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Castleman's disease is a rare lymphoproliferative disease that usually presents as a solitary mass in the mediastinal or cervical region. Castleman's disease can be usually of two types: unicentric type (which involves only one site of lymph nodes) and multicentric type (which involves multiple sites of lymph nodes). We report the case of a 26-year-old female with multiple sclerotic bone lesions in unicentric Castleman's disease. The definitive diagnosis was made by excisional biopsy with immunohistochemistry, 18F-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) study, and MRI scan. This case report emphasizes the need for proper workup for systemic manifestations in unicentric Castleman's disease.
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Castleman disease is a rare type of lymph node hyperplasia primarily affecting the mediastinum, with mesenteric localization being extremely uncommon. It is classified into solitary and multicentric forms. In this case report, we present the case of a 46-year-old female patient in whom an incidental mesenteric mass was discovered during the workup for a ventral hernia. The mass was completely excised, and the histopathological examination confirmed the diagnosis of mixed-type Castleman disease. Surgery is the treatment of choice for localized forms of this condition, and histological examination is crucial in confirming the diagnosis.
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Castleman disease (CD) is a benign lymphoproliferative disorder of unknown etiology, which can involve any part of the body. CD can involve a single lymph node (unicentric) or multiple lymph nodes (multicentric) of which unicentric CD is the most common type. The unicentric CD is usually localized, asymptomatic, and often appears as an incidental mass on radiographs, whereas multicentric CD is characterized by systemic involvement. Mesenteric involvement of CD is very rare. In this article, we present a case of the unicentric CD of small bowel mesentery, which mimicked a neuroendocrine tumor preoperatively.
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A patient with a large neck mass underwent appropriate imaging, routine blood tests, and serological evaluations. The medical history revealed the patient had undergone a lymphadenectomy in the same region 8 years ago, and the pathological diagnosis was the hyaline-vascular subtype of unicentric Castleman's disease (UCD). The incisional biopsy and subsequent histopathological and immunohistochemical examination revealed the diagnosis of follicular dendritic cell sarcoma, consistent with the malignant transformation of UCD. UCD is uncommon and the malignant transformation of UCD is extremely rare in the head and neck region. Regional lymph node resection of one or more adjacent regions is the preferred treatment choice. Appropriate treatment procedures for UCD and regular follow-up are essential for a good prognosis. Laryngoscope, 2024.
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Castleman's disease is a rare benign lymphangioproliferative disorder. The hyaline vascular subtype has a better outcome and is curable after surgical resection. Typically, Castleman disease manifests in the thorax, with rare reports of a renal hilum location. We present a 42-year-old male patient who had an incidentally detected right hilar hyaline vascular type of Castleman's disease, which we managed with surgical excision. Cross-sectional imaging modalities help in suggesting the diagnosis based on enhancement patterns and, more importantly, define the extent of the tumor pre-operatively. Although the renal hilum is a rare location for Castleman disease, it needs to be considered when imaging features suggest it.
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INTRODUCTION: Castleman Disease (CD) is a rare benign disorder characterized by abnormal lymphoid tissue growth. It can be classified as unicentric CD (UCD) affecting a single lymph node region or multicentric CD (MCD) involving multiple regions. While UCD typically occurs in the mediastinum, MCD is associated with inflammatory symptoms and abnormal lab findings. We present a case of a young Syrian male with pelvic UCD, exhibiting unusual symptoms. Surgical excision led to complete recovery. CASE PRESENTATION: A 27-year-old male presented with a well-defined pelvic mass discovered during routine ultrasonography. Symptoms included fever, fatigue, weight loss, and lower extremity numbness. Laboratory analysis revealed lymphocytopenia. Imaging identified a retroperitoneal pelvic mass, raising concerns of lymphoma. Surgical resection confirmed Castleman disease, specifically the hyaline vascular variant, dispelling malignancy concerns. Post-surgery, the patient demonstrated complete recovery, with normalized lymphocyte count and resolution of symptoms, affirming the effectiveness of the treatment. DISCUSSION: Castleman Disease (CD) is characterized by abnormal lymphoid tissue growth. It presents as Unicentric (UCD) or Multicentric (MCD) forms. UCD cases are usually asymptomatic or show compressive symptoms due to mass effect, while MCD is associated with systemic symptoms. The estimated UCD incidence is 16 cases per million person-years, with median age at diagnosis around 30-34 years. Histologically, UCD is categorized into hyaline vascular (HV) and plasma cell (PC) variants. In this case, systemic symptoms and lymphocytopenia deviated from typical UCD presentation, leading to initial lymphoma suspicions. Surgical intervention facilitated recovery without adjuvant therapies. CONCLUSION: This study emphasizes the spectrum of Castleman Disease (CD) manifestations, distinguishing Unicentric (UCD) and Multicentric (MCD) forms crucial for accurate management. It highlights atypical pelvic UCD presentation and successful surgical treatment's importance.
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Lymphoproliferative disorders comprise of a group of heterogenous diseases characterised by uncontrolled production of lymphocytes.Castleman disease (CD) is one such disease and comprises of a group of rare disorders that involve enlarged lymph nodes and a broad range of inflammatory symptoms and laboratory abnormalities [1]. We came across young girl who presented to us in the out patient department with this disease After ruling out other pathologies and confirming the diagnosis by doing a tissue biopsy the girl was treated surgically and is disease free till date.
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Introduction: Castleman's disease (CD), or benign angio-follicular lymph node hyperplasia, is an uncommon condition in childhood. When a child presents with a huge retroperitoneal mass and inconclusive findings on clinico-radiological evaluation or tissue sampling, management becomes exceedingly difficult. CD herein becomes an uncommon presentation of an uncommon diagnosis. Case presentation: A six-year-old girl with no past medical problems presented to the office with a slowly progressive, painless mass over the right lumbar region for a year. Abdominal ultrasound showed a well-defined oval mass in the right periumbilical region, further evaluation of which with a computed tomography scan suggested lymphoma. A preoperative core-cut biopsy could not confirm the findings and suggested a neoplastic lesion, probably an inflammatory myofibroblastic tumour or small round cell tumour. She underwent an exploratory laparotomy with in-toto excision of the mass. Intraoperatively, a solid retroperitoneal tumour measuring 8×8×6 cm was found. Histopathology and immunohistochemistry confirmed a unicentric CD of the hyaline-vascular type. At two years of follow-up, she remained asymptomatic and disease-free. Conclusion: While CD in children is rare, retroperitoneal localization of the same can further add to the diagnostic conundrum. However, if carefully considered, an en-bloc surgical resection offers complete treatment.
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Castleman disease is a rare and benign disorder, characterized by enlarged lymph nodes and angiofollicular lymphoid hyperplasia. We report a case of a 57-year-old male, who was admitted to our surgical department because of a retroperitoneal nodular mass measuring about 4 cm in maximum diameter, incidentally discovered on a radiologic exam performed for the onset of vague abdominal pain with posterior irradiation. The patient was subdue to laparoscopic removal of the mass and no intra- and post-operative complications were recorded. Histologic diagnosis of hyaline-vascular variant of the Castleman disease was confirmed. Only two cases have been found in the literature reporting the paraduodenal unicentric Castleman disease localization like our case. Although rare, the Castleman disease must be considered in the differential diagnosis among all the lymph nodes diseases, for avoiding improper therapies.
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Unicentric Castleman disease (UCD) is a rare lymphoproliferative disorder. Paraneoplastic pemphigus (PNP) is a major complication associated with poor UCD prognosis. However, the genomic profiles and prognostic biomarkers of PNP-associated UCD remain unclear. In this study, we performed whole-exome sequencing analysis for 28 matched tumor-normal pairs and 9 tumor-only samples to define the genomic landscape of Chinese patients with PNP-associated UCD. An integrative analysis was performed to identify somatic variants, the mutational signatures, and key pathways in tumors. Besides, we analyzed the relationship among mutated genes, clinical characteristics, and prognosis. Sixty-one somatic mutant genes were identified in >1 patient with PNP-associated UCD. Specifically, IL6ST and PDGFRB were the most frequently mutated genes (32%), followed by DPP6 (18%) and MUC4 (18%). Signaling molecules and interactions, cellular processes, and signal transduction pathways were enriched. Furthermore, we found that poor overall survival was related to IL6ST variants (P = .02). Finally, we classified PNP-associated UCD into 4 genomic subgroups: IL6ST, PDGFRB, IL6ST-PDGFRB, and an unknown subgroup. In summary, we defined the molecular profile of PNP-associated UCD and identified a potential molecular biomarker for predicting prognosis, which may provide therapeutic targets for treating this severe disorder.
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Hiperplasia do Linfonodo Gigante , Síndromes Paraneoplásicas , Pênfigo , Humanos , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/genética , Hiperplasia do Linfonodo Gigante/complicações , Pênfigo/genética , Prognóstico , Sequenciamento do Exoma , Receptor beta de Fator de Crescimento Derivado de Plaquetas , Síndromes Paraneoplásicas/diagnóstico , Síndromes Paraneoplásicas/genética , Biomarcadores , Receptor gp130 de CitocinaRESUMO
BACKGROUND: Castleman's disease is a rare and benign lymphoproliferative disorder which can be unicentric (UCD) or multicentric (MCD). UCD usually involves a single lymph node or less frequently a group of lymph nodes. The most common sites of nodal UCD presentation are the mediastinum, neck, abdomen and retroperitoneum. Rarely extranodal involvement has been reported. The intramuscular location is very unusual with only about 10 cases described in medical literature so far. CASE REPORT: We present a case of atypical localization of Castleman's disease occurring in the right gluteal area in a 40-years-old female patient. The patient was asymptomatic and clinical examination was unremarkable except for a right gluteal palpable mass. The CT scanner-guided needle core biopsy was inconclusive. A surgical excision was then performed that revealed a hyaline-vascular type of Castleman's disease. The patient has an uneventful post-operative course. CONCLUSION: The present case is instructive in the work-up of primary soft tissue tumors, for which Castleman's disease is extremely rare and not considered in the differential diagnosis of clinicians. Pathologists must be aware of its existence so that it can be evoked in the presence of a lymphoid population on histological examination.
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Hiperplasia do Linfonodo Gigante , Humanos , Feminino , Adulto , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/cirurgia , Hiperplasia do Linfonodo Gigante/patologia , Linfonodos/patologia , Biópsia , Mediastino/patologia , Diagnóstico DiferencialRESUMO
Unicentric Castleman disease, particularly the hypervascular variant subtype, commonly presents as a localized lymphadenopathy without systemic symptoms. Surgical excision is often curative for this subtype, leading to a good prognosis. However, some patients with autoimmune complications may require additional systemic therapy along with surgery. Accurate diagnosis through a combination of clinical, radiological, and pathological findings is crucial for optimal management.
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Fundamento: La enfermedad de Castleman es un proceso poco común y se caracteriza por la proliferación de linfocitos no clonales. Objetivo: Describir la presentación clínica, diagnóstico y tratamiento de un paciente con enfermedad de Castleman. Presentación del caso: Paciente masculino de 53 años de edad, color de la piel blanca, que acudió al servicio de cirugía por presentar una masa en región abdominal. Con la administración de anestesia general se realizó exéresis de la lesión y se diagnosticó por el departamento de Anatomía Patológica una enfermedad de Castleman unicéntrica variedad hialino vascular. Conclusiones: La enfermedad de Castleman es poco frecuente, su sintomatología y tratamiento varían según la presentación clínica; y el diagnóstico definitivo se obtiene del análisis de la biopsia de un ganglio afectado.
Background: Castleman disease is an uncommon process and is characterized by the non-clonal lymphocyte proliferation. Objective: To describe the clinical presentation, diagnosis and treatment in a patient with Castleman disease. Case presentation: 53 years old male patient, fair skin color, who attended to the surgery service for presenting a mass in the abdominal region. With the general anesthesia administration, the lesion was excised and an unicentric Castleman disease was diagnosed by the Pathological Anatomy department, hyaline vascular variety. Conclusions: Castleman disease is not frequent, its symptomatology and treatment vary according to the clinical presentation; and the definitive diagnosis is obtained by a biopsy analysis of an affected ganglion.
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Castleman disease (CD) describes a group of rare heterogeneous lymphoproliferative disorders characterized by enlarged hyperplastic lymph nodes. It is classified into unicentric CD (UCD) and multicentric CD (MCD). The present retrospective study examined the data of 11 patients with CD diagnosed and treated at a tertiary cancer center from 2017 to 2022. The median age of the study group was 41 years (range, 24 to 68 years). There were 8 males and 3 females. In total, 7 patients were diagnosed with UCD and 4 patients with MCD. The hyaline-vascular variant was the most common histology in both UCD and MCD. Among the 7 patients with UCD, 5 patients underwent excision, 1 patient underwent debulking followed by radiotherapy and 1 patient received single agent rituximab. Of the patients with UCD, 6 had a complete response (CR) and 1 patient had a partial response (PR). All 4 patients with MCD received systemic treatment, which included single agent rituximab (2 patients), rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (RCHOP) (1 patient) and CHOP (1 patient). Among the patients with MCD, 1 patient attained a CR, 2 patients had a PR and 1 patient succumbed. The 3-year survival rate for the study population was 91%. In summary, CD is a rare disease occurring in immunodeficient patients. UCD is more common and is associated with better outcomes. Surgery is the mainstay of management in UCD whereas MCD requires combination chemotherapy.
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Castleman disease (CD) is a rare lymphoproliferative disease. Hyaline-vascular type unicentric CD has a good prognosis if completely resected during surgery. However, follicular dendritic cell proliferative lesions have the potential for recurrence and metastasis. A 22-year-old man was referred to our hospital with the chief complaint of nausea and vomiting. These symptoms were caused by a right mesocolonic tumor pushing the duodenum. The patient underwent laparoscopic tumorectomy and complete surgical excision. The postoperative course was uneventful, with no complications. Pathological examination confirmed that the tumor was an enlarged lymph node, typical of hyaline vascular-type CD; however, follicular dendritic cell proliferative lesions were noted. We report a rare case of hyaline-vascular-type CD with follicular dendritic cell proliferative lesions associated with malignancy, as limited case reports exist on this particular disease.
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Castleman disease (CD) is a rare lymphoproliferative disorder of undetermined etiology. Unicentric CD (UCD) and multicentric CD (MCD) are two phenotypes of CD diagnosed by the histopathology of lymph nodes. We attempted to describe a pediatric CD cohort to optimize the management of this disease. We reviewed the medical records of pediatric patients diagnosed with CD between April, 2004, and October, 2022, at the Children's Hospital of Fudan University. Prognosis information was collected in January, 2023, by telephone inquiry. Twenty-two patients with UCD and 2 patients with MCD were identified, all with hyaline vascular (HV) type. The median ages at diagnosis were 10.75 years (IQR 8, 12.81) for UCD and 14.42 years (IQR 13.42, 15.42) for MCD. The most common lesion location of UCD was the neck (9/22, 40.91%) and abdomen (9/22, 40.91%). Systematic symptoms occurred on 10/22 (45.45%) patients with UCD and 1/2 (50%) patients with MCD, and abnormal laboratory indexes were detected in both. Resection and biopsy were performed on all patients. One out of two patients with MCD also received rituximab for upfront therapy. After a median of 4 years (IQR 1.5, 6) of follow-up time, the overall survival was 100% and the complete remission rate in UCD was 63%. There was no relapse or progression. CONCLUSIONS: Our series demonstrated that HV-UCD was the most common type in children. Resection and biopsy were used for both deterministic diagnoses and treatments. Despite the high possibility to develop systematic inflammation, children with CD showed promising outcomes. WHAT IS KNOWN: ⢠Castleman disease is a rare lymphoproliferative disorder with limited cohort studies, especially in pediatrics. ⢠The ubiquity of delayed confirmations and misdiagnoses points to a lack of knowledge about etiology and characteristics, which is a prerequisite for novel therapeutics. WHAT IS NEW: ⢠We retrospectively reviewed and analyzed the clinical and pathological symptoms, laboratory and imaging features, and treatment outcomes of a Chinese pediatric cohort with Castleman disease. ⢠Our work may improve the recognition and optimize the management of this rare disease in children.
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Hiperplasia do Linfonodo Gigante , Humanos , Criança , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/terapia , Hiperplasia do Linfonodo Gigante/patologia , Estudos Retrospectivos , Linfonodos/patologia , Resultado do Tratamento , ChinaRESUMO
INTRODUCTION: Castleman's disease is an extremely rare disease in the abdomen region characterized by benign lymphoepithelial proliferation. CASE PRESENTATION: We report a case of a 63-year-old female who presented with abdominal pain. Abdominal CT Angio and dynamic contrast-enhanced abdominal MRI revealed a mass lesion showing markedly contrast enhancement, no vascular invasion sign, and diffusion restriction lesion in the truncus coelicus bifurcation region. The mass was surgically resected completely. Pathological evaluation showed a hyaline-vascular type of Castleman's disease. CONCLUSION: Castleman's disease should be kept in mind in the differential diagnosis of an isolated intra-abdominal mass.
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Castleman disease (CD) is a rare lymphoproliferative disorder that is associated with an increased risk for lymphoma. The association between CD and classical Hodgkin lymphoma (HL) is rare. The patient described here is a 44-year-old, HIV-seronegative male who presented with significant weight loss, fever, night sweats, and right axillary swelling. Imaging showed bulky infraclavicular, subpectoral, and axillary lymph nodes. A biopsy revealed classical HL on the background of a human herpesvirus-8 (HHV-8)-negative plasma cell variant of CD. The patient had a complete remission after six cycles of doxorubicin, bleomycin, vincristine, and dacarbazine (ABVD) that were followed by consolidative radiotherapy and continued to be disease-free for more than two years.
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Background: Unicentric Castleman's disease (UCD), a lymphoproliferative disorder characterized by enlargement of the lymph nodes, is a rare cause of Amyloid-A amyloidosis. While patients usually present with impaired kidney function and proteinuria, heart involvement is neither common nor the main cause of signs and symptoms. Case summary: We present a patient who was admitted to the hospital for impaired exercise capacity. Diagnostic work-up revealed severe left ventricular hypertrophy suggestive of cardiac amyloidosis. Although Congo red staining of endomyocardial biopsies was initially negative, subsequent immunohistochemical staining against serum amyloid A finally confirmed the diagnosis of cardiac amyloidosis. 18F-fluorodeoxyglucose positron emission tomography/computed tomography revealed a tumour located in dorsal of the duodenum. Fine-needle aspiration biopsy of the tumour was suggestive but could not confirm the presence of UCD beyond reasonable doubt. Rapid worsening of heart failure symptoms warranted urgent surgical tumourectomy, which resulted in immediate post-operative lowering of serum amyloid protein. However, post-operative cardiogenic shock could not be stabilized even with veno-arterial extracorporeal membrane oxygenation, and the patient eventually died. The UCD of the hyaline vascular (HV) subtype was confirmed by pathologic work-up of the excised tumour. Discussion: This case report presents for the first time a patient with malignant cardiac Amyloid-A amyloidosis caused by unicentric Castleman's disease of the HV subtype. Since the disease progresses swiftly, rapid diagnosis is essential for potential curative treatment.
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Castleman disease is an uncommon lymphoproliferative disorder, located most commonly in the mediastinal lymph nodes. The intrapulmonary presentation is extremely rare, with 14 published cases in the English literature. The clinical presentation of the disorder is not specific and the diagnosis is often challenging. The main goal is to achieve an R0 surgical resection which gives the potential chance for a recurrence-free survival. We present the case of a symptomless, 15-year-old female patient with left-sided tumor mass. Transthoracal invasive tissue biopsy confirmed Castleman disease. The central mass involved the main structures in the left hilus and therefore left pulmonectomy was necessary to reach the complete, tumor-free resection margins. The patient had no local or distant relapse during the 7-year follow-up. Although Castleman disease treated by complete surgical resection provides excellent results, radical and extended lung resection is sometimes inevitable to reach tumor-free margins. Orv Hetil. 2023; 164(37): 1476-1483.