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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.
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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
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Humans , Male , Adult , Castleman Disease/diagnosis , Lupus Erythematosus, Systemic/diagnosis , Biopsy , Radiography, ThoracicABSTRACT
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.
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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.
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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).
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Humans , Female , Aged , Lymphoma, Large B-Cell, Diffuse/complications , Castleman Disease/complications , Lymph Nodes/pathology , Lymphoma, Large B-Cell, Diffuse/pathology , Castleman Disease/pathologyABSTRACT
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.
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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.
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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.
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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.
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Female , Humans , Fatigue , Fever , Castleman Disease , Head , Korea , Lymph Nodes , Lymphoproliferative Disorders , Mediastinum , Neck , Plasma , Temporal LobeABSTRACT
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.
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Female , Humans , Fatigue , Fever , Castleman Disease , Head , Korea , Lymph Nodes , Lymphoproliferative Disorders , Mediastinum , Neck , Plasma , Temporal LobeABSTRACT
A doença de Castleman é um distúrbio linfoproliferativo raro. Há três tipos histológicos: hialino-vascular (mais comum), variante de células plasmáticas e forma mista. A forma hialino-vascular é caracterizada tipicamente por apresentar uma evolução clínica benigna e localizada, sem sintomas constitucionais. É geralmente tratada com cirurgia e/ou radioterapia. A doença multicêntrica apresenta sintomas sistêmicos. Ainda não há um consenso sobre qual a melhor abordagem terapêutica. Reportamos o caso da doença em um homem de 47 anos com diagnóstico de doença de Castleman variante hialino-vascular e anemia hemolítica autoimune associada, com presença de CD-20, CD-10, CD3 e Ki67 positivos. Foi tratado com protocolo quimioterápico esquema CHOP e corticoterapia com prednisona, evoluindo com melhora do quadro.
Castleman's disease is a rare lymphoproliferative disorder. There are three histological types: hyaline-vascular (most common), plasma cell variant, and mixed form. The hyaline-vascular form is typically characterized by a benign and localized clinical course without constitutional symptoms. It is usually treated with surgery and/or radiotherapy. The multicentric disease has systemic symptoms. There is still no consensus on the best therapy approach. We report a case of the disease in a 47-year-old man diagnosed with hyaline-vascular variant of Castleman's disease, and associated Autoimmune Hemolytic Anemia, with the presence of CD20-positive, CD10-positive, CD3-positive and Ki67-positive cells. He was treated with chemotherapy protocol of CHOP regimen and corticotherapy with Prednisone, and evolved with improvement.
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Humans , Male , Middle Aged , Anemia, Hemolytic, Autoimmune/diagnosis , Castleman Disease/drug therapy , Diagnosis, Differential , Immunohistochemistry , Lymphoproliferative DisordersABSTRACT
Purpose To evaluate imaging characteristics of normal and dynamic contrast enhanced multi-slice-spiral CT (MSCT) and 18F-FDG PET/CT in the patients with the mesenteric Castleman's disease (CD).Materials and Methods A retrospective analysis was made in 11 patients who were pathologically diagnosed CD and investigated their imaging characteristics of the MSCT and 18F-FDG PET/CT.Results 6 cases showed single lesion,all of them were round or oval.5 cases presented satellite lesions.The maximum diameter was (6.11 ± 1.45)cm.3 cases showed stippled or branch calcification,and 3 cases showed slightly patchy low density.11 cases had clear margins except 1 case.All cases showed the same strong lesion enhancement as that in aorta.9 cases showed significantly enhanced tumor blood vessels around the edge or shadow.The degree of 18F-FDG uptake increased in 11 cases,SUVmax was 3.1-5.7,and the average was (4.16 ± 0.86).Conclusion Multimodality imaging performances play an important role in the diagnosis of mesenteric CD.
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Castleman’s disease (CD) is a rare disease primarily involving lymph nodes and lymphatic system. Other synonyms used for the Casleman's disease are giant lymph node hyperplasia, and angio- follicular lymph node hyperplasia (AFH). CD is not a true cancer and is a lympho-proliferative disorder which means there is an abnormal overgrowth of cells of the lymphatic system that is similar in many ways to lymphomas (cancers of lymph nodes). Even though CD is not officially a true cancer, one form of this disease (known as multi-centric Castleman’s disease) acts very much like lymphoma. In fact we can call it as premalignant condition as many people with this disease eventually develop lymphomas. Multi-centric CD is more common in people infected with HIV. In recent years, it’s become clear that another virus, known as human herpesvirus-8 (HHV-8) or Kaposi sarcoma herpes virus (KSHV), is often found in the lymph node cells of people with multi-centric CD. In fact, HHV-8 is found in the lymph nodes of nearly all CD patients who are HIV positive. We present a case of unicentric plasma cell type of Castleman' s disease involving central nervous system ( CNS) or brain perenchyam in a young male patient which is a rare occurrence.
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Objective To analyze the imaging features of Castleman disease (CD) and its correlation with histopathologic and clinical features. Methods The images of 20 patients with CD were retrospectively reviewed and compared with their clinical and pathological findings. All the patients received CT or MRI plain scan and dynamic enhanced scan. 12 patients underwent CT examination and 8 underwent MRI examination. Results Locations of the lesions were found in the neck (n=6), abdomen and pelvis (n=6), mediastinum (n=3), retroperitoneum (n=2),multiple locations (n=2) and left subclavicular region (n=1). The clinical subtypes included unicentric type (n=17) and multicentric type (n=3). Histopathologic classification was as follows: hyaline-vascular type (n=16), plasma cell type (n=3) and mixed cell type (n=1). The lesions were manifested as homogeneous masses and well-defined margins of soft-tissue attenuation on the plain scan, apart from three cases with lobular, three cases with unclear margins, five cases with multiple masses and one case with heterogeneous texture. After contrast administration, 19 cases showed marked or persistent enhancement, one showed slight enhancement; 14 showed homogeneous enhancement, six showed heterogeneous enhancement; and 17 showed enlarged blood vessels within or around the mass. Conclusion Imaging features of CD are closely correlated with its clinical and pathological subtypes. A good knowledge of imaging features can contribute to a better understanding of the lesion before operation and a suitable therapeutic regimen.
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Objective To evaluate the imaging characteristics of 18 F-FDG PET/CT in patients with Castleman's disease (CD).Methods Twelve patients (male 7,female 5,mean age:(51.2±19.3)years) with pathologically confirmed CD and 18F-FDG PET/CT were retrospectively enrolled.A lesion with SUVmax greater than that of normal liver tissue was defined as positive.Lesion size (short diameter) was measured.Correlation between size and SUVmax was analyzed by Pearson correlation analysis.SUVmax of different clinical and pathological types was compared using Mann-Whitney rank sum test.Results Among 12patients,7 patients had localized CD (LCD) involving a single group of lymph nodes and 5 patients had multicentric CD (MCD) involving more than one group of lymph nodes with some having lung and spleen infiltration.A total of 129 positive lesions were found (7 of LCD,122 of MCD) and the average SUVmax was 4.20±1.84 (range:2.50 to 10.70).There was no significant correlation between SUVmax and lesion size (r =0.189,P＞0.05).There was no significant difference in SUVmax between LCD and MCD (4.23±1.77 vs 4.19±1.86; Z=-0.108,P＞0.05) ; but there was significant difference in SUVmax between the patients with and without clinical manifestations (4.56±2.06 vs 3.41±0.87; Z=-2.443,P＜0.05).No significant difference was found between SUVmax of hyaline vascular CD and plasma cell CD (3.63±0.89 vs 4.26±1.91; Z=-0.519,P＞0.05).Conclusions CD shows primarily lymph node involvement with increased 18F-FDG uptake.However,the degree of FDG uptake does not correlate with clinical presentation or pathological types.
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Castleman's disease (CD) is a rare benign disorder of unknown etiology characterized by proliferation of lymphoid tissues. Seventy percent of this tumor occurs in the mediastinum and it is seldom found in neck, pancreas or pelvis. We report a case of asymptomatic pelvic CD initially presenting as an adnexal tumor in a 27-year-old woman. Initial transvaginal sonography revealed 7-cm-sized hyperechoic mass adjacent to the right ovary and the following abdominal computed tomography scanning showed the same sized mass located on the right extraperitoneal pelvic cavity. Laparoscopic mass excision was performed without any complication and pathological diagnosis was made as CD. CD should be included in the differential diagnosis of female pelvic masses which are noted in the pelvic cavity. In this report, we review the clinicopathological findings in a presentation of CD.
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Adult , Female , Humans , Diagnosis , Diagnosis, Differential , Castleman Disease , Lymphoid Tissue , Mediastinum , Neck , Ovary , Pancreas , PelvisABSTRACT
Castleman's disease is an uncommon lymphoproliferative disorder which can be unicentric or multicentric. Hialine vascular variant is the most common pathologic form, which is usually unicentric and presenting as mediastinal tumors. We report a 31-year-old female with a history of retrosternal pain. A chest CAT sean showed a tumor in the posterior mediastinum. The patient was operated and the tumor excised. The pathology report showed a Castleman's disease. No other tumors were found in the patient, who had a favorable evolution.
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Adult , Female , Humans , Castleman Disease/pathology , Diagnosis, Differential , Castleman Disease/surgeryABSTRACT
Objective To describe the CT features of giant lymph node hyperplasia in abdomen.Methods A retrospective study of CT features of giant lymph node hyperplasia in abdomen confirmed by surgery or biopsy pathology was performed.CT findings of all lesions were assessed by two radiologists including size,location,pattern of enhancement and density characteristics.Results 1 2 patients with giant lymph node hyperplasia in abdomen were confirmed including 7 women and 5 men with age range from 11 to 75 years (median age 3 9 years).All patients underwent pre-and post-contrast enhanced CT.CT showed a single mass in 9 patients lo-cated at retroperitoneum in 5,porta hepatic in 2,and mesentery in 2.Multiple masses were located at chest,abdomen and neck in 3.The lesions ranged in size from 1.2 to 11.9 cm in maximum diameter with an average size of 3.7cm.CT showed all lesions with well-defined margin and regular shape.The lesions less than 5 cm in diameter usually showed homogeneous enhancement,and how-ever those more than 5 cm showed heterogeneous enhancement.The calcification was seen in two patients.Conclusion CT features of giant lymph node hyperplasia in abdomen are characteristic.
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A 47-year-old man presented with recurrent oral ulcerations for more than 1 year and generalized vesicles for 9 months.Physical examination revealed multiple oral ulcers,patchy labial erosions with yellow or dark brown crusts,maceration and blanching of the angle of mouth resulting in difficulty in mouth opening.Cinnamomeous crusts were seen on the nasal limen,palpebral margin and coronary sulcus of penis,with pale red macules after decrustation.Diffuse erythematous patches and bullae were present on the trunk and extremities.The walls of bullae tightly clung to the skin,and some bullae were ruptured,leaving an erythematous and moist surface and giving an erythema multiforme-like appearance; some bullae were turbid and covered with adherent drugcontaining white crusts or black crusts.Nikolsky's sign was negative.Shrinking,edematous dark-red patches were seen in periungual regions of all the fingers and toes.Histologic biopsy and direct immunofluorescence examination of bullous lesions confirmed a diagnosis of paraneoplastic pemphigus (PNP).Histopathological and immunohistochemical findings from the mediastinal tumor were consistent with Castleman's disease (hyalinevascular type) with proliferation of follicular dendritic cells.Laboratory examination revealed a decrease in serum complement C3 and the presence of antinuclear,anti-nucleosome and anti-dsDNA antibodies.The final diagnosis included Castleman's disease,PNP and systemic lupus erythematosus.After 2 months of treatment with low-dose prednisone and azathioprine,skin lesions completely regressed.Then,the dose of prednisone and azathioprine was tapered.Six months later,the patient himself suddenly withdrew prednisone and azathioprine,and began to take traditional Chinese medicine; thereafter,the lesions developed into toxic epidermal necrolysis (TEN),and the patient died finally.This case demonstrates that immunosuppressive therapy should be maintained for a long period of time in patients with Castleman's disease after tumor removal,otherwise,skin lesions may recur or get worse.
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BACKGROUND: Multicentric Castleman's disease (CD) is commonly associated with poor prognosis, and well-known prognostic factors are scarce. We performed a retrospective analysis to define the clinical features and prognostic factors for patients with multicentric CD. METHODS: Between 1990 and 2013, 32 patients with multicentric CD were identified from the database of the Asan Medical Center, Seoul, Korea. Clinicopathologic data were collected by reviewing the medical records. With the exclusion of 4 patients because of unknown human immunodeficiency virus infection status, 28 human immunodeficiency virus-negative patients with multicentric CD were included in this analysis. RESULTS: Most of the patients were male (76%) and had a median age of 54 years. Hyaline vascular variant was the most common subtype (N=11, 39%). Hepatosplenomegaly (61%), fever (39%), edema (29%), and ascites (18%) were the most frequently reported symptoms and signs at diagnosis. With a median follow-up of 67 months, the 5-year overall survival (OS) was 77%. Patients with extravascular fluid accumulation (i.e., peripheral edema, ascites, and/or pleural effusions) were significantly associated with a poor survival rate (5-year OS, 94% vs. 56%; P=0.04). The extent of disease involvement was also a significant prognostic factor (5-year OS, 91% for involvement on a single side vs. 73% on both sides of the diaphragm; P=0.03). Other clinicopathologic factors were not significantly associated with patient survival. CONCLUSION: Our findings suggest that the hyaline vascular variant is not a rare subtype of multicentric CD. Extravascular fluid accumulation and disseminated disease involvement seem to be significant prognostic factors.