Localized Castleman's Disease Research Articles

Paraneoplastic pemphigus with underlying Castleman's disease in a16-year-old girl

Paraneoplastic pemphigus is arare, life-threatening autoimmune disease that is clinically characterized by mostly extensive and refractory mucosal erosions and polymorphous skin lesions. We report here on a16-year-old girl with isolated oral erosions, in whom mucosal pemphigoid was initially suspected and after treatment with prednisolone and dapsone marked improvement was achieved. However, afew months later the patient developed massive respiratory insufficiency as aresult of bronchiolitis obliterans, so that alung transplant was planned. As part of the preparatory diagnostic workup, unicentric, abdominally localized Castleman's disease was diagnosed, which ultimately led to the diagnosis of paraneoplastic pemphigus as evidenced by envoplakin autoantibodies. Tumor resection and subsequent lung transplantation achieved good results with sustained mucocutaneous remission.

Multicentric Castleman disease revealing complete signal transducer and activator of transcription 1 deficiency treated by JAK1/2 inhibition

Castleman disease (CD) is a rare and poorly understood nonmalignant lymphoproliferation of unclear pathogenesis. First described in 1954, CD has two main clinical presentations: a localized or unicentric CD (UCD) and a multifocal or multicentric CD (MCD), which is frequently associated with systemic inflammation through the secretion of IL-6. The UCD phenotype is the most commonly reported in children and has the most favorable outcome.1 The diagnosis of CD remains histopathologic with two defined pathologic types: (1) the hyaline vascular type, which is characterized by abnormal germinal centers, penetrating hyalinized vessels, and abnormal follicular dendritic cells; and (2) the plasma cell variant, in which normal or enlarged germinal centers are associated with interfollicular plasma cell infiltration.

Next Generation Sequencing of Castleman Disease and Follicular Dendritic Cell Sarcomas Associated with Castleman Disease

Castleman disease (CD) is a rare lymphoproliferative disorder, that can transform into malignant lymphomas during clinical progress. Histologically CD can be subdivided into a hyaline-vascular variant (CD-HVV), a plasma cell variant (CD-PCV) and an intermediate variant (CD-IV) and further sub-classified into localized unicentric CD (U-CD), and multicentric CD (M-CD). An association with human herpes virus 8 (HHV8) and human immunodeficiency associated virus (HIV) in some CD cases is well documented, though the pathogenesis of CD still remains to be understood (Casper et al. Br J Haematol 2005). In recent years, cytogenetic and molecular studies have demonstrated clonal abnormalities in CD (Fajgenbaum et al. Blood 2017) as well as somatic mutations in follicular dendritic cell sarcoma (FDCS) - a rare clinically aggressive mesenchymal tumor associated with CD-HVV (Griffin et al. Mod Pathol 2015). However, an understanding of the genetic and epigenetic landscape of these rare diseases is lacking. To the best of our knowledge, our study is the first to analyze the genetic and epigenetic features of CD and FDCS associated with CD.We analyzed 18 cases of CD and 3 cases of FDCS associated with CD by whole exome and/or targeted next generation deep sequencing (NGS). Hybrid-capture NGS [FoundationOne (F1), Foundation Medicine Inc. (FMI), Cambridge, MA] of 343 genes was performed on our 18 CD cases as previously described (Lee et al. Cancer Discov 2016). 100 base-pair paired end whole exome sequencing was performed on our 3 FDCS cases using Sureselect whole exome V5 kit (Agilent Technologies) at an average depth of coverage of 180-fold. Alignment was performed using BWA-MEM with further analysis performed using GATK (version 3), Varscan2, and SNNPET (Agilent Technologies). Annotation of variants was performed using Surecall (version 3.5.1.46, Agilent Technologies) and Seattleseq (University of Washington, Seattle WA; Version 9.10). Variants were additionally curated using Exome Aggregation Consortium (EXAC), Clinvar, 1000 genomes, and Exome Variant Server (EVS). Copy number variation (CNV) analysis was performed with Surecall and CNVSeq, both run with default parameters, and analysis was performed on the “hits” file.Of our 18 CD cases, 13 were CD-HVV, 3 were CD-PCV and 2 were CD-IV. 14 cases were unicentric and 4 were multicentric. All cases were negative for HHV8 and HIV. A single case of multicentric CD, CD-PVC, showed a somatic DNMT3A L295Q mutation; a gene frequently mutated in hematopoietic neoplasms and possibly related to clonal hematopoiesis of indeterminant potential (Zhang et al. Biomark Res 2017; Yang et al. Nat Rev Cancer 2015). Two other cases of multicentric CD showed common amplification of PTPN6 , ETS1 and TGFBR2 . These genes are known to interface in oncogenesis or critical biologic processes: deletion of PTPN6 results in alteration in dendritic cell function and autoimmunity (Clare et et al. Nano 2013), overexpression of ETS1 is seen in other instances such as breast carcinoma, and leukemia (Dittmer et al. Mol Cancer 2013) and relatively increased expression of TGFBR2 is seen in stromal/vascular tissues. Only one CD-HVV case, presenting clinically untypically aggressive, showed amplifications of HIST genes. These findings could be consistent with a clonal, neoplastic nature to at least a subset of CD cases.To understand the possible association between CD-HVV and FDCS we also analyzed the mutational landscape of FDCS associated with CD. Our FDCS cases had 3-5 somatic mutations and CNVs in oncogenes and genes known to be critical in tumorigenesis, such as: ZBTB7a - playing a key role in the development from lymphoid progenitors into B-cell lineage, and STAT3 - a transcription factor mutated in NK, T-cell and B-cell disorders (Yu et al. Nat Publ Gr 2014). Given the diverse clinical presentation of FDCS it is not surprising, that previously published mutations seen in FDCS not arising from CD were missing in our study. However, previously described CNVs in FDCS such as NFKBI , and JAK2 were present, next to overlapping CNVs in BRAF and FANCB in two of our cases. In one of our FDCS cases loss of several HISTH genes were further seen. No Translocations or recurrent mutations were seen. [Display omitted] DisclosuresMughal:Foundation Medicine, Inc: Employment, Other: Stock. Ali:Foundation Medicine, Inc: Employment, Other: Stock.

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.

Radiologic and Histopathologic Features in an Intracranial Localized Castleman Disease

Castleman disease (CD) is a rare pathologic process of unknown etiology, characterized by non-neoplastic lymph node enlargement. Two distinct histologic patterns are recognized; the hyaline-vascular type and the less common plasma cells type. Another intermediate type has been described. The clinical features are classified into 2 categories, localized (unicentric) and generalized (multicentric), the later associated with systemic manifestations and poor prognosis. CD affecting the central nervous system is extremely rare. We report a new case of localized intracranial CD and we accomplish a review of the literature. A 30-year-old man presented with a generalized tonic-clonic seizure. Computerized tomography and magnetic resonance imaging showed a small mass in the right temporoparietal convexity with homogenous enhancement after contrast administration. Extensive vasogenic edema in comparison with the size of the mass was also identified and based on the neuroradiologic finding, a suspected diagnosis of meningoangiomatosis was formulated. The mass was completely resected and his histologic examination identified the hyaline-vascular type of CD. One year after surgery, the patient remains seizure free, without evidence of systemic involvement or recurrence of the mass. Our case and review of the literature show the value of the extensive brain edema on neuroimaging finding to the differential diagnosis for a solitary mass arising from the meninges. We emphasize on the need for histologic examination when the diagnosis of meningioma is not entirely clear.

Maladie de Castleman localisée avec prolifération lymphoblastique T indolente

Herein we report the case of a 41-year-old woman who presented with pelvic pain. Magnetic Resonance Imaging exhibited a single pelvic mass, measuring 50mm long axis, alongside the right iliac vessels. Histological examination of the excision specimen showed a lymphoid tumor with features of localized Castleman disease, hyaline vascular type. Moreover we identified multiple interfollicular dark clusters, composed of cells morphologically resembling cortical thymocytes. Their immunophenotype was consistent with an intermediate stage of T-cell differentiation, with the expression of CD3, CD4, CD8, TdT, CD1a, CD99, CD2, CD5, CD7 and CD10, with 40% Ki67. After integration of clinical and molecular data, the retained diagnosis was an indolent T-cell lymphoblastic proliferation associated with hyaline vascular localized Castleman disease. The clinical course confirmed the indolent nature of the proliferation, despite a late local recurrence at 7 years of the initial diagnosis, without histological modification, due to an incomplete initial resection surgery.

Diagnosis and treatment of 41 cases of head and neck Castleman's disease

Objective: To explore the diagnosis and treatment of head and neck Castleman's disease (CD), and to improve the understanding of the disease. Methods: The clinical data of 41 patients with head and neck CD treated from January 2007 to July 2017 in the First Affiliated Hospital of Zhengzhou University were analyzed retrospectively. The patients were divided into two groups: localized CD (LCD, n=27) and multicentric CD (MCD, n=14). The clinical manifestations, laboratory examinations, operations, pathological findings and follow-up data were analyzed. Results: LCD was characterized by asymptomatic enlargement of the single lymph node with mild clinical symptoms. The main pathological type was hyaline vascular type, which can be cured by surgical treatment. MCD accompanied by fever 9 cases(64.3%), anemia 8 cases (57.1%), splenomegaly 7 cases (50.0%), respiratory symptoms 6 cases (42.9%), with multiple enlarged superficial and deep lymph nodes with pain, pleural effusion, and edema of lower extremity, C-reaction protein increased in 8 cases (57.1%), hypoproteinemia 8 cases(57.1%), globulin increased 7 cases (50.0%) and neutropenia 6 cases(42.9%). The main pathological type was plasma cell type in 7 cases (50.0%), and also there were 4 cases of hyaline vascular type and 2 cases of mixed type. Chemotherapy with or without rituximab was the main treatment. Most patients received complete or partial remission except for 2 patients who died of lung infection due to chemotherapy. Conclusions: The diagnosis of head and neck CD depends on pathological examination. LCD shows mild symptoms and good prognosis after surgery. However, clinical manifestations of MCD are complex, with relatively poor prognosis after comprehensive treatment.

Castleman's Disease Presenting in the Pelvis

Castleman's disease (CD) is a rare benign lymphoid disorder with unknown etiology. There are 2 major forms of this disease, unicentric (ie, localized CD) and multicentric, which play a major role in determining therapy. The mediastinum is the most common localization for the localized CD, whereas its occurrence in the pelvis is even rarer. We report the case of a 22-year-old woman who had a pelvic mass located in the region of the left iliac fossa. The patient subsequently underwent a robotic-assisted laparoscopic tumorectomy. Pathological examination revealed pelvic localized CD of the hyaline-vascular type. CD should be included in the differential diagnosis of pelvic masses that are noted in the pelvic cavity.

Retrospective analysis of 65 Castleman diseases

Objective To explore and clarify the clinical features and management of patients with Castleman disease. Methods The clinicopathological features and therapeutic method of 65 patients with Castleman disease admitted to our hospital continuously from 2005 to 2012 were analyzed retrospectively. The mean follow-up time for the entire series was 40(15-71) months. Results In contrast to Localized Castleman disease(LCD), Multi-centric Castleman disease (MCD) patients were relatively older and exhibit various symptoms and abnormal laboratory tests. We found 44 tumor in LCD group. LCD ones, most of which were hyaline vascular(33/42, 78.6%), were more likely originated in retroperitoneum, enterocoelia(10/44, 22.7%) or mediastinum(10/44, 22.7%). The initial treatment for LCD patients was complete surgical resection and all patients are current alive with no evidence of recurrence. MCD ones always accompanied by fever(9/23, 39.1%), anemia(9/23, 39.1%) and splenomegaly, and most of MCD patients were plasmacytic variants(14/23, 60.9%) or mixed cellular variants(5/23, 21.7%). We found 66 tumor in MCD group and they were more likely originated in neck(16/66, 24.2%) and groin area(12/66, 18.2%). Surgery combined with CHOP-like chemotherapy or CHOP-like chemotherapy alone was the mainstream treatment for MCD ones. 21 patients achieved complete remission or partial remission, and only 2 patients died of lung infection. Whether patients could tolerate long-term chemotherapy or not was an important prognostic factor. Conclusions LCD patients had no typical clinical symptoms and could be cured by surgery. Most of MCD patients always had a good prognosis after receiving CHOP-like chemotherapy. Key words: Castleman disease; Clinical feature; Treatment outcome; Prognosis

Localized Castleman’s Disease in Retroperitoneum Mimicking Broad Ligament Leiomyoma – a Diagnostic Challenge

Primary retroperitoneal tumours pose a diagnostic dilemma and therapeutic challenges because of their location and late detection. Localized Castleman Disease (CD) in retroperitoneal location is a rare occurrence, with incidence of only 4% cases occurring in diverse locations like retroperitoneum. A rare case of unicentric localized Castleman Disease localized in broad ligament is hereby reported which was clinically &amp; radiologically suspected as broad ligament leiomyoma but finally diagnosed as Castleman’s Disease, hyaline vascular type in a young female posing a diagnostic challenge both for the pathologist and clinician. Definite diagnosis is essential as complete surgical excision in case of unicentric Castleman’s Disease has good prognosis after resection.

Adrenal Castleman's disease: A case report and literature review

Objective To raise the awareness of adrenal Castleman's disease by analyzing the clinical features and management of a patient with adrenal Castleman's disease. Methods A case of adrenal Castleman's disease of our hospital was retrospectively analyzed, including clinical feature, laboratory findings, pathology, treatment, and follow-up. All the data and pertinent literatures were reviewed and analyzed. Results An incidentaloma measuring 4.8 cm×6.3 cm in the right adrenal gland was observed in a 30-year-old men in a ultrasonography examination performed due to a medical check-up. Laboratory analysis showed that the lesion was not hyperfunctioning. The patient subsequently underwent an exploratory laparotomy. Pathological examination revealed retroperitoneally localized Castleman's disease of the hyaline vascular type. Conclusion Adrenal Castleman's disease is a rare cause of lymph node hypertrophy, and it is necessary to keep in mind the possibility of its occurrence and take it into consideration in the differential diagnosis of any solitary, heterogeneous, and hypervascular retroperitoneal mass. The proper cooperation between the clinician and pathologist allows early diagnosis and suitable therapy. (Chin J Endocrinol Metab, 2015, 31: 672-677) Key words: Adrenal Castleman's Disease; Clinical Features; Diagnosis; Pathogenesis

Sonographic findings of localized Castleman disease of the abdomen and pelvis.

The purpose of this study was to sonographically evaluate the diagnosis of localized Castleman disease in the abdomen and pelvis. This was a retrospective analysis of 18 cases of Castleman disease localized in the abdomen and pelvis. The following features of the lesions were assessed on sonography (US): location, size, margin, echogenicity, echotexture, intralesional cystic necrosis, intralesional calcification, posterior acoustic enhancement, and blood supply. Of the 18 tumors, 16 were located in the abdomen and 2 were located in the pelvis close to iliac vessels. The most frequent appearance of localized Castleman disease in the abdomen and pelvis on US was of a single, well-defined, hypoechoic solid mass with no intralesional cystic necrosis. The internal echotexture was homogeneous in 4 cases and heterogeneous in 14 cases, with thin hyperechoic septa (n = 14) or calcifications (n = 3). Posterior acoustic enhancement was seen in 17 of the 18 cases (94%). Ninety-four percent of the lesions (17/18) had marked vascularity on color Doppler US. Localized Castleman disease in the abdomen and pelvis usually appears on US as a heterogeneously hypoechoic lesion containing thin septa, and more commonly than not, demonstrates posterior acoustic enhancement and marked vascularity.

CT and MRI Features and pathological characteristics of retroperitoneal localized Castleman's disease

To analyze the CT and MRI findings and pathological characteristics of retroperitoneal localized Castleman's disease and discuss the diagnostic and differential value of CT and MRI. CT, MRI and clinical data of retroperitoneal localized Castleman's disease, proved by surgery and pathology, of 13 patients were reviewed. Among them, all the 13 cases received CT, and 4 cases received MRI examination. Among the 13 cases, 12 cases were of hyaline vascular type, and one was of mixed type. All foci were single mass and most foci located in the periphery of the kidney. The maximum diameter was 4.2 cm to 8.6 cm and the mean diameter of all tumors was 5.9 cm. The outline of most tumors was clear and kidney-shaped. On unenhanced CT, 10 cases showed low density and 3 cases showed isodensity. On plain MRI, four cases showed iso-signal on T1WI, three cases showed slightly high signal on T2WI and one showed moderately high signal. The CT and MRI findings were similar on contrast enhanced scan. In arterial phase, 10 cases showed moderate enhancement, 2 cases showed significant enhancement and one case showed moderate enhancement. Five cases showed rather homogeneous enhancement and 8 cases showed heterogeneous enhancement.In venous phase, all the 13 tumors showed continuous enhancement. Eight cases showing heterogeneous enhancement in arterial phase showed expanded range, and among them two cases showed tending to be homogeneous enhancement. Six cases showed areas of low density without enhancement, and 9 cases were accompanied with single or multi-satellite punctuate or striped foci around the tumors. Dynamic contrast-enhanced CT and MRI combined with MR T2WI fat-suppression are of great importance in the diagnosis and identification of retroperitoneal localized Castleman's disease.

Color Doppler imaging feature of localized Castleman disease

Objective To summary the color Doppler imaging feature of localized Castleman disease.Methods From January 1997 to November 2011,32 localized Castleman diseases which were proved by pathology were analyzed.Results Round-like,hypoechoic,hypervascular lesions were showed in 23 hyalinevascular type lesions,2 of them with calcium,3 of them with structure liked lymph node hilum.Round-like,hypoechoic,hypervascular lesions or normal lymph node were showed in 5 plasma- type lesions and 4 mixed type lesions.Conclusions When round like,hypoechoic,hypervascular lesion is found by ultrasonography,Castleman should be considered.Calcium or lymph nod hilum-like structure is special finding in diagnosis of Castleman disease by ultrasonography. Key words: Ultrasonography, Doppler, color; Giant lymph node hyperplasia

The Characterization and Management of Castleman's Disease

Castleman's disease (CD) is a rare disorder of unknown aetiology characterized by lymph node enlargement. This study evaluated the clinical features, diagnosis and outcomes of surgical treatment in patients with CD. The medical records of 10 patients with histologically confirmed CD who underwent surgery between 2003 and 2008 were retrospectively reviewed. Study endpoints were survival and recurrence. The age of disease onset ranged from 11 to 63 years (median 38 years). Of the 10 patients included in the study, eight were diagnosed with localized (unicentric) CD and two with multicentric CD. All eight patients with localized (unicentric) CD were asymptomatic. Histological evidence indicated the hyaline vascular form of CD in eight patients and the plasmacytic form in the remaining two patients. The primary treatment was complete surgical resection in nine cases, and these patients were alive with no evidence of recurrence or exacerbation at the time of writing (June 2011). Localized (unicentric) CD is usually of the hyaline vascular form and complete surgical excision of the tumour allows full recovery in most cases.

Clinical characteristics and surgical treatment for localized Castleman's disease

To investigate the clinical characteristics and treatment of localized Castleman's disease (CD), and review the literatures to improve the diagnosis and management of this disease. The clinical symptoms, histopathology, CT, MRI findings and results of surgery in 20 patients with localized CD were evaluated retrospectively. The average age of the patients was 37.7 years. The lesions were located in the retroperitoneal space (9 cases), mediastinum (7 cases), pelvic cavity (1 case), neck (1 case), upper arm (1 case), and axillary (1 case). All patients underwent surgical resection, including 9 cases for retroperitoneal resection (6 cases had open operation and 3 cases laparoscopic resection) and 7 cases for mediastinal resection (open operation in 5 cases and thoracoscopic resection in 2 cases). The Castleman's disease was confirmed by histopathology. There were hyaline vascular type of CD in 17 cases, plasma cell type of CD in 1 case, and mixed cellularity type of CD in 2 cases. The duration of follow-up ranged from 12 to 165 months for 16 cases. Among them 15 patients were alive without recurrence, and 1 case had recurrence in the primary site at 47 months after the operation. Patients with Castleman's disease have no typical clinical symptoms and have normal laboratory results. The majority of patients are of hyaline vascular type of the disease. Imaging examination is helpful to diagnosis, and the final diagnosis depends on pathologic examination. Complete surgical resection of the tumor is the best treatment for localized Castleman's disease.

Castleman Disease in the Kidney and Retroperitoneum Mimicking Renal Cell Carcinoma with Retroperitoneal Lymphadenopathy: A Case Report

Castleman disease (CD) is an uncommon benign lymphoproliferative disorder, characterized by hyperplasia of lymphoid follicles. Histologically, CD can be classified into hyaline-vascular and plasma cell types. And clinically, it also can be divided into localized and disseminated types. Ninety six percent of localized CD is hyaline vascular type, and on the other hand, disseminated CD is usually a plasma cell type. CD is commonly located in the mediastinum. Retroperitoneal and pararenal locations are relatively uncommon accounting for 7% and 2% of all cases, respectively. To date, to the best of our knowledge, there have been 100 cases of renal involvement in CD. Sixty eight cases were disseminated and 32 cases were localized CD. Among localized CD, only 6 cases confined to the kidney have been reported (1-8). There are no reports in the radiologic literature describing CD, involving both kidney and pararenal space, mimicking renal cell carcinoma with lymph node metastasis. Here, we present a case of renal and pararenal CD misdiagnosed as a neoplasm of the kidney with retroperitoneal lymphadenopathy.

Ultrasonographic appearance of localized cervical Castleman's disease

Objective To investigate the ultrasonographic appearance of localized cervical Castleman's disease.Methods Six patients with localized cervical Castleman's disease confirmed by pathological examination during the period of May 2006 to August 2011 were enrolled in this study.The ultrasonographic features were compared with the pathological findings.Results All the ultrasonographic imagie of 6 patients showed low-echo lesions,with internal interrupted signals of thread-like high-echo.The lesions were isolated,oval,and regular,without characteristic structure of the lymph node and disappearrance of the hilum of lymph node.The blood flow signals were extremely rich inside the lesions,with a distribution of a similar “inferno” sign in patients with hyperthyroidism.Conlusions Ultrasonography has a extremely high value in the diagnosis of localized cervical Castleman's disease. Key words: Ultrasonography; Pathology; Castleman's disease

Diagnosis and surgical treatment of Castleman's disease

To explore the clinical features and surgical treatment of tumors associated with Castleman's disease (CD). The clinical profiles of 19 patients with neck giant lymph node hyperplasia were analyzed retrospectively. There were 8 males and 11 females with a median age of 40 years old (range: 7 - 74). The tumor locations were neck (n = 12), neck & mediastinal cavity (n = 2), axillary fossa (n = 2), retroperitoneal area (n = 2) and abdominal cavity (n = 1). Eighteen of them underwent surgical resection of tumor or lymph nodes. All were diagnosed as CD by pathological examinations. There were 16 localized CD (LCD) including hyaline vascular type (HV type, n = 11), mixed type (mix type, n = 4) and plasma cell type-Hodgkin's disease (n = 1). Among 3 multicentric CD (MCD), there were 2 case of plasma cell type (PC type) and 1 case of mixed type (mix type). Long-term survival was achieved in 19 cases among which 1 case of plasma cell type MCD survived for 5 years and underwent a second operation and postoperative chemotherapy of CVP (cyclophosphamide, vincristine & prednisone) regimen for 3 cycles due to recurrence in 2 years and 1 case of plasma cell type LCD-Hodgkin's disease survived for 15 months and underwent a second operation and postoperative chemotherapy of ABVD (adriamycin, bleomycin, vinblastine & dacarbazine)regimen for 6 cycles due to recurrence in 6 months. One case of plasma cell type MCD in abdominal cavity on chemotherapy of CHOP (cyclophosphamide, hydroxydaunorubicin, vincristine & prednisone) regimen for 6 cycles was discharged after a successful management of intestinal obstruction. The major clinical symptom of CD is a gradually enlarging painless mass. Surgical resection of tumor remains the first-line treatment for localized CD and the prognosis is excellent. Multicentric and plasma cell type CDs are prone to recurrence and transformation to lymphoma. And their first-line therapeutic should encompass multi-modality regimens of surgery and adjuvant chemotherapy. However, the clinical prognosis is still poor.

Diagnosis and treatment of cervical Castleman disease presenting as a neck mass

To investigate the clinical and pathological features, diagnosis and treatment of Castleman disease (CD). Clinical features and related information on diagnosis and treatment of eight cases of CD were retrospectively analyzed. The size of involved lymph nodes ranged from (2 cm x 2 cm x 3 cm) - (4 cm x 3 cm x 2 cm). The lymph nodes were found in level I (1 case), level II (3 cases), level III (3 cases) and level IV (1 case). CT examination in eight patients showed the lesions manifested as ellipse soft masses. Dynamic contrast CT scan in four patients showed ring-enhanced area around the masses. Blood routine examination in eight patients were generally normal, with six patients had mild anemia. Based on the clinical classification, all lesions in this group were localized CD. Histopathology indicated that all lesions were of hyaline-vascular type. After surgery, there was no recurrence during the follow-up period. Patients with localized CD mainly have lymphadenectasis in a single location. The CT scan can give some evidence. Surgery should be given first priority.