Unicentric Type Research Articles

Castleman disease in the parapharyngeal space: a case report

Objective:Castleman's disease is a group of heterogeneous lymphoproliferative disorders with unclear etiology and pathogenesis.This article reports a case of Castleman's disease of the parapharyngeal space, with a comprehensive literature review of the clinical feature and typing, pathologic diagnosis, treatment, and prognosis of the disease.This case is a unicentric type, which is clinically characterized by significant enlargement of lymph nodes without systemic symptoms. The pathology was hyaline vascular type, and the treatment of unicentric type was mostly based on surgery with a better prognosis.

The clinicopathologic features of unicentric Castleman disease in the children's neck

Objective:To investigate the clinical features, imaging findings, pathological phenotype, treatment and prognosis of unicentric Castleman disease in the children's neck, in order to improve the understanding of CD among Otolaryngology Head and Neck Surgery. Methods:Retrospective cross-sectional, observational study was undertaken in Kunming Children's Hospital, from the archival data between January July 2015 and June 2020. Only 6 cases of CD were identified after studying the histomorphological characteristics and neck mass diagnosed. The imaging and pathological features were summarized and the pathogenesis was discussed. Results:Among the 6 cases of Castleman disease, five were male and one was female. Histopathology: Five cases were hyaline vascular subtype, one was mixed type. The uniform clinicopathologic features seen in all hyaline vascular subtype of CD included atrophic germinal centre with lymphocyte depletion, concentric rings of small lymphocytes, increased vascularity and predominance of high endothelial vessels in interfollicular region. Twinning, in which two or more germinal centers are combined and surrounded by lymphocytes in the mantle zone was observed in two cases with lollipop pattern at the same time. All the cases underwent complete surgical resection, the median follow-up time was 48 months（26, 84）, both of them had good prognosis. Conclusion:Most cases of unicentric type CD in children are diagnosed late, which is clinical showed by painless lymphadenopathy. The most common pathological type is hyaline vascular. The overall prognosis of surgical treatment was good.

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

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

The clinical features of Castleman disease in the head and neck

Objective:To investigate the clinicopathological features, treatment and prognosis of Castleman disease in the head and neck. Methods:The clinical and pathological data of 18 patients with Castleman disease of the head and neck in Nanjing Drum Tower Hospital from 2007 to 2021 were retrospectively analyzed. There were 14 cases of unicentric type and 4 cases of multicentric type. The clinical characteristics, treatment and prognosis were analyzed. Results:Among the 18 cases of Castleman disease in the head and neck, 1 case was located in the parotid gland, 1 case was behind the ear, 1 case was in the parapharyngeal space, 3 cases were in the neck region Ⅰ, 2 cases were supraclavicular, 2 cases were in the neck region Ⅲ, the rest were located in more than two subregions of the neck. In patients with unicentric type, no tumor recurrence and progression were found in the postoperative re-examination with neck Doppler ultrasound and CT; in the multicentric type, multiple organ dysfunction, such as edema of both lower extremities, hepatosplenomegaly, and cough, were found. Of the 4 patients with multicentric type, only 1 patient received chemotherapy, and the remaining 3 patients refused chemotherapy and only received symptomatic treatment. All patients survived during follow-up, but the disease of multicentric patients progressed significantly, and the number of involved lymph nodes increased, and hepatosplenomegaly were found in some patients. Conclusion:Castleman disease of the head and neck is mostly unicentric type, which is manifested as multiple asymptomatic enlarged lymph nodes in the neck. The surgical resection is effective and the prognosis is good. Multicentric Castleman disease of the head and neck has complex clinical symptoms and involves multiple organs over time, requiring follow-up treatment.

Induction immunotherapy followed by surgery for hilar unicentric Castleman disease

Castleman disease is a rare disease borne of a B cell lymphoproliferative disorder of uncertain cause. Standard therapy for the unicentric type of Castleman disease localized as a single mass or single lymph‐node station is surgical extirpation. Nevertheless, in the thoracic cavity, unresectable cases or cases of incomplete extirpation of the tumor without lung scarring owing to tumor size/location have been noted. In such cases, lung resection (e.g., lobectomy, pneumonectomy) or additional therapy (immunotherapy, chemotherapy, radiotherapy) after resection is required. However, few instances of patients receiving induction immunotherapy or chemotherapy followed by surgery have been reported. Here, we describe a 21‐year‐old woman with unicentric Castleman disease originating from the left hilum. The tumor seemed to involve/be in contact with the pulmonary vein and bronchus. Tumor location indicated that initial resection was necessary to sacrifice upper and lower pulmonary lobes. To avoid these pulmonary resections, induction therapy followed by surgery was selected. Induction therapy using rituximab was very efficacious. Resection after induction therapy was completed only by tumor extirpation, and resulted in preservation of pulmonary function. Thoracic surgeons might consider induction therapy followed by resection if the tumor is resectable UCAD, but initial resection is needed and sacrifices a large amount of pulmonary function.

Clinicopathological and therapeutic analysis of Castleman's disease in children

Objective: To investigate the clinical features, pathological phenotype, treatment and prognosis of Castleman's disease in children. Methods: Clinical data of 15 children diagnosed with Castleman's disease in Henan Provincial People's Hospital and the First Affiliated Hospital of Zhengzhou University from May 2010 to October 2019 were analyzed retrospectively. The clinical characteristics, laboratory examination and histopathological data were analyzed. Results: Among the 15 Castleman's disease patients, 12 were males and 3 females. The age of first visit was 12 (10, 15) years. The time from mass discovery to pathologic diagnosis was 9.0 (2.0, 13.0) months. The majority of patients were unicentric (13 cases), and the histopathological type was hyaline vascular (11 cases). Unicentric lesions were most common in the neck (11 cases), all 13 patients received complete surgical resection of the lesions, the follow-up time was 20.0 (13.5, 50.5) months, and the prognosis was good. Two cases were multicentric type, the pathological types were mixed variant, meeting the criteria of idiopathic Castleman's disease, the two children underwent partial surgical resection, one was treated with rituximab and prednisone and the other was treated with thalidomide and prednisone. The follow-up time was 32 months and 10 month, both of them had good prognosis. Conclusions: Most cases of Castleman's disease in children are diagnosed late, and the unicentric type is dominant. The most common pathological type is hyaline vascular, which is characterized by painless lymphadenopathy, while multicentric type has systemic symptoms and both of them have a good overall prognosis.

8. An autoinflammatory syndrome resembling Castleman’s disease with excellent response to IL-6 blockade

Introduction Autoinflammatory diseases are an emerging group, characterised by recurrent inflammatory episodes due to dysregulated innate immunity. Common features include fevers, rash, arthralgia, lymphadenopathy and systemic symptoms. Castleman’s disease is a rare lymphoproliferative disease, associated with the overproduction of interleukin-6 (IL-6). Its two main variants: unicentric and multicentric differ in aetiology and clinical outcomes. The cytokine storm driven by IL-6 can mimic autoinflammatory disease. We present the case of an acquired autoinflammatory syndrome in a 33-year-old male with the clinical phenotype of Castleman’s but no culprit lymph node detected radiologically. Symptoms dramatically improved with yocilizumab, an IL-6 blocker.

Castleman Disease in the Buccal Space Mimicking Lymphoma: A Case Report

Castleman disease is a benign lymphoproliferative disorder of unknown etiology, more commonly appearing in the mediastinum. Craniofacial involvement is rare. Surgical excision is the only intervention needed for the unicentric type, while systemic treatment would be necessary for the multicentric type. Here, we report a rare case of unicentric castleman disease of the buccal space of a 23-year-old woman. Additionally, we review the radiological and histopathological features of this lesion.

Castleman’s Disease: A Unicentric Case of Abdominal Origin and Literature Review

Castleman’s disease (CD) is a rare lymphoproliferative disorder, which may be unicentric or multicentric. We present the case of a young man with the hyaline vascular (HV) variant of unicentric CD, with a review of cases of CD in the abdominal cavity. A 38-year-old man presented with post-meal epigastric pain and lactose intolerance that had started two months earlier. A solitary 7 cm retroperitoneal mass was detected using computed tomography (CT) scan. The mass was surgically resected and histological examination revealed CD of the HV variant. The symptoms resolved following the operation and the patient was free of disease at 1-year follow-up. A comprehensive literature review yielded reports of a further 214 patients with CD in the abdominal cavity. The female: male ratio was 1.64:1 (133 females, 81 males). The multicentric type of the disease was reported in 9/194 (4.6%) and the unicentric type in 185/194 (95.4%). The HV variant of the disease was reported in 168/191 patients (87.9%), the plasma cell variant in 13/191 (6.8%), the mixed variant in 9/191 (4.7%) and the stroma rich variant in 1/191 (0.5%). Regarding the location, 106/187 (56.6%) of the lesions were found in the retroperitoneal space, 34/187 (18.2%) in the mesentery, 8/187 (4.1%) in the liver and biliary tract, 12/187 (6.3%) adjacent to the pancreas and 17/187 (9%) in an unspecified region of the abdomen. Although further research is required to identify the exact etiology of the disease, the HV variant of CD, which is the most common, can be effectively treated with surgical resection. The plasma cell type of CD is a more serious condition and the high risk associated with misdiagnosis and delay in its treatment renders high clinical suspicion and early identification of this variant of the utmost importance.

The role of surgical resection in Unicentric Castleman's disease: a systematic review.

Castleman's disease is a rare benign lymphoproliferative disorder of unknown etiology. The disease occurs in two clinical forms with different prognoses, treatments and symptoms: a unicentric form (UCD), which is solitary, localized, and a multicentric form characterized by generalized lymphadenopathy and systemic symptoms. This article aims to review the current literature to consolidate the evidence surrounding the curative potential of surgical treatment to the unicentric type. A systematic review of English-language literature was performed and databases (Medline, Pubmed, the Cochrane Database and grey literature) were searched to identify articles pertaining to the treatment of unicentric form of Castleman's disease. Each article was critiqued by two authors using a structured appraisal tool, and stratified according to the level of evidence. After application of inclusion criteria, 14 studies were included. There were no prospective randomized control studies identified. One meta-analysis including 278 patients with UCD reported that resective surgery is safe and should be considered the gold standard for treatment. Seven retrospective studies enhance this standpoint. Radiotherapy (RT) has been used in six studies with controversial results. We conclude that surgical resection appears to be the most effective treatment for Unicentric Castleman's Disease of the thoracic cavity. Radiotherapy can also achieve clinical response and cure in selected patients.

Surgical management of isolated retroperitoneal Castleman's disease: A case report.

Castleman's disease (CD) is an uncommon, poorly understood lymphoproliferative disease. Retroperitoneal forms may present as either a unicentric or multicentric disease. The present study reports the case of a 36-year-old man who was referred to the First Affiliated Hospital, School of Medicine, Zhejiang University (Hangzhou, China), for a detailed examination of an abdominal mass. The abdominal ultrasound and computed tomography scans revealed a solid mass localized in the region between segment 1 of the liver and the pancreas. An endosonography-guided fine-needle aspiration biopsy revealed chronic inflammation and lymphadenosis. The present study reports a rare case, in which the patient was treated with an exploratory laparotomy and resection. The retroperitoneal mass was pathologically diagnosed as CD of the hyaline vascular type. The patient was closely followed-up for 11 months and is presently free of disease. In conclusion, the possibility of unicentric CD should be considered when facing a solid hypervascular retroperitoneal mass. A complete surgical resection may successfully treat the disease without an unnecessarily extensive resection for the unicentric type.

Castleman's Disease as a Rare Differential Diagnosis of Lymphadenopathy: 2 Case Reports and Review of Literature

ntroduction: Lymphadenopathy is a common complaint in children. It's defined as abnormality in size or character of lymph node. It may be localized or generalized. It may be part of a group of signs and symptoms or the sole finding and chief complaint. Several reasons can lead to Lymphadenopathy. Castleman's disease is a rare differential diagnosis of generalized or localized Lymphadenopathy. It's a rare lymph proliferative disorder that rarely occurs in children. Case presentation: We reported two cases, the first one was 13 years old female with multicenteric castleman's disease associated with constitutional manifestations and unusual hyaline vascular pathology successfully treated with monthly combination chemotherapy, the second one was 2.5 years old male with unicentric hyaline vascular castleman's disease with unusual young age at presentation successfully treated with surgical excision. Conclusion: Castleman's disease is a rare cause of Lymphadenopathy. Although, it's rare in children, it can occur at any age. Most of cases of multicenteric castleman's disease are plasma cell variant, yet hyaline vascular type may occur. Multicenteric castleman's disease can be successfully treated with monthly combination chemotherapy. Most cases of unicentric castleman's disease are hyaline vascular type. Although, surgical removal of unicentric type is usually sufficient to achieve complete remission, recurrence can still occur.

Castleman's disease

Castleman's disease (CD) is a lymphoproliferative disease. Two histological subtypes of CD are hyaline-vascular and plasma cell variants. CD is classified clinicoradiologically into unicentric (localized) type and multicentric (diffuse) type according to the extent of spreading of the disease. Initial CD diagnosis is usually based on imaging findings; further confirmation is made by excisional biopsy and histopathological evaluation.

Castleman's disease: A rare case report of unicentric type in a young female

Castleman's disease (CD), or angiofollicular lymph node hyperplasia, is a relatively rare disorder characterized by the benign proliferation of lymphoid tissue. Two clinical entities have been described: A unicentric presentation with the disease confined to a single anatomic lymph node and a multicentric presentation characterized by generalized lymphadenopathy and a more aggressive clinical course. In addition, three histopathological subtypes have been described: Hyaline-vascular (80–90%), plasma cell (10–20%), and a mixed variant. Preoperative diagnosis of CD is difficult, and the definitive result is based on postoperative pathological findings. The gold standard therapy for unicentric type is the complete surgical excision. Overall prognosis is good, particularly in the unicentric variety of disease. We report a case of 22-year-old female presenting with a large subpectoral mass on left side chest wall below clavicle. It was painless and gradually increasing since 6 months.

Hyaline vascular type of unicentric Castleman’s disease with proliferation of glomerular endothelial cells

Castleman’s disease (CD) is a lymphoproliferative disorder of uncertain etiology. It can be classified histopathologically as hyaline vascular (HV) or plasma cell (PC) type; and clinically as unicentric or multicentric. Multicentric CD mostly manifests as the PC type and is often associated with a variety of systemic complications, although renal complications are uncommon. We present here a case of HV type, unicentric CD with a variety of systemic and renal complications, including the proliferation of glomerular endothelial cells. Various cytokines are thought to be involved in the etiology of this disease, especially interleukin-6 (IL-6) and vascular endothelial growth factor (VEGF), which are the main cytokines inducing systemic complications. It has been reported that most PC types and/or multicentric types of CD show increasing levels of serum IL-6 and VEGF, as well as systemic symptoms. Although the current case showed high serum IL-6 and VEGF levels as well as systemic symptoms, the pathological and clinical type were of the HV type and unicentric type, respectively. After immunosuppressive therapy, the serum levels of IL-6 and VEGF returned to the normal range, and systemic complications, including renal involvement, also improved. We report the possibility of pathogenesis via the serum and the pathology of this rare case.

Localized retroperitoneal Castleman’s disease: a case report and review of the literature

IntroductionCastleman’s disease, also known as angiofollicular lymph node hyperplasia, is a rare disease with two known expansion types, unicentric and multicentric, which play a major role in determining therapy. We focus here on the unicentric type, which can be treated and cured by surgery. To date, approximately 1000 cases of Castleman’s disease have been reported in the literature.Case presentationA 50-year-old Caucasian woman presented to our Department of Hematology and Internal Oncology with increasing fatigue as her sole symptom. Diagnostic investigations including laboratory studies, ultrasound, computed tomography and magnetic resonance imaging were performed. These revealed an interaortocaval, retroperitoneal tumor mass in her upper abdomen as the only manifestation of the disease. No enlarged lymph nodes were detected. We conducted a laparotomy with radical extirpation of the tumor mass (10×9×5.7cm). Complete tumor resection with clear margins was achieved. A pathological analysis of the resected sample showed atypical lymphoid tissue of small to medium cells with some clearly visible nucleoli, enlarged sinusoidal vessels, pleomorphic calcifications and focally preserved germinal-center-like structures. Histological and immunohistochemical analysis confirmed the diagnosis of Castleman’s disease: staining for CD3, CD5, CD10, CD20, CD23, CD79 and Ki-67 was strongly positive in the germinal-center-like structures. Histological findings clearly showed the disease to be the hyaline vascular subtype. Staining for cyclin D1 and CD30 was negative. Expression of CD15 was positive in the enlarged sinusoidal vessels. A supplementary clonality analysis was without pathological findings. Tests for human immunodeficiency virus and human herpes virus 8 were negative and results from a bone marrow biopsy were normal. Our patient recovered well from surgery and was discharged from our hospital. To date, no recurrence of the disease has been detected.ConclusionCastleman’s disease is a rare disorder that remains a diagnostic challenge. Radical surgical resection is considered to be the gold standard for treating the unicentric variant of this disease.

ROLE OF RADIOTHERAPY IN THE MANAGEMENT OF RECURRENT CASTLEMAN\S DISEASE

Castleman’s disease is a benign and rare disease developing in lymph nodes. It is characterized by B-cell hyperproliferation and B-cells produce interleukin-6. This disease has two types; multicentric type often presents with fever, hepatomegaly, splenomegaly or lymphadenopathy, whereas unicentric type is diagnosed occasionally. Multicentric type is treated with combination chemotherapy regimens and unicentric type is treated with surgery and/or radiotherapy. In this article, a 29-year-old male patient with unicentric type, hyalen vascular Castleman’s disease with local recurrence after surgery and treated with radiotherapy is presented with management and related literature review.

Peripancreatic Unicentric Castleman's Disease: A Case Report

Castleman's disease is a morphologically distinct form of lymph node hyperplasia. The disease is rare and the etiology remains poorly understood. Three morphological variants of the disease are described- hyaline vascular, plasmacytic and a mixed variant. Clinically, the presentation of the disease is heterogeneous. Most often, the disease presents as a localized lymph node enlargement, referred to as the unicentric type and the multicentric form is rare. In unicentric disease, surgical resection is usually curative, however in the multicentric form the management is poorly defined and the disease has a more fatal outcome. We present a case of 48 year old female presenting with peripancreatic unicentric Castleman's disease involving peripoartal lymph nodes.

Castleman′s disease: A case report of the unicentric type

Castleman's disease, or angiofollicular lymph node hyperplasia, is a relatively rare disorder characterized by the benign proliferation of lymphoid tissue. Two clinical entities have been described: Unicentric with the disease confined to a single anatomic lymph node and multicentric characterized by generalized lymphadenopathy and more aggressive clinical course. Also, three histopathological subtypes have been described: Hyaline-vascular, plasma cell, and a mixed variant. Preoperative diagnosis of hyaline-vascular Castleman's disease is difficult, and the definitive result is based on postoperative pathological findings. The gold standard therapy is the complete surgical excision.

Castleman's Disease Mimicking Carotid Body Tumor

Castleman's Disease Mimicking Carotid Body TumorThe authors present a case of 41-year-old patient with unicentric type of Castleman's disease in the neck imitating carotid glomus tumor. This rare condition can affect any lymph-node in the body and neck localization occurs only in 14% of cases. The case when Castleman's disease simulating chemodectoma have not been described yet in the literature. The authors give details on the course of the disease, its diagnosis and treatment and the discussion focuses on the reasons why the auxiliary imaging methods have led to the clinical diagnosis of carotid glomus tumor, and why the treatment was chosen.