Intraperitoneal hyaline vascular Castleman disease: Three case reports

BACKGROUND Castleman disease(CD)was first reported in 1954.It is a rare non-malignant lymphoproliferative disease with unclear etiology.As the clinical manifestations of CD are different,there are difficulties in its diagnosis and treatment.Therefore,for patients with CD,it is important to establish the diagnosis in order to choose the appropriate treatment.CASE SUMMARY In this report,three patients with intraperitoneal CD treated at our center from January 2018 to June 2023 were reviewed,and the clinical and paraclinical exa-minations,diagnosis,and treatment were analyzed,and all three patients were diagnosed with CD by routine histopathological and immunohistochemical exa-minations.CONCLUSION CD is a complex and rare disease.Because there are no special clinical symptoms and laboratory abnormalities,the diagnosis often depends on routine pathological and immunohistochemical findings.

Pancreatic Castleman disease treated with laparoscopic distal pancreatectomy

BACKGROUND: Castleman disease is an uncommon lympho-proliferative disorder most frequently occurring in the mediastinum. Abdominal forms are less frequent, with pancreatic localization of the disease in particular being extremely rare. Only seventeen cases have been described in the world literature. METHOD: This report describes an interesting and unusual case of pancreatic Castleman disease treated with laparoscopic resection. RESULTS: A 48-year-old woman presented with epigastric pain. CT scan showed a well-encapsulated mass on the ventral border of the pancreas. Endosonography with fine needle aspiration biopsy was performed. Biopsy showed lymphoid elements and structures of a normal lymph node. The patient was treated with laparoscopic distal pancreatectomy. The pancreas was transected with a Ligasure device and the pancreatic stump was secured with a manual suture. One year after surgery the patient was complaint-free and showed no signs of recurrence of the disease. CONCLUSIONS: Laparoscopic distal pancreatectomy is a feasible and safe method for the treatment of lesions in the body and tail of the pancreas. Transection of the pancreas with a Ligasure device offers the advantages of low bleeding and low risk of pancreatic fistula.

Immunoglobulin G4-related lymph node disease with an orbital mass mimicking Castleman disease:A case report

BACKGROUND Immunoglobulin(Ig)G4-associated diseases are a group of systemic diseases involving multiple organs and are also known as IgG4-associated sclerosing diseases.IgG4-associated lymphadenopathy occurring in the lymph nodes is characterized by a lack of specificity due to its clinicopathological characteristics and must be differentiated from a variety of lesions,such as Castleman disease,lymphatic follicular reactive hyperplasia,and lymphoma.CASE SUMMARY A 65-year-old male patient,with Guillain-Barre syndrome for 5 years,presented to our hospital complaining of bilateral orbital mass for 2 years.After hospitalization,the results of the patient’s laboratory tests showed that immunoglobulin subgroup IgG4 was 33.90 g/L and IgG was 30.30 g/L,but serum interleukin-6 was normal.The pathological morphology of orbital mass and cervical lymph node were consistent,which showed that a large number of plasma cells and eosinophils were observed in the lymphatic follicles,and the interstitial fibrous tissue was proliferative.Immunohistochemistry showed that CD20(B cells)(+),CD3(T cells)(+),CD38(+),IgG(+),IgG4 positive cells>100/high powered field,and IgG4/IgG>40%.Combined with clinical and immunohistochemical results,lymphadenopathy was consistent with Castleman disease-like IgG4-associated sclerosing disease.Prednisone acetate treatment was given at 40 mg/d.After 2 wk,the superficial lymph nodes and orbital masses shrank,and the IgG4 level decreased.As prednisone acetate was regularly used at a reduced dosage,no recurrence of the disease has been observed.CONCLUSION This case suggested that it is necessary to proceed cautiously in clinical practice with such patients,and immunoglobulin,complement,interleukin-6,C-reactive protein,and other examinations should be performed to confirm the diagnosis.

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.

Castleman disease and TAFRO syndrome:To improve the diagnostic consciousness is the key

BACKGROUND Castleman disease(CD)and TAFRO syndrome are very rare in clinical practice.Most clinicians,especially non-hematological clinicians,do not know enough about the two diseases,so it often leads to misdiagnosis or missed diagnosis.AIM To explore the clinical features and diagnosis of CD and TAFRO syndrome.METHODS We retrospectively collected the clinical and laboratory data of 39 patients who were diagnosed with CD from a single medical center.RESULTS Clinical classification identified 18 patients(46.15%)with unicentric Castleman disease(UCD)and 21 patients(53.85%)with multicentric Castleman disease(MCD),the latter is further divided into 13 patients(33.33%)with idiopathic multicentric Castleman disease-not otherwise specified(iMCD-NOS)and 8 patients(20.51%)with TAFRO syndrome.UCD and iMCD are significantly different in clinical manifestations,treatment,and prognosis.However,a few patients with MCD were diagnosed as UCD in their early stage.There was a correlation between two of Thrombocytopenia,anasarca and elevated creatinine,which were important components of TAFRO syndrome.In UCD group,the pathologies of lymph modes were mostly hyaline vascular type(13/18,72.22%),however plasma cell type or mixed type could also appear.In iMCD-NOS group and TAFRO syndrome group,the pathologies of lymph mode shown polarity of plasma cell type and hyaline vascular type respectively.Compared with patients with TAFRO syndrome,patients with iMCD-NOS were diagnosed more difficultly.CONCLUSION The clinical and pathological types of CD are not completely separate,there is an intermediate situation or mixed characteristics between two ends of clinical and pathological types.The clinical manifestations of patients with CD are determined by their pathological type.TAFRO syndrome is a special subtype of iMCD with unique clinical manifestations.

Castleman disease of the pancreas mimicking pancreatic malignancy on^(68)Ga-DOTATATE and 18F-fluorodeoxyglucose positron emission tomography/computed tomography:A case report

BACKGROUND Castleman disease is an uncommon nonclonal lymphoproliferative disorder,which frequently mimics both benign and malignant abnormalities in several regions.Depending on the number of lymph nodes or regions involved,Castleman disease(CD)varies in diagnosis,treatment and prognosis.It rarely occurs in the pancreas alone without any distinct clinical feature and tends to be confused with pancreatic paraganglioma(PGL),neuroendocrine tumors(NETs),and primary tumors,thus impeding proper diagnosis and treatment.CASE SUMMARY A 28-year-old woman presented with a lesion on the neck of the pancreas,detected by ultrasound during a health examination.Physical examination and laboratory findings were normal.The mass showed hypervascularity on enhanced computed tomography(CT),significantly increased 18F-fluorodeoxyglucose uptake on positron emission tomography(PET)/CT,and slightly increased somatostatin receptor(SSTR)expression on^(68)Ga-DOTATATE PET/CT,suggesting no distant metastases and subdiagnoses such as pancreatic PGL,NET,or primary tumor.Intraoperative pathology suggested lymphatic hyperplasia,and only simple tumor resection was performed.The patient was diagnosed with the hyaline vascular variant of CD,which was confirmed by postoperative immunohistochemistry.The patient was discharged successfully,and no recurrence was observed on regular review.CONCLUSION High glucose uptake and slightly elevated SSTR expression are potentially new diagnostic features of CD of the pancreas.

Development of an Ultrasonic Nomogram for Preoperative Prediction of Castleman Disease Pathological Type

An ultrasonic nomogram was developed for preoperative prediction of Castleman disease(CD)pathological type(hyaline vascular(HV)or plasma cell(PC)variant)to improve the understanding and diagnostic accuracy of ultrasound for this disease.Fifty cases of CD confirmed by pathology were gathered from January 2012 to October 2018 from three hospitals.A grayscale ultrasound image of each patient was collected and processed.First,the region of interest of each gray ultrasound image was manually segmented using a process that was guided and calibrated by radiologists who have been engaged in imaging diagnosis for more than 5 years.In addition,the clinical characteristics and other ultrasonic features extracted from the color Doppler and spectral Doppler ultrasound images were also selected.Second,the chi-square test was used to select and reduce features.Third,a naïve Bayesian model was used as a classifier.Last,clinical cases with gray ultrasound image datasets from the hospital were used to test the performance of our proposed method.Among these patients,31 patients(18 patients with HV and 13 patients with PC)were used to build a training set for the predictive model and 19(11 patients with HV and 8 patients with PC)were used for the test set.From the set,584 high-throughput and quantitative image features,such as mass shape size,intensity,texture characteristics,and wavelet characteristics,were extracted,and then 152 images features were selected.Comparing the radiomics classification results with the pathological results,the accuracy rate,sensitivity,and specificity were 84.2%,90.1%,and 87.5%,respectively.The experimental results show that radiomics was valuable for the differentiation of CD pathological type.

Solitary hyoid plasmacytoma with unicentric Castleman disease:A case report and review of literature

BACKGROUND Solitary plasmacytoma and unicentric Castleman disease(UCD)are rare lymphoproliferative disorders characterized by monoclonal plasma cells and a single set of locally enlarged lymph nodes,respectively.CASE SUMMARY A 48-year-old Han Chinese man presented to our department with a neck mass and progressive foreign body sensation in his throat.18F-FDG positron emission tomography revealed focally increased radioactivity centered around the hyoid,and computed tomography(CT)revealed osteolytic lesions.Histopathology revealed Castleman-like features and CD138/CD38-positive mature plasma cells.Systemic work-up ruled out the possibility of POEMS syndrome,lymphoma,and multiple myeloma,leading to a final diagnosis of solitary hyoid plasmacytoma with UCD.The patient underwent partial hyoid resection and selective neck dissection,followed by intensity-modulated radiotherapy.99mTc-MDP singlephoton emission computed tomography/CT reevaluation showed neither local recurrence nor distant bone metastasis at the 40-mo follow-up.CONCLUSION The diagnostic process and differential diagnosis of this rare case provided valuable educational information to clinicians.

Castleman disease in the hepatic-gastric space: A case report

BACKGROUND Castleman disease, also known as giant lymph node hyperplasia, was first reported in 1956. It is a rare benign proliferative pathological change of the lymph nodes.CASE SUMMARY The patient, a 33-year-old woman, had epigastric distension for half a year.Examinations were performed in a local hospital. Computed tomography scan showed round soft tissue nodules, about 5.45 cm in diameter, in the hepaticgastric space. Endoscopic ultrasound and endoscopic ultrasound guided fine needle aspiration was performed on the patient. Rapid on-site evaluation,hematoxylin eosin staining and histopathology of the puncture smear was performed. According to the Diff-Quik staining and hematoxylin eosin staining results of preoperative endoscopic ultrasound guided fine needle aspiration puncture smears as well as the immunohistochemistry results, Castleman disease was highly suspected. A sufficient preoperative evaluation was made, and a precise surgical plan was developed. Postoperative pathology confirmed Castleman disease.CONCLUSION Endoscopic ultrasound guided fine needle aspiration can extract internal tissues of the tumor for histological and cytological examinations and provide accurate diagnosis as much as possible. Therefore, a sufficient preoperative evaluation can be made, and a precise surgical plan can be developed.

Castleman disease in the neck with hypothyroidism and metabolic syndrome: a case report

Castleman disease(CD)is a rare nonclonal lymph proliferative disorder of unknown etiology.Here,we report a case of multicentric Castleman disease(MCD)with hypothyroidism and metabolic syndrome presenting as a pain and slowly expanding,isolated neck mass in a 47-year-old Asian woman.It contributes to the practical aspects of diagnosis and treatment of Castleman disease in the neck.

A Case of Plasma cell variant Castleman Disease

Castleman disease,also known as angiofollicular lymph node hyperplasia,is a rare lymphoproliferative disease with a poorly understood etiology.We herein report a 27-year-old male with a six months history of painless and gradually enlarged lymph nodes presented recurrent cough and expectoration with fever and chills for ten days.The patient was recurrent fever even received empiric anti-inflammatory therapy.Then the right cervical follicles biopsy and pathological examination revealed Castleman disease Plasma cell variant.Castleman disease is a rare disorder that is hard to diagnose early.Now,we will review this case,summarize Castleman disease and propose a feasible diagnostic protocol.

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.

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.

Localized Castleman disease of plasma cell type in the abdomen

Castleman disease is a relatively rare entity, with the hyaline-vascular type the predominant form. Although the plasma cell type is uncommon, it still comprises approximately 10% of cases of localized diseases. In addition, the abdomen is a rare site for involvement and localized Castleman disease of the plasma cell type in the abdomen is rare. The radiologic features of localized plasma cell type in the abdomen are mostly limited to case reports. In addition to the conventional imaging findings, we present some new imaging findings of localized plasma cell type in the abdomen.

Salvage therapy with lenalidomide containing regimen for relapsed/refractory Castleman disease： a report of three cases

Castleman disease （CD） is an uncommon non-clonal lymphoproliferative disorder with unknown etiology. No standard therapy is recommended for relapsed/refractory CD patients, thus requiring development of novel experimental approaches. Our cohort of three adult patients with multicentric CD （MCD） were treated with refractory to traditional chemotherapy lenalidomide-containing regimens （10-25 mg lenalidomide perorally administered on days 1-21 in 28-day cycle） as second- to fourth-line treatment. Partial remission was achieved in first plasma-cell CD patient, who relapsed seven months after autologous hematopoietic stem cell transplantation and then failed to respond to four cycles of chemotherapy. Partial remission was obtained in second patient with CD and polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes syndrome. Third case showed complete remission with complete disappearance of pleural effusion and ascites and normalization of platelet count. To conclude, encouraging clinical responses were achieved in cohort of three patients with lenalidomide-based regimen, though long-term efficacy remains to be observed. We propose further investigation of therapeutic potential of this drug in treating MCD.

A rare case of Castleman disease presenting as pulmonary mass mimicking central pulmonary malignancy

Castleman disease is a rare disorder of the lymphoid system characterized by noncancerous growths that may develop in lymph node tissues throughout the body. Most often this occurs in the neck, mediastinum, and abdomen where lymph nodes aggregate. Here we describe a case of asymptomatic intrapulmonary Castleman disease adjacent to the left pulmonary hilum that mimicked central pulmonary malignancy in a 27-year-old man who underwent curative surgical removal of the mass.

POEMS syndrome associated with Castleman disease:a case report and literature review

Polyneuropathy,organomegaly,endocrinopathy,M proteins,and skin changes(POEMS)syndrome is a multisystemic disorder that clinically manifests as paraneoplastic and monoclonal plasma cell dyscrasia.Its acronym is derived from its principal characteristics:polyneuropathy,organomegaly,endocrinopathy,M proteins,and skin changes.Here,the authors reported a case of POEMS syndrome that was also associated with Castleman disease.A 53-year-old female patient was admitted to our hospital with limb weakness,numbness,edema,abdominal distention,and fever.Physical examination revealed tetraplegia,paraesthesia,and hyporeflexia in all four limbs,in addition to lymphadenectasis,splenomegaly,skin hyperpigmentation,hypertrichosis,and pitting edema.Laboratory tests and imaging revealed thrombocytosis,hypothyroidism,diabetes,hydropericardium,hydrothorax,splenomegaly,and lymphadenectasis.Electromyography showed the characteristic patterns of both demyelinating disease and axonal degeneration.Serum protein electrophoresis revealed monoclonal immunoglobulin G-lambda paraproteins.Histological examination clearly diagnosed the disease as the hyaline vascular subtype.The final diagnosis in this case was POEMS syndrome in association with Castleman disease.

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.

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.