1/78. Epstein-Barr virus-associated intravascular lymphomatosis within Kaposi's sarcoma in an AIDS patient.Intravascular lymphomatosis (IL) is an unusual neoplasm characterized by multifocal proliferation of lymphoma cells exclusively within the blood vessels. We report here a patient with acquired immunodeficiency syndrome (AIDS) and disseminated Kaposi's sarcoma. A 233-bp amplification product of HHV-8 was detected in the dna extracted from specimens of Kaposi's sarcoma at different sites by polymerase chain reaction (PCR). At autopsy, the vessels within the Kaposi's sarcoma were dilated and filled with atypical large mononuclear cells. No such feature was seen in the vessels of non-Kaposi's sarcomatous regions. Immunohistochemically, the spindle cells of Kaposi's sarcoma were positive for CD31 (endothelial cell marker). The intravascular tumor cells were positive for CD45 (leukocyte common antigen) but negative for others, including chloroacetate esterase, CD45-RO (UCHL-1, Pan-T), CD3, CD43, CD20 (L26, Pan-B), CD30 (Ki-1), immunoglobulin heavy chains and light chains, CD56 (natural killer cell antigen), and CD31. Monoclonal rearrangement of immunoglobulin heavy chain gene was detected in the dna extracts from fresh tissue of Kaposi's sarcoma by PCR, which indicated that the lymphoma cells within the Kaposi's sarcoma were of monoclonal B cell origin. in situ hybridization revealed that EBER-1 transcripts were present in the lymphoma cells of IL but not in the spindle cells of Kaposi's sarcoma. To the authors' best knowledge, this is the first instance of IL in an AIDS patient with direct evidence of EBV association.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/78. Favourable effect of chemotherapy on clinical symptoms and human herpesvirus-8 dna load in a patient with Kaposi's sarcoma presenting with fever and anemia.The case of a patient infected with human immunodeficiency virus type 1 (hiv-1) with Kaposi's sarcoma who presented with fever of unknown origin, severe anemia, thrombocytopenia and hypoalbuminemia but only limited involvement of the skin is presented. Chemotherapy directed at Kaposi's sarcoma resulted in resolution of these clinical signs and symptoms and was associated with a significant reduction in human herpesvirus-8 dna load in serum, despite continued hiv-1 replication. Such a decreasing human herpesvirus-8 load following Kaposi's sarcoma-directed chemotherapy has not been reported previously. These findings suggest that Kaposi's sarcoma was indeed responsible for the clinical syndrome and that this neoplasm is a source of human herpesvirus-8 virus particle production, which can be inhibited by chemotherapy-induced reduction in tumor burden.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/78. Congenital cutaneous multifocal kaposiform hemangioendothelioma.Kaposiform hemangioendothelioma is a rare vascular neoplasm in children often associated with kasabach-merritt syndrome. The tumor usually presents in retroperitoneal location and is rarely present at birth. Cutaneous lesions manifest after birth and must be clinically differentiated from infantile hemangiomas and diffuse neonatal hemangiomatosis. Histologically, it bears similarities with Kaposi sarcoma. We present a case of congenital cutaneous multifocal kaposiform hemangioendothelioma (KHE).- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/78. 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) |
5/78. A rare case of cutaneous kaposiform haemangioendothelioma.Kaposiform haemangioendothelioma is a rare vascular neoplasm with a wide anatomical distribution. We describe an unusual case arising in the post-auricular skin of a male infant overlying a ventriculoperitoneal shunt.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
6/78. Acquired immune deficiency syndrome-related hyperkeratotic Kaposi's sarcoma with severe lymphoedema: report of five cases.Kaposi's sarcoma (KS) is the most frequent neoplasm in acquired immune deficiency syndrome (AIDS) patients. Whereas typical cases present as erythematous, plaque or nodular lesions, hyperkeratotic variants of AIDS-associated KS are rare. We describe five patients with AIDS-associated KS with hyperkeratosis and lymphoedema as prominent features. We also speculate on its pathogenesis.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/78. A report of two non-AIDS associated Kaposi's sarcoma in malaysia.Kaposi's sarcoma is an uncommon cutaneous neoplasm seen classically in elderly males of East European or Jewish extract. It has been known to be endemic in sub-Saharan africa for many years. Numerous cases had been described in patients on long-term immunosuppressive therapy and in patients living with acquired immunodeficiency syndrome (AIDS). In spite of the increasing number of organ transplant recipients and people living with AIDS. Kaposi's sarcoma remains rare in asia. We report two cases seen in Johor, malaysia.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
8/78. Kaposiform hemangioendothelioma of the thymus.Kaposiform hemangioendothelioma is a rare pediatric neoplasm that presents most commonly in the soft tissues. We report the case of a 1-month-old infant who presented with stridor and was found to have a diffusely infiltrating tumor in the thymus that extended into the pericardium and up the carotid sheaths. Histologic examination revealed a vascular tumor infiltrating among the lobules of the lymphocyte-depleted thymus. The lesion had features of both a capillary hemangioma and Kaposi sarcoma. Immunoperoxidase studies on formalin-fixed, paraffin-embedded tissue demonstrated the neoplastic endothelial cells to be positive for vascular markers CD31 and CD34. Antibody to factor viii-related antigen labeled feeding vessels, but failed to stain the lobules of tumor. Although these tumors have been treated in a fashion similar to capillary hemangiomas in the past, it may be important to differentiate Kaposiform hemangioendotheliomas because of their association with kasabach-merritt syndrome and recent success with more aggressive chemotherapy regimens.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/78. Novel association of haemophagocytic syndrome with Kaposi's sarcoma-associated herpesvirus-related primary effusion lymphoma.Haemophagocytic syndrome (HPS) is a fulminant, often fatal, systemic illness that occurs in association with infection and malignancy. We provide the first report of HPS that heralded a primary effusion lymphoma (PEL), a rare neoplasm linked to Kaposi's sarcoma-associated herpesvirus. The patient was a 38-year-old man with acquired immunodeficiency syndrome who presented with fever, sweats, lymphadenopathy, splenomegaly and refractory anaemia. Examination of the spleen demonstrated haemophagocytosis; analysis of ascites revealed PEL. Treatment with chemotherapy and ganciclovir resulted in complete remission of both conditions. This case illustrates the diagnostic challenges posed by HPS and supports the trial of antiviral agents in combination with chemotherapy in patients with PEL.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/78. Endemic (African) Kaposi's sarcoma presenting as a plantar tumour.We present a case of the aggressive variant of African endemic Kaposi's sarcoma (AKS) which presented as a large fungating and ulcerated plantar mass. Our patient responded extremely well to chemotherapy with no recurrence for 9 months after treatment completion. AKS is one of the most common cutaneous neoplasms in black Africans and although rare in europe, it may be seen more frequently in the future because of the ease of international travel. The existing classification of KS into five different types (classic, African-endemic, iatrogenically immunocompromised patients, epidemic HIV-related and Mediterranean-endemic) cannot address fully the many anomalies described in the disease. The detection of a new herpes simplex-like viral dna sequence (HHV-8) in different types of KS helps to explain some of the enigma described in this disease.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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