Castelman disease

Chordoid meningioma is classified in the latest classification of brain tumours as a grade II meningioma, along with large cell meningiomas. It is a rare tumour often associated with systemic symptoms, as described by Castelman. He described a case of chordoid meningioma discovered incidentally in a 45-year-old female patient following road accident. Brain CT scan showed left isodense intradiploid pterional lesion with bone lysis, which was homogeneously enhanced after injection of contrast medium. Magnetic resonance imaging showed T1-hypointense and spontaneously T2 FLAIR hyperintense lesion, which was very strongly enhanced after injection of Gadolinium...

BACKGROUND: In literature, a few reports described an association between paraneoplastic pemphigus (PNP) and Castelman's disease (CD), but no consensus have been proposed for the diagnostic-therapeutical approach. Aim of this study is to present a case report and explore the relationship between PNP and CD in pediatric patients, focusing on clinical manifestations, histopathological findings, treatment and outcome to find elements for an early diagnosis. CASE PRESENTATION: We present the clinical case of a 13 years old girl with a challenging diagnosis of PNP and CD who underwent therapy at first with Rituximab and then with Siltuximab, for the control of symptoms...

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Castleman's disease is a rare disease characterized by benign lymphoepithelial proliferation. There are two forms: unicentric and multicentric Castleman's disease. Mediastinal location is the most frequent. Intra-abdominal Castleman's disease is a rare presentation. We report a case of 65-year-old female who presented with epigastric pain. Investigations revealed a retroperitoneal mass which was surgically resected. Histopathological examination showed hyaline-vascular type Castleman's disease. In conclusion, Castelman´s disease is a diagnostic challenge and it must be included in the differential diagnosis of retroperitoneal tumors...

Diagnosing Behcet's disease (BD) in a patient already diagnosed with Castelman's disease (CD) is rare. There are only a few cases reported in the literature and all of them were diagnosed as BD prior to the patient experiencing symptoms and signs of CD. We present a patient who was initially diagnosed as having CD. However, after being managed with chemotherapy, specifically after the fourth cycle, the patient was found to have an incidental finding of pulmonary artery aneurysm, which led to the diagnosis of BD...

INTRODUCTION: Castleman's disease (CD) is a rare disorder characterized by benign angio follicular lymphoid hyperplasia, with gentle behavior. Its etiology and prevalence are unclear. It is usually found in the mediastinal area and it is rare to see this tumor in the retroperitoneum. PRESENTATION OF CASE: A 53 year old male with a retroperitoneal hypervascular calcified mass densely adherent to the psoas muscle that was accidentally encountered on a CT scan. Provisional diagnosis of retroperitoneal liposarcoma was made and the patient underwent an exploratory laparotomy...

Variants in MAGT1 have been identified as the cause of an immune deficiency termed X-linked immunodeficiency with magnesium defect, Epstein-Barr virus (EBV) infection and neoplasia (XMEN) disease. Here, we describe 2 cases of XMEN disease due to novel mutations in MAGT1, one of whom presented with classical features of XMEN disease and another who presented with a novel phenotype including probable CNS vasculitis, HHV-8 negative multicentric Castelman disease and severe molluscum contagiosum, thus highlighting the clinical diversity that may be seen in this condition...

Castleman's disease is a very rare and potentially severe lymphoproliferative disorder. First sign may be cervical lymphadenitis, requiring sufficient support in diagnosis and therapy by an ENT specialist. Based on a case series the current manuscript gives an overview of the symptoms, the course of disease and the therapy options. Patients with the first diagnosis of a Castleman's disease at the ENT clinic of Ulm University during the years 2011-2015 were included. The duration of symptoms, the applied diagnostic and therapeutic algorithms were evaluated...

UNLABELLED: The oncogenic herpesvirus Kaposi's sarcoma-associated herpesvirus (KSHV) is known to encode four viral interferon regulatory factors (vIRF1 to -4) to subvert the host antiviral immune response, but their detailed DNA-binding profiles as transcription factors in the host remain uncharacterized. Here, we first performed genome-wide vIRF2-binding site mapping in the human genome using chromatin immunoprecipitation coupled with high-throughput sequencing (ChIP-seq). vIRF2 was capable of binding to the promoter regions of 100 putative target genes...

Castleman disease (CD) is a benign lymphoepithelial disorder characterised by abnormal growth of a lymph node. This disease may be seen anywhere along the lymphatic chain but it usually occurs in the mediastinum. Two clinical types (localised and generalised) and three histopathological types (hyaline vascular, plasma cell, and mixed) have been described. We present a case of a 35-year-old female patient with parotid CD of the hyaline-vascular type as an extremely rare entity. We also discuss recent updates on this disease...

PURPOSE: To study the pattern of ocular adnexal lymphoproliferative disorders (OALD) in an ophthalmic referral center in Saudi Arabia and to review their, histopathological characteristics with clinical correlation. METHODS: Retrospective chart review of 40 cases of patients who underwent incisional biopsy with the suspected diagnosis of periocular and/or adnexal lymphoid lesions over the period: 2000-2012 at the King Khaled Eye Specialist Hospital (KKESH), Riyadh, Saudi Arabia...

IL-27 is an APC-derived IL-6/IL-12 family composite cytokine with multiple functions such as regulation of Th1, Th17, and regulatory T cell differentiation, B cell proliferation, and Ig class switching. The IL-27 complex is formed by the association of the cytokine p28 with the soluble cytokine receptor EBV-induced gene 3 (EBI3). The IL-27 cytokine and soluble receptor subunits p28 and EBI3 can be secreted independently. The p28 subunit has been shown to have IL-27-independent biological activities. We previously demonstrated that p28 can form an alternative composite cytokine with the EBI3 homolog cytokine-like factor 1 (CLF; CRLF1)...

BACKGROUND: Recombination is an important mechanism in the generation of genetic diversity of the human (HIV) and simian (SIV) immunodeficiency viruses. It requires the co-packaging of divergent RNA genomes into the same retroviral capsid and subsequent template switching during the reverse transcription reaction. By HIV-specific fluorescence in situ hybridization (FISH), we have previously shown that the splenocytes from 2 chronically infected patients with Castelman's disease were multi-infected and thus fulfill the in vivo requirements to generate genetic diversity by recombination...

OBJECTIVES: We undertook a systematic review of 404 published cases of Castleman's disease to identify the role of the surgeon beyond assistance in tissue-based diagnosis. BACKGROUND: Castleman's disease is a rare primary disease of the lymph node caused by infection with herpesviridae. Little is known about the role of surgery in this condition. DATA SOURCES: Medline, Embase, Cochrane Database of Systematic Reviews, ISI Thompson Web of Knowledge, and hand search of articles' bibliography...

The first case of endobronchial sarcoidosis was described by Benedict and Castelman in 1941. The endoscopic aspects in sarcoidosis can reveal: erythema, edema, network of capillary ectases, granularity, irregular mucosa thickening, macro and micro nodules in mucosa, bronchial and tracheal stenosis, distortions, tractions (including at the large and medium airways), sometimes bronchial ectases secondary to these modifications. The doctors must be aware of the possibility of affecting the great airways in sarcoidosis, such situation, although rare, could make for a difficult diagnosis, especially in the case of the tusigen syndrome or persistent and unexplained wheezing...

CK2 denotes a pleiotropic, constitutively active protein kinase whose abnormally high level in many cancer cells is held as an example of "non oncogene addiction". A wide spectrum of cell permeable, fairly specific ATP site-directed CK2 inhibitors are currently available which are proving useful to dissect its biological functions and which share the property of inducing apoptosis of cancer cells with no comparable effect on their "normal" counterparts. One of these, CX-4945, has recently entered clinical trials for the treatment of advanced solid tumors, Castelman's disease and multiple myeloma...

Paraneoplastic pemphigus is an autoimmune blistering disease associated with an occult or previously diagnosed tumor. Its clinical, histological, and immunological features have been clearly defined. It is characterized by the presence of polymorphic skin lesions and by erosions of the oral and genital mucosas that are refractory to conventional treatments. The histology can be variable and includes acantholysis or lichenoid dermatitis. Circulating autoantibodies are a constant feature and confirm the diagnosis...

We report a historical case of hyperparathyroidism in a young patient hospitalized for an array of osteolytic foci and incomplete fracture associated with a swollen neck, revealing a very special form of a metastatic parathyroid carcinoma with unusual multiple locations and exceptional medullary flooding. Carcinoma of the parathyroid gland produces a malignant hypersecreting tumor particularly difficult to diagnose. Treatment of this rare tumor is primarily surgical. The preoperative syndrome is unusually severe primary hyperparathyroidism...

KSHV infection is the causative agent in three different tumor types: Kaposi's sarcoma, a plasmablastic variant of multicentric Castelman's disease and an AIDS-related form of B cell lymphoproliferative disorder called primary effusion lymphoma (PEL). PEL manifests as an effusion malignancy in Kaposi's sarcoma patients with advanced AIDS, but also occurs in HIV-negative individuals. PEL is a very aggressive disease, and currently there are no efficient therapies for treating PEL. In our recent paper we report that p53 reactivation by a small molecule inhibitor of p53-MDM2 interaction, Nutlin-3a, induces selective and massive apoptosis in PEL cells, and has striking anti-tumor activity in a mouse xenograft PEL model...

Inflammatory liposarcoma represents a rare variant of well-differentiated liposarcoma in which a chronic inflammatory infiltrate predominates simulating the appearance of malignant lymphoma and other nonneoplastic lesions. A case of this lesion occurring in the retroperitoneum was described in 43-year-old man. The differential diagnosis, especially from lymphoma and non neoplastic lesions such as inflammatory myofibroblastic tumor and Castelman disease are discussed.