1/26. Paraneoplastic pemphigus with fatal pulmonary involvement in a woman with a mesenteric Castleman tumour.A 42-year-old woman presented with oral and labial erosions, conjunctivitis, facial rash and lichenoid erythematous papules on the trunk. Paraneoplastic pemphigus (PNP) was suspected, and a search for a neoplasm revealed an intra-abdominal Castleman tumour sized 7 x 5 x 6 cm. After removal of the Castleman tumour, the skin and mucosal inflammation gradually subsided over the next 12 months. However, due to irreversible pulmonary involvement the patient died of intractable respiratory distress 2 years after the onset of the disease. Systemic corticosteroids, azathioprine, cyclophosphamide, high-dose intravenous immunoglobulins and thalidomide were ineffective. The diagnosis of PNP was confirmed by keratinocyte antigen immunoprecipitation with the patient's serum.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/26. Systemic lupus erythematosus and Castleman's disease.Lymphadenopathy is a common finding in systemic lupus erythematosus (SLE), yet lymphoid malignancy is rare. Typically, adenopathy associated with SLE responds to glucocorticoid therapy. We evaluated a patient with a diagnosis of SLE who had progressive lymphadenopathy despite receiving aggressive corticosteroid therapy for SLE associated thrombocytopenia. Histopathology initially revealed an aggressive plasmacytosis characteristic of Castleman's disease (CD). CD, or angiofollicular hyperplasia, is a rare lymphoproliferative neoplasm that has features overlapping many autoimmune diseases. This disorder should be considered in autoimmune diseases with unremitting or progressive adenopathy.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/26. Human herpesvirus 8-associated solid lymphomas that occur in AIDS patients take anaplastic large cell morphology.Human herpesvirus type 8 (HHV-8; Kaposi's sarcoma-associated herpesvirus) is a recently isolated human herpesvirus frequently identified in Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman's disease. Here we report three cases of HHV-8-bearing solid lymphomas that occurred in AIDS patients (Cases 1-3). All three patients were homosexual men presenting extranodal masses in the lungs (Case 1) or skin (Cases 2 and 3), together with the presence of Kaposi's sarcoma (Case 1), primary effusion lymphoma (Case 2), or multicentric Castleman's disease (Case 3). These solid lymphomas exhibited anaplastic large cell morphology and expressed CD30, corresponding to the recent diagnostic criteria of anaplastic large cell lymphoma (ALCL). The chromosomal translocation t(2;5)-associated chimeric protein p80NPM/ALK was not observed in any of these cases. HHV-8 was detected in all of these cases by polymerase chain reaction, immunohistochemistry of HHV-8-encoded ORF73 protein, and in situ hybridization of T1.1. Epstein-Barr virus was detected only in Cases 2 and 3 by in situ hybridization. It is interesting that inoculation of a cell line obtained from a primary effusion lymphoma cell in Case 2 to severe combined immunodeficiency mice produced HHV-8-positive and Epstein-Barr virus-negative tumors in inoculated sites. These tumor cells exhibited phenotypes of ALCL that were identical to the subcutaneous tumor cells of this particular patient. These findings clearly show that HHV-8 can associate with solid lymphomas and that it can take anaplastic large cell morphology. Those lymphomas should be distinguished from the classical ALCL as were defined by the revised European-American classification of lymphoid neoplasms even though morphology and a part of immunophenotype mimic that of classical ALCL.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/26. Hyaline vascular-type Castleman disease: a rare cause of a hypervascular retroperitoneal mass.We present the cross-sectional imaging and angiographic findings of hyaline vascular-type Castleman disease located in the retroperitoneum. The diagnosis was made postoperatively. This entity can simulate a malignant neoplasm. The histologic subtypes and presentations of Castleman disease and the differential diagnosis of retroperitoneal masses are discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/26. Follicular dendritic cell sarcoma of the neck: report of two cases complicated by pulmonary metastases.BACKGROUND: Follicular dendritic cell (FDC) sarcoma is an uncommon neoplasm occurring primarily in lymph nodes but also in extranodal sites. A correct diagnosis can be difficult to make, especially in the latter sites. methods: Two patients with FDC sarcoma of the cervical soft tissues that metastasized to the lungs are reported. Both were initially misdiagnosed as having CASTLE (carcinoma showing a thymus-like element). Additional immunohistochemical stains were performed. RESULTS: The primary tumors showed jigsaw puzzle-like lobulation resembling thymic epithelial tumor and consisted of spindly cells arranged in fascicles, whorls, and a storiform pattern. The spindly cells had indistinct cell borders, vesicular nuclei, and distinct nucleoli. Perivascular spaces were present. lymphocytes were sprinkled throughout the tumor in one case but were sparse in the other. The metastatic deposits in the lungs appeared 27 and 2 years, respectively, after the initial presentation and were histologically similar to the original tumors. The FDC nature of the primary and metastatic tumors was confirmed by positive staining with CD21/CD35 cocktail and CD23 and by negative staining for cytokeratin. In one case, in direct continuity with the main tumor, there was a lobulated lesion composed of small lymphocytes punctuated by large cells with vesicular nuclei, histologically reminiscent of thymoma. The large cells were shown by immunohistochemistry to represent FDCs forming complex interconnecting meshworks. It is unclear whether this contiguous mass represents a precursor lesion or an unusual-looking component of the neoplasm. CONCLUSIONS: FDC sarcoma can look deceptively like a thymic epithelial tumor histologically. A correct diagnosis requires a high index of suspicion and immunohistochemical evaluation. The tumor shows a propensity to metastasize to the lungs, which can be delayed until more than 20 years after initial presentation.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
6/26. Castleman's disease masquerading as sigmoid colon tumor and Hodgkin lymphoma.Castleman's disease is a benign lymphoid neoplasm first reported as hyperplasia of mediastinal lymph nodes. Some authors referred to the lesions as isolated tumors, described as a variant of Hodgkin's disease with a possibility of a malignant potential and others proposed that the lymphoid masses were of a hamartomatous nature. Three histologic variants and two clinical types of the disease have been described. The disease may occur in almost any area in which lymph nodes are normally found. The most common locations are thorax (63%), abdomen (11%) and axilla (4%). We report two separate histologic types of Castleman's disease which were rare in the literature, mimicking sigmoid colon tumor and Hodgkin lymphoma. The diagnostic and therapeutic aspects of this rare entity is discussed.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/26. Hyaline vascular Castleman's disease with HMGIC rearrangement in follicular dendritic cells: molecular evidence of mesenchymal tumorigenesis.Chromosomal aberrations involving chromosome segment 12q13-15 are a common finding in a variety of benign mesenchymal tumors. The target gene encodes for HMGIC, a member of the high mobility group protein family. These proteins act as architectural transcription factors. HMGIC plays a role as a common genetic denominator in benign mesenchymal tumorigenesis. We report a case of hyaline vascular Castleman's disease with intragenic HMGIC rearrangement, due to a clonal cytogenetic aberration involving the long arm of chromosome 12 [46,XX, add(1)(q21),der(6)t(6;12) (q23;q15),add(7)(p22), -9,inv(9)(p11q13),del(12)(q15), mar] obtained after short-term primary cultures. A combined immunocytologic-cytogenetic approach enabled us to demonstrate the exclusive presence of HMGIC rearrangement in anti-CD21 reactive follicular dendric cells. This finding confirms that a clonal proliferation of follicular dendritic cells occurs in the hyaline vascular variant of Castleman's disease. It also provides a possible molecular pathway explaining stromal overgrowths and stromal neoplasms developing from this disorder.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/26. Mediastinal Castleman's disease complicated by follicular dendritic cell tumour.We describe a rare case of follicular dendritic cell tumour which had arisen over the background of hyaline vascular type of Castleman's disease at the mediastinal location. Constellation of histology and immunohistochemistry using CD21 antibody and non-reactivity to CD15, CD30, cytokeratin and epithelial membrane antigen helped us diagnose this case. The literature is reviewed, specially with reference to the genesis of follicular dendritic cell neoplasm at the backdrop of Castleman's disease and its clinical relevance.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/26. Paraneoplastic pemphigus in children and adolescents.BACKGROUND: Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with specific B-cell lymphoproliferative neoplasms. There has been an increasing number of individual reports in the childhood and adolescent population. OBJECTIVES: To examine the clinical and immunopathological features of PNP occurring in children and adolescents. patients AND methods: We analysed the clinical and immunopathological findings of 14 patients under the age of 18 years with a confirmed diagnosis of PNP. Sera from all patients were analysed by indirect immunofluorescence (IF) and immunoprecipitation for plakin autoantibodies, immunoblotting for detection of plectin autoantibodies, and enzyme-linked immunosorbent assay (ELISA) for the detection of desmoglein (Dsg) 1 and Dsg3 autoantibodies. RESULTS: Severe oral mucositis was observed in all patients, and lichenoid cutaneous lesions in eight of 14 patients. The average age at presentation was 13 years. Striking findings included: pulmonary destruction leading to bronchiolitis obliterans in 10 patients, association with Castleman's disease in 12 patients, and a fatal outcome in 10 patients. The underlying neoplasm was occult in 10 patients. Histological findings include lichenoid and interface dermatitis with variable intraepithelial acantholysis. Deposition of IgG and C3 in the mouth and skin by direct IF was not found in some cases, but indirect IF detected IgG autoantibodies in all cases. immunoprecipitation revealed IgG autoantibodies against desmoplakin I, envoplakin and periplakin in all cases, and against desmoplakin II and the 170-kDa antigen in 13 and 10 patients, respectively. Dsg3 and Dsg1 autoantibodies were present in 10 and three patients, respectively, and plectin autoantibodies in 13 patients. CONCLUSIONS: PNP in children and adolescents is most often a presenting sign of occult Castleman's disease. It presents with severe oral mucositis and cutaneous lichenoid lesions. serum autoantibodies against plakin proteins were the most constant diagnostic markers. Pulmonary injury appears to account for the very high mortality rates observed.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
10/26. 2-Chloro-deoxyadenosine induces durable complete remission in Castleman's disease but may accelerate its transformation to non-Hodgkin's lymphoma.There is currently no consensus on the best treatment for unresectable hyaline-vascular variant or for multicentric Castleman's disease (MCD), because none of the reported regimens have consistently produced complete response or durable remission in the majority of patients In the present study, we report on the use of 2-CdA (2-chloro-deoxyadenosine) in three patients, two of them with MCD and one with unresectable hyaline-vascular type disease. Relapse-free survival of the responding patients was 24 and 20 months. Later, both patients evolved to non-Hodgkin's lymphoma (NHL) (diffuse large B-cell lymphoma and peripheral T-cell NHL, respectively). 2-CdA typically causes a long-lasting state of immunodeficiency and the profound influence of this drug on the immune system has raised questions concerning the emergence of secondary neoplasms after its use. Therefore, it is reasonable to conclude that: 1) 2-CdA can induce durable complete remission in MCD patients but unfortunately it cannot cure the disease; 2) the possibility that 2-CdA may accelerate the transformation of MCD to NHL cannot be ruled out.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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