Hyaline-vascular Castleman's Disease Research Articles

Clinicopathological characteristics of mediastinal follicular dendritic cell sarcoma: report of three cases

BackgroundFollicular dendritic cell sarcoma (FDCS) is a rare malignant neoplasm of follicular dendritic cells that form a tight meshwork within lymphoid follicles. It occurs most commonly in the lymph nodes and occasionally at extranodal sites, but rarely in the mediastinum. It is an under-recognized clinical entity without clear diagnosis. Due to its rarity, FDCS is easily misdiagnosed by clinicians or pathologists.Case PresentationHerein, we report three unusual cases of mediastinal FDCS, including one with hyaline vascular Castleman’s disease in woman. The clinicopathological characteristics and CT imagings were described. Their diagnosis was confirmed by immunohistochemical stainings of specific markers. Their therapeutic intervention, follow-up and outcomes were presented with brief literature review.ConclusionsA huge mediastinal shallowly-lobulated, demarcated soft tissue mass, with speckled, strip-like, coarse or arborizing calcification inside, and mild to moderate enhancement after contrast material administration on CT image, should consider FDCS as a possible diagnosis.

Intra-abdominal localized hyaline-vascular Castleman disease: imaging characteristics and management of a rare condition.

Castleman disease (CD) is a rare lymphoproliferative disorder of unknown etiology and one of the uncommon causes of nonneoplastic lymphadenopathy. Earlier synonyms of CD included angiofollicular lymph node hyperplasia, giant lymph node hyperplasia, lymphoid hamartoma, benign lymphoma and follicular lymphoreticuloma. This disease was first reported by Castleman et al. in 1954 [1] and later defined by Castleman et al. in 1956 in a series of 13 patients with unicentric hyaline vascular CD presenting with large thymoma-like masses in the anterior mediastinum [2]. The condition was later found to be occasionally associated with hypochromic anemia, hypergammaglobulinemia and bone marrow plasmacytosis [3, 4]. By the late 1960s and the early 1970s, the histological features of the different forms of the disease and the classification in current use had been established [5, 6]. The disease features three main histopathological presentations: hyaline vascular, plasma cell-rich, and mixed variants [6, 7]. The exact incidence of CD is unknown. Association of certain forms of the disease with the human immunodeficiency virus (HIV) and human herpes virus-8 (HHV-8) infections has been long recognized. In 1985, Lachant et al. reported on 2 patients with the acquired immunodeficiency syndrome (AIDS) who developed multicentric CD followed by Kaposi sarcoma [8]. In 1995, Soulier et al. reported frequent coincidence of HHV-8 in 31 patients with multicentric CD [9].

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.

Unicentric mesenteric Castleman's disease with littoral cell angioma, anemia, growth retardation and amenorrhea: A case report.

Castleman’s disease (CD) is a rare lymphoproliferative disorder of unknown origin, and littoral cell angioma (LCA) is a rare vascular tumor of the spleen with an unknown etiology. The current study reports the case of a 28-year-old female who presented with anemia, growth retardation and amenorrhea. Physical examination revealed a mass in the mesentery, splenomegaly with multiple small nodules, hepatomegaly and an infantile uterus. Histopathological analysis of the resected mass and spleen confirmed the diagnosis of hyaline-vascular CD and LCA. The patient’s anemia resolved, and menstruation and breast development also commenced following surgery. To the best of our knowledge, this is the first report of CD accompanied by littoral cell angioma, anemia, growth retardation and amenorrhea.

Preoperative Embolization of a Hyaline Vascular Castleman Disease Tumor Located in the Mediastinum

Preoperative Embolization of a Hyaline Vascular Castleman Disease Tumor Located in the Mediastinum

Monoclonality and cytogenetic abnormalities in hyaline vascular Castleman disease

Hyaline vascular Castleman disease is traditionally regarded as a reactive hyperplastic process. Occasional cases, however, have been reported with cytogenetic anomalies bringing this concept into question. In this study, we used conventional and methylation-specific polymerase chain reaction methods to assess the human androgen receptor α (HUMARA) gene in 29 female patients with hyaline vascular Castleman disease and compared the results with three cases of plasma cell Castleman disease and 20 cases of age-matched lymphoid hyperplasia. We also assessed for immunoglobulin gene and T-cell receptor gene rearrangements, and conventional cytogenetic analysis was performed in three cases of hyaline vascular Castleman disease. In cases with informative results, conventional and methylation-specific human androgen receptor α gene analyses yielded a monoclonal pattern in 10 of 19 (53%) and 17 of 23 (74%) cases of hyaline vascular Castleman disease, respectively. A monoclonal pattern was also detected in three cases of plasma cell Castleman disease but not in cases of lymphoid hyperplasia. The frequency of monoclonality was higher for lesions >5 cm in size (100%) and for the stromal-rich variant (91%). Cytogenetic abnormalities in stromal cells were revealed in two cases of hyaline vascular Castleman disease and no cases showed monoclonal immunoglobulin or T-cell receptor gene rearrangements. Follow-up data showed persistent disease in 4 of 23 (17%) patients. We conclude that hyaline vascular Castleman disease is often a monoclonal proliferation, most likely of lymph node stromal cells.

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.

Clinical value of 18 F-FDG PET/CT in the management of Castleman's disease

Objective To assess the value of 18F-FDG PET/CT in clinical classification,monitoring of chemotherapeutic response and surveillance of histopathological transformation of Castleman's disease (CD).Methods Fourteen pathologically diagnosed CD patients (7 males,7 females; mean age:(45.64±14.30) years) were retrospectively reviewed.18F-FDG PET/CT was performed before chemotherapy in all patients and 4 of 14 patients were reexamined after the treatment.The study parameters included histopathological results,sites,number and highest SUVmax of the lesions.Mann-Whitney and Kruskal-Wallis tests were used for data analysis.Results Of all the 12 patients without histopathological transformation,one or more enlarged and metabolically active lymph nodes were found in each patient (SUVmax =3.94± 1.44,range:1.9-6.8),including 2 unicentric CD (UCD) and 10 multicentric CD (MCD).There was no statistically significant difference of SUVmam between UCD and MCD (4.55±3.18 vs 3.82±1.14; Z=0.22,P＞0.05).There was also no significant difference of SUVmax among different pathological types (hyaline-vascular CD (4/12):3.56±0.96,plasma cell CD (6/12):4.73±1.41,mixed CD (2/12):2.30±0.57; x2 =4.74,P＞0.05).For the 4 patients with follow-up PET/CT after chemotherapy,the lesion activity was normalized in 3 patients and clearly reduced in 1 patient.The SUVmax of 2 patients with histopathological transformation (10.85±2.05) was significantly higher than that without transformation (3.94± 1.44; Z=-2.19,P＜0.05).Conclusion 18F-FDG PET/CT may play an important role in clinical classification,monitoring of chemotherapeutic response and surveillance of histopathological transformation of CD. Key words: Giant lymph node hyperplasia; Tomography, emission-computed; Tomography, X-ray computed ; Deoxyglucose

T-lymphoblastic proliferation and florid multifocal follicular dendritic cell proliferation occurring in hyaline-vascular Castleman disease in a patient with a possible familial predisposition

Hyaline-vascular Castleman disease (HVCD) often presents as unicentric disease which is usually cured by surgical resection. Here, we report a case of a 23-year-old man with HVCD in the mediastinum, in which an apparently indolent T-lymphoblastic proliferation and extensive organoid proliferation of follicular dendritic cells were also present. The patient had no systemic involvement and no further disease at follow-up. In addition, there is a possible unique family history of HVCD. This case highlights an interesting combination of elements, mostly likely benign in nature.

Follicular dendritic cell sarcoma presenting as a neck mass, arising in background of castleman’s disease in cervical lymph nodes

Follicular dendritic cell sarcoma is a rare tumour of intermediate malignant potential. Hyaline vascular Castleman's disease is known to be a precursor lesion in a subset of patients. We present a case of a 39-year-old female who presented with a right neck lump. CT scan showed a 9 cm mass in the right neck extending into the superior mediastinum. Open biopsy showed the lesion to be composed of spindle cells arranged in a fascicular to storiform pattern, with interspersed lymphocytes. Immunohisto-chemistry showed staining for CD21 and CD23, consistent with the diagnosis of follicular dendritic cell sarcoma. The patient underwent subsequent surgical resection. Hyaline vascular Castle-man's disease was found in the adjacent cervical lymph nodes.

Transformation of Castleman's Disease into Follicular Dendritic Cell Sarcoma, Presenting as an Asymptomatic Intra-abdominal Mass

Follicular dendritic cell (FDC) sarcoma is an extremely rare malignant neoplasm arising from FDCs. The exact origin of FDCs remains unclear; both a hematopoietic lineage origin and a stromal cell derivation have been proposed. Proliferation of FDCs can lead to benign reactive lesions or generate neoplastic conditions. The lesions are most commonly found in lymph nodes and usually involve the head and neck area. Castleman's disease is a rare non-neoplasitic lymphoproliferative disorder. Rare cases of hyaline-vascular Castleman's disease have been associated with FDC sarcoma, but a clonal relationship has not been convincingly demonstrated. A pathway toward tumor evolution, beginning with hyperplasia and dysplasia of FDCs, has been proposed. Despite this known association between Castleman's disease and FDC sarcoma, there have only been few reported cases of sarcoma arising as a complication of pre-existing Castleman's disease, especially in abdominal lesions. We describe here a 51-year-old female with an FDC sarcoma arising from unicentric, hyaline-vascular type Castleman's disease in an intra-abdominal mass. Pathologically, the lesion showed a series of changes during the process of transformation from Castleman's disease to FDC sarcoma. (Korean J Gastroenterol 2013;62:131-134).

The number and growth pattern of plasmacytoid dendritic cells vary in different types of reactive lymph nodes: an immunohistochemical study

Plasmacytoid dendritic cells, which play a fundamental role in the innate immune response, are best known for their presence in hyaline-vascular Castleman disease and histiocytic necrotizing lymphadenitis. The relative number and distribution in many reactive entities as detected using more sensitive methods are uncertain, and their diagnostic implications are unknown. Immunohistochemical studies for plasmacytoid dendritic cell-associated markers CD123 and CD2AP were performed on 42 lymph nodes with hyaline-vascular Castleman disease, histiocytic necrotizing lymphadenitis, sarcoidosis, necrotizing granulomatous inflammation, viral infection, dermatopathic lymphadenopathy, autoimmune disease, and a histologic pattern compatible with toxoplasmosis. The overall plasmacytoid dendritic cell numbers and growth patterns (tight aggregates, loose aggregates/clusters, scattered single cells) were assessed. Plasmacytoid dendritic cells were present in all cases and were predominantly distributed in loose aggregates/clusters or singly. They were most numerous in granulomatous inflammation and histiocytic necrotizing lymphadenitis, whereas viral infections showed the fewest overall numbers and a predominant pattern of scattered single cells. Tight aggregates of plasmacytoid dendritic cells were most numerous in hyaline-vascular Castleman disease (100% sensitive, 68% specific). Plasmacytoid dendritic cells are not limited to a small number of reactive lymphadenopathies but are found in many reactive processes, often with a predominant pattern of loose aggregates/clusters and scattered single cells. However, tight aggregates were a characteristic feature of hyaline-vascular Castleman disease, and viral infections typically showed only few scattered cells distributed singly.

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 related to the chronic human herpes virus 8 (HHV-8) infection and the human immunodeficiency virus (HIV). 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. 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.

Hyaline Vascular Castleman Disease Involving Renal Parenchyma and a Lymph Node: A Case Report

Castleman disease is a rare lymphoproliferative lesion that is predominantly found in the mediastinum. Retroperitoneal and pararenal localizations are very rare. We describe a 36-year-old man with a hyaline vascular type of Castleman disease involving renal parenchyma and a paraaortic lymph node. Most reported renal Castleman disease was plasma cell type with systemic symptoms. Herein, we report the first Korean case of the hyaline vascular type of Castleman disease involving the renal parenchyma and the paraaortic lymph node simultaneously.

Castleman Disease: The Great Mimic

Castleman disease is a nonclonal lymphoproliferative disorder and one of the more common causes of nonneoplastic lymphadenopathy. Because of its diverse manifestations and ability to affect any body region, Castleman disease is a great mimic of both benign and malignant abnormalities in the neck, chest, abdomen, and pelvis. Castleman disease most commonly manifests as unicentric disease (unicentric Castleman disease) with a hyperenhancing lymph nodal mass and should be considered in the differential diagnosis of lymphoma, metastatic adenopathy, and infectious and/or inflammatory diseases that result in adenopathy. Castleman disease includes a spectrum of pathologic variants, including the classic hyaline vascular type, the less common plasma cell variant of Castleman disease, and the more recently described multicentric Castleman disease and Castleman disease associated with human herpesvirus 8. Castleman disease has been associated with the human immunodeficiency virus, lymphoma, POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome, paraneoplastic pemphigus, and plasma cell dyscrasias. Aggressive forms of Castleman disease with systemic manifestations may occur. Unicentric hyaline vascular Castleman disease is often curable with surgery; however, multicentric Castleman disease may require steroid treatment, chemotherapy, antiviral medication, or the use of antiproliferative regimens because a surgical procedure cannot be curative in this setting. Supplemental material available at http://radiographics.rsna.org/lookup/suppl/doi:10.1148/rg.316115502/-/DC1.

Folliculocentric B-cell–rich follicular dendritic cells sarcoma: a hitherto unreported morphological variant mimicking lymphoproliferative disorders

We report three cases of follicular dendritic cell sarcoma (FDCS) showing a hitherto undescribed histological pattern consisting of nodular tumor growth associated with small B lymphocytes. FDCS tumor cells consistently showed large epithelioid features and were intermingled with small lymphocytes in the nodules in two cases, whereas they formed cohesive aggregates surrounded by lymphocyte mantle in the other. These features were easily confused with lymphomatous proliferations and, in particular, subtypes of Hodgkin lymphoma, high-grade follicular lymphoma, and germinotropic large B-cell lymphomas. The diagnosis was established by the use of a broad panel of antibodies that showed a variable expression of the FDC markers CD21, CD23, CD35, clusterin, podoplanin, claudin 4, epidermal growth factor receptor, and CXCL13. The associated B lymphocytes revealed a mantle zone B phenotype, with expression of CD20 and PAX5, together with TCL1 and IgD. Of notice, in all cases, morphological features suggesting hyaline-vascular Castleman disease were recognized in the interfollicular areas, containing scattered epithelioid cells similar to those found in the nodules, thus providing a useful clue for FDCS diagnosis. Of the 3 cases, 1 presented multiple recurrences unresponsive to chemotherapy and radiotherapy and finally died of disease 14 years after diagnosis. This study further emphasizes the extreme variability of morphological presentation of FDCS and expands the spectrum of lesions showing a nodular growth pattern occurring in human lymph nodes.

The value of MDCT in diagnosis of hyaline-vascular Castleman's disease

PurposeCastleman's disease (CD) is an uncommon entity characterized by a massive growth of lymphoid tissue. There are two types: the hyaline-vascular (HV) type and the plasma cell (PC) type. The purpose of this study was to evaluate the clinical value of multiple detector computed tomography (MDCT) in the diagnosis and planning of treatment for hyaline-vascular CD. Materials and methodsFifty-two cases of confirmed hyaline-vascular CD were retrospectively reviewed. Unenhanced and contrast-enhanced MDCT scans had been performed in all patients, followed by surgery and pathological analysis of the lesion. Original MDCT transverse and reconstructed images were used for image interpretation. Features of the lesion and its adjacent structures were identified. ResultsThe lesion was present in the thorax of 24 patients and the abdomen in 28. Obvious features of hyaline-vascular CD (especially feeding vessels and draining veins) and its adjacent structures were demonstrated on 52 patients. ConclusionOn MDCT imaging, original MDCT transverse and reconstructed images provide an excellent tool for diagnosis of hyaline-vascular CD and have high value in the determination of a treatment plan.

B-Cell Lymphoma With Hyaline Vascular Castleman Disease–Like Features

Hyaline vascular Castleman disease (HV-CD) is a localized benign mass characterized by follicular hyperplasia with atrophic germinal centers, mantle zone hyperplasia, hyaline deposits, and vascular proliferation. Before establishing a diagnosis of CD, several B-cell lymphomas (BCLs) must be considered, including follicular lymphoma (FL), mantle cell lymphoma (MCL), and nodal marginal zone lymphoma (NMZL). Conversely, BCLs with prominent atrophic germinal centers and hyaline vascular penetration may closely resemble HV-CD, leading to misdiagnosis. We report 6 cases of BCL with prominent HV-CD-like features, including FL (2 cases), MCL, NMZL (2 cases), and interfollicular large B-cell lymphoma. Histologically, all were initially considered to be HV-CD before additional tests revealed a neoplastic B-cell proliferation. We highlight the clinicopathologic features of these cases in comparison with cases diagnostic of HV-CD. In contrast with HV-CD, BCLs with HV-CD-like features are more likely to manifest clinically with systemic symptoms or generalized lymphadenopathy. Careful histopathologic examination, supported with immunohistochemical studies, flow cytometric immunophenotyping, and judicious use of cytogenetic and molecular analyses, allows identification of the masked neoplastic process. A multifaceted approach, integrating clinical, histologic, and ancillary tests, can help avoid this diagnostic pitfall.

Mediastinal Castleman's disease mimicking thoracic paravertebral schwannoma

A 51-year-old female underwent resection of a solid lesion in the posterior mediastinum, preoperatively interpreted at imaging as thoracic schwannoma, requiring double sequential surgical procedure to be resected. The histologic examination of the resected mass diagnosed a hyaline-vascular Castleman's disease.

HGAL Protein Expression Persists in Disorders of Germinal Center Dissolution

Human Germinal Center-associated Lymphoma (HGAL) is a germinal center (GC) B-cell marker associated with a favorable outcome in diffuse large B-cell and classic Hodgkin lymphomas (CHL). To test its potential role in GC function, 75 cases involving GC disruption including 23 progressive transformation of germinal centers (PTGC), 25 follicle lysis and 27 Castleman disease (CD) were studied. HGAL protein expression uniformly correlated with GC B-cells in all except a subset of hyaline-vascular CD that showed severe regression of GCs. HGAL staining highlighted dismantled GCs in PTGC, in contrast to weak or absent CD10 and BCL6 staining. In follicle lysis, HGAL staining was comparable to that of CD10, BCL6, and CD21 in highlighting lysed follicles. Our findings show that HGAL protein expression effectively discriminates clusters of GC B-cells in disrupted follicles. Its persistence in disrupted GCs, suggests that it may be necessary for GC maintenance and supports its proposed role of confining B-cells to the GC microenvironment.