There are 4 types, one of them is the multicentric CD that is not associated with any viruses and has re-cently been called idiopathic MCD (iMCD). iMCD is a lymphoproliferative disorder with specific histopathological characteristics, more than one region of affected lymph nodes and absence of infection associated to human herpesvirus 8 and human immunodeficiency virus (HIV). iMCD covers multiple differential diagnoses and might simulate autoimmune diseases such as syste-mic lupus erythematosus. The aim of this article is to report the case of a patient with Castle-man's disease and lupus-like presentation. We present the case of a 38-year-old man without morbid history, who presented lumbago, fever, diaphoresis and asthenia with two months of evolution, associated to bilateral cervical adenopathies. General examinations result negative, antinuclear antibodies at a dilution of 1/640 were positive, and extractable nuclear antigens were positive suggesting moderate Systemic Lupus Erythematosus (SLE) plus secondary Sjö-gren's. Methylprednisolone and Hydroxycloroquine boli were thus initiated. The patient evolved with anasarca, severe anemia, acidosis, polyserositis and multiple mediastinal adenopathies. Immunoglobulin and cyclophosphamide were thus initiated. He later presented fever, throm-bocytopenia and nephrotic syndrome. Biopsy of cervical lymph node reported lymphadenitis with polyclonal plasmacytosis and concentric lymphoid hyperplasia, in agreement with iMCD. Treatment with Rituximab was initiated, which led to the favorable evolution of the patient. iMCD is a systemic inflammatory disease, its presentation corresponds to a constitutional syndrome resulting in a wide differential diagnosis. Every time suspicious adenopathies appear, they must be biopsied since this might lead to a definitive diagnosis

Diagnosis and treatment of abdominal and pelvic Castleman's disease / 中华普通外科杂志

Objective To explore the diagnosis and treatment of Castleman's disease (CD) of the abdomen and pelvis.Methods The clinical data of 51 cases of abdomen and pelvis CD were analyzed retrospectively from Jan 2007 to Oct 2017 in the First Affiliated Hospital of Zhengzhou University.Patients were divided into two groups according to the different clinical classifications:Localized CD (n =42) and Multicentric CD (n =9).Results 32 cases of LCD were asymptomatic (76％),others accompanied by abdominal or waist pain,abdominal distention.The main pathological type was hyaline vascular (93％),which can be cured by surgical treatment (41/42).MCD always accompanied by multiple superficial and deep lymph node enlargement in the whole body.Most of them suffered from anemia,fever,weight loss and dyspnea.CRP increased,hypoproteinemia,globulin increased,neutropenia were found.The main pathological type was plasma cell type (56％).There were no progress in 3 cases,and 6 cases were on remission after operation.Conclusion The diagnosis of abdomen and pelvis CD depends on pathological examination.LCD clinical symptoms are mild and good prognosis after surgery.However,MCD clinical manifestations are complex,and relatively poor prognosis after comprehensive treatment.

Analysis of clinical characteristics and prognosis factors of 71 cases with HIV-negative Castleman's disease: hypoproteinemia is an unfavorable prognostic factor which should be treated appropriately.

PURPOSE: HIV negative Castleman's disease has been reported as a group of poorly understood lymphoproliferative disorder, and we want to explore the clinical feature and prognosis factors of CD. METHODS: We retrospectively collected the clinical information of 71 CD patients without HIV infection diagnosed in the first affiliated hospital of Zhengzhou university. RESULTS: Different clinical classifications, including 35 patients (49.30%) with unicentric Castleman disease and 36 (50.7%) with multicentric Castleman disease, has their specific features compared with each other and unfavorable risk factors calculated by the univariate analysis. As for all of CD patients without HIV infection, there were 7 significant risk factors identified by the results of log-rank test, including clinical complaint, edema (hydrothorax, ascites, pelvic effusion), fatigue, anemia, hypoproteinemia and elevated serum ß2-MG. Then, we created a Cox regression model of these clinical and statistic significant factors which indicated hypoproteinemia was an independent poor prognosis factors of CD in both univariate and multivariate analysis. CONCLUSIONS: Our study emphasized the distinction of clinical characteristics between UCD and MCD and the importance of different poor risk factors of different clinical classifications which may directed more precise and appropriate treatment strategy.

Primary Castleman's disease in the liver: A case report and literature review.

Castleman's disease (CD) is a lymphoproliferative abnormality, also referred to as giant lymph node hyperplasia or follicular lymphoid hyperplasia. The occurrence of CD in the liver is rare. Radiological diagnosis of hepatic CD by computed tomography (CT) and magnetic resonance imaging (MRI) remains difficult. On imaging, hepatic CD is often expressed as a single, well-defined soft tissue lesion, with rare cystic degeneration and focal necrosis. The CD lesions appear as hypervascular with abundant cell proliferation on CT and MRI. Polymorphic calcification is commonly identified in the lesion. However, these findings are non-specific for a definitive radiological diagnosis. We herein present a case of hepatic CD and review its pathological and imaging characteristics. In particular, a strip-like area was observed extending from the nodule, which may be helpful for the diagnosis of CD in the liver.

Impact of siltuximab on patient-related outcomes in multicentric Castleman's disease.

Multicentric Castleman's disease (MCD) is a rare, widespread lymphoproliferative disorder and a life-threatening disease involving hyperactivity of the immune system, excessive proinflammatory cytokine release, immune cell proliferation, and organ system dysfunction. Interleukin-6 (IL-6) is a cytokine that plays a key role in the pathogenesis of MCD, as it is involved in the synthesis of acute-phase reactants and aids in the induction of B-cell proliferation. Siltuximab is an anti-IL-6 chimeric monoclonal antibody that acts as a novel treatment modality to bind to IL-6 with high affinity, thus neutralizing the cytokine bioactivity and inhibiting B-cell proliferation. Clinical trials with siltuximab have shown early clinical promise for patients with MCD for many years, leading to recent US Food and Drug Administration approval as a novel agent for the treatment of MCD. Here, a systematic review was conducted to include 171 cases of MCD patients treated with siltuximab. While traditional treatment methods were able to achieve a 5-year survival rate of only 55%-77%, results of siltuximab treatment demonstrated 5-year survival rates of nearly 96.4% (only 2 deaths reported out of 55 patients with follow-up data). Ultimately, the results from multiple clinical trials have demonstrated that siltuximab is extremely efficacious in alleviating disease symptoms (fatigue, pain, and lymphadenopathy) while simultaneously achieving disease remission, thus extending progression-free survival for years longer than the average 5-year survival rates for MCD.

CT manifestations of lung in Castleman disease / 中国医学影像技术

Objective To explore CT manifestations of the lung in Castleman disease (CD) patients.Methods Totally 14 CD patients with abnormal pulmonary manifestations were enrolled,including 8 multicentric CD (MCD) and 6 unicentric CD (UCD).CT manifestations of the lung were analyzed retrospectively.Results In 3 patients of pulmonary hilar UCD,CT showed circular masses with solitary homogeneous density,obviously or markedly enhanced after contrast media administration.Bronchiolitis obliterans was found in 1 patient of retroperitoneal UCD with paraneoplastic pemphigus.Two patients of mediastinal UCD showed well defined centrilobular nodules in the lung.Eight patients of MCD mainly showed multiple small nodules,and large nodules with diameters of 1-2 cm were found in 2 of them.Four patients showed poorly defined nodules,and the rest 4 showed well-defined nodules.The nodules were mainly demonstrated as small centrilobular nodules,and a few of them located in subpleural regions in 3 patients.Other CT signs included interlobular septal thickening in 4 patients,ground glass opacity in 4 patients,streaks in 4 patients,patchy consolidation in 3 patients,cysts in 2 patients and thickening of the bronchovascular bundles in 1 patient.Conclusion CT manifestations of the lung in CD patients are complex and variable,and might be related to pathological types.

[Unicentric Castleman's disease in cervical back space neck]. / Enfermedad de Castleman unicéntrico en espacio cervical posterior del cuello.

BACKGROUND: Castleman´s disease is a rare, benign condition of lymphoid tissue. There are two clinical types: unicentric and multicentric with three histological variants, hyaline-vascular, plasma celular and mixed. The most common sites of this are mediastinum, adbomen and neck. Magnetic resonance imaging is well suited to depict the characteristics of the masses and their relationship to adjacent structures. The knowledge of this disease and its inclusión in the differential diagnosis from other neck masses will contribute to the therapeutic approach. CLINICAL CASE: A 21 years old female patient with a left neck mass characterized by magnetic resonance as solid, heterogeneous, vascularized lesión, pre dominantly isointensa on T1-weighted images an high signal on T2-weighted images and fat sat that demonstrate moderate enhancement after contraste material administration located in posterior space of the neck with extensión at thoracic outlet displacing structures of carotid, anterior cervical and viceral spaces. She underwent complete surgical resectión of the mass with histopathological diagnosis of hyaline-vascular type of Castleman´s disease. CONCLUSION: Magnetic resonance is well suited to depict characteristic and the extent of mass in the neck contributing to narrow the differential diagnosis. Imaging findings, especially of magnetic resonance are very important to choose the treatment of Castleman´s disease.

INTRODUCCIÓN: la enfermedad de Castleman es una condición benigna poco frecuente que afecta al tejido linfático. Existen dos formas clínicas: unicéntrica y multicéntrica, así como tres variantes histológicas: hialino vascular, de células plasmáticas y mixta. Los sitios más afectados son el mediastino, abdomen y cuello. La resonancia magnética detalla las características de las masas y su relación con las estructuras adyacentes. El conocimiento de esta enfermedad y su inclusión en el diagnóstico diferencial de las masas cervicales contribuirán en la conducta terapéutica. CASO CLÍNICO: paciente femenina de 21 años con masa cervical izquierda caracterizada por resonancia magnética como lesión sólida, heterogénea, vascularizada, predominantemente isointensa en T1, hiperintensa en T2 y saturación grasa, con moderado realce postcontraste, localizada en el espacio cervical posterior y extensión al opérculo torácico que desplaza las estructuras de los espacios: carotídeo, visceral y cervical anterior. Fue sometida a resección quirúrgica completa con diagnóstico histopatológico de enfermedad de Castleman de tipo hialino vascular. CONCLUSIÓN: la resonancia magnética detalla las características y extensión de las masas cervicales contribuyendo a estrechar el diagnóstico diferencial. Los hallazgos de imagen, especialmente de la resonancia magnética son importantes para elegir el tratamiento en la enfermedad de Castleman.

Cardiac arrest caused by rapidly increasing ascites in a patient with TAFRO syndrome: a case report.

Case: Thrombocytopenia, anasarca, fever, renal insufficiency, and organomegaly (TAFRO) syndrome is a newly defined systemic inflammatory disorder with gradual progression of symptoms. A 59-year-old man with fever and ascites of unknown cause developed sudden-onset shock and respiratory failure in the general ward. Cardiac arrest immediately followed. Although he was resuscitated, frequent administration of adrenaline was required to maintain his blood pressure. His circulation was most effectively stabilized by drainage of fluid from his distended abdomen. The volume of discharged ascites reached 4,000 mL at that time, and several liters continued to be discharged for >1 month. The diagnosis of TAFRO syndrome was based on the clinical features and laboratory and histological findings. Outcome: The ascites volume and concentrations of inflammatory parameters decreased with treatment using several immunosuppressive agents. Conclusion: The newly defined TAFRO syndrome may be life-threatening. Patients should be monitored for progression to shock and cardiac arrest, especially those with rapidly increasing ascites.

Rare Location of Castleman's Disease in the Temporal Region: A Case Report Involving a Young Korean Woman and Review of the Literature.

Castleman's disease (CD) is an uncommon benign lymphoproliferative disorder of unknown etiology. Histopathologically, it is divided into three types: hyaline-vascular, plasma cellular, and multicentric CD. The mass usually presents asymptomatically; however, it can cause non-specific symptoms such as fever and fatigue. Although CD can be found wherever lymph nodes are present, 75% of cases are reported in the mediastinum, and occurrence in the head and neck is rare. Herein, we report a rare case of CD presenting as a superficial mass in the temporal region. To the best of our knowledge, this is the first report of temporal CD in Korea involving a young patient.

Linfoma de células grandes B originado en enfermedad de Castleman: reporte de un caso y revisión de la literatura / Large B-cell lymphoma coexisting with Castleman's disease: report of one case

We report a 73-year-old female patient with Castleman's disease coexistent with large B cell type non-Hodgkin's lymphoma in a right axillary lymphadenopathy. An excisional biopsy was performed: microscopically, the lymph node revealed the presence of numerous plasma cells and small lymphoid cells characteristic of Castleman's disease. An analysis of another portion of the specimen revealed lymphoid cells with large abnormal nuclei gathered locally that were CDD 79+, CD 38+ and MUM-1+ as well as positive for Kaposi sarcoma-associated herpesvirus and negative for Epstein Barr virus encoded RNA-1 (EBER).

Diagnostic values of MSCT and MRI for Castleman disease / 医疗卫生装备

Objective To investigate the diagnostic values of multi-slices spiral computed tomography (MSCT) and magnetic resonance imaging (MRI) for Castleman disease.Methods Six Castleman disease patients confirmed pathologically in some hospital from January 2011 to December 2016 had their clinical data and imaging findings by MSCT and MRI analyzed retrospectively,of whom,all the ones underwent examinations by noncontrast and contrast-enhanced CT scans,1 patient went through MRI examination.Results All the patients had the lesions being solitary and focal,of whom,3 female patients aging from 44 to 52 years old had thoracic lesions and the remained 3 male patients aging from 27 to 53 years old had abdominal lesions.MSCT showed round or oval soft tissue masses with homogeneous density and clear boundary in the 6 patients.Contrast-enhanced scan found significantly homogeneous enhancement similar to the adjacent artery in arterial phase and persistent enhancement in delayed phase,and there were feeding artery and draining vein in adjacent area.Volume rendering and maximul intensity projection displayed the feeding artery and draining vein clearly.Conclusion MSCT and MRI contribute to evaluating the blood supply and adjacent anatomy of Castleman disease patient,and thus can provide references for its clinical diagnosis.

Clinical features of intracranial giant lymph node hyperplasia / 肿瘤

Objective: To investigate the clinical features of intracranial Castleman's disease, and to improve the understanding of the disease. Methods: The clinical features of one case of intracranial mixed type Castleman's disease, who was diagnosed in Department of Neurosurgery, Zhujiang Hospital, Southern Medical University in February 9, 2017, were retrospectively analyzed, and the relevant literatures about intracranial Castleman's disease were reviewed. The PubMed, Embase, Ovid, Cochrane, Wanfang Database and China Journal Full-text Database were searched using the keywords including "Castleman", "Intracranial"and "Central nervous system" for related literatures published between January 1, 1980 and January 31, 2017. Results: A 32-year old female was admitted because of headache for one month. CT examination showed an irregular and well-defined hyperdense lesion in the right temporo-parietal area with surrounding extensive edema and this mass was markedly contrast enhanced. Laboratory examination revealed anemia and hyperimmunoglobulinemia. The mass was removed completely by surgical resection. Postoperative pathology examination revealed mixed type Castleman's disease in the right temporo-parietal area. MRI was performed 3 months after the surgery. No residual tumor and recurrence were demonstrated. Seventeen related case reports were retrieved and 20 cases of intracranial Castleman's disease were reported. Among total 21 cases, there were 7 males and 14 females, ranging from 8 to 82 years old, and the median age was 47 years old. The mass arised from the dura matter or leptomeninges, and the most common sites of origin were located at the cerebral convexity and the parafalcine. The most common clinical features were seizures and headache. Radiographically, the previously reported cases presented as a homogeneously enhanced extra-axial well-defined mass with a marked peritumoral edema. Conclusion: Intracranial Castleman's disease is a rare clinical condition. The diagnosis can be made in combination with clinical findings and the laboratory examination.

Rare Location of Castleman's Disease in the Temporal Region: A Case Report Involving a Young Korean Woman and Review of the Literature

Castleman's disease (CD) is an uncommon benign lymphoproliferative disorder of unknown etiology. Histopathologically, it is divided into three types: hyaline-vascular, plasma cellular, and multicentric CD. The mass usually presents asymptomatically; however, it can cause non-specific symptoms such as fever and fatigue. Although CD can be found wherever lymph nodes are present, 75% of cases are reported in the mediastinum, and occurrence in the head and neck is rare. Herein, we report a rare case of CD presenting as a superficial mass in the temporal region. To the best of our knowledge, this is the first report of temporal CD in Korea involving a young patient.