Cases reported "Sarcoma, Kaposi"

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1/8. 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.
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2/8. A unique case of a benign disseminated angioproliferation combining features of Kaposi's sarcoma and diffuse dermal angioendotheliomatosis.

    A female patient undergoing chronic hemodialysis had disseminated, violaceous, and partly ulcerated plaques develop on the trunk. Lesions had erupted simultaneously over a period of 4 weeks and resolved within 5 months after steroid treatment. By histopathology, the papillary dermis was densely filled with blood vessels lined by a single layer of differentiated endothelial cells, a growth pattern resembling diffuse dermal angioendotheliomatosis. In some areas, endothelial cells were spindle shaped and formed discontinuous lumina. Red blood cells were interspersed within these slits, giving the lesions a kaposiform appearance. By immunohistochemistry, endothelial cells reacted with the antibodies anti-von willebrand factor, anti-CD31, and anti-CD34 and with the lectin ulex europaeus-1. The course of the disease combined with the unusual histopathology makes this case a unique form of a benign disseminated kaposiform angioproliferation.
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3/8. cytomegalovirus vasculitis accompanied by an exuberant fibroblastic reaction in the intestine of an AIDS patient.

    A case of cytomegalovirus (CMV) vasculitis in the intestine of a patient with acquired immune deficiency syndrome (AIDS) is reported. The distal jejunum and ileum had multiple well-demarcated mucosal ulcers and microscopic examination revealed an unusual, exuberant fibroblastic proliferation in the ulcer base. Amidst these fibroblasts, there were several small blood vessels of which the endothelial cells contained cytomegalic inclusion bodies. The lesion showed a Kaposi's sarcoma-like appearance, but spindle cells were negative in immunostaining for factor viii-related antigen or ulex europaeus agglutinin-1. Although the CMV infection was observed in almost all organs, the exuberant fibroblastic proliferation seen in the intestine was not found in other organs. This lesion might represent a peculiar reaction of the immunologically compromised host to the CMV in the intestinal blood vessels.
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4/8. Solitary eyelid Kaposi sarcoma in an hiv-negative patient.

    PURPOSE: To describe a case of localized Kaposi sarcoma (KS) of the eyelid in an hiv-seronegative patient. methods: An 80-year-old man developed an ulcerated nodular tumor-like mass that grew rapidly on his left upper eyelid. There were no similar lesions elsewhere. The eyelid lesion was completely excised and histopathologically examined. Serological analyses and molecular biologic techniques, including polymerase chain reaction, were used. RESULTS: Laboratory examinations were within normal limits, and serology for hiv was negative. Histological sections revealed a vascular proliferation composed predominantly of small slit-like blood vessels and epithelioid spindle cells, supporting the diagnosis of KS. polymerase chain reaction was positive for human herpesvirus 8. During a 2-year follow-up, no recurrences, development of new lesions, or hiv seroconversions were observed. CONCLUSION: This is a classic KS involving only the eyelid in an hiv-negative patient. Location in the eyelid is a possible, albeit rare, initial solitary manifestation of KS in elderly hiv-negative patients. Surgery is both safe and effective.
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5/8. A bullous variant of Kaposi's sarcoma in an elderly female.

    We report the case of Kaposi's sarcoma (KS) in an elderly Italian female that has slowly progressed and evolved clinically with bullous formation. The skin biopsy was diagnostic of KS, showing the typical spindle cell formations, but at the same time showing a labyrinth-like collection of vessels that correlated with the bullous nature of the clinical lesions. Immunohistochemical staining for factor viii antigens demonstrated strong reactivity of non-neoplastic blood vessel endothelium but virtually no staining of tumor cells. This is similar to the reactivity of normal lymphatic endothelial cells and supports the notion that these labyrinth-like vessels are derived from lymphatic endothelium.
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6/8. Clinically uninvolved skin in AIDS: evidence of atypical dermal vessels similar to early lesions observed in Kaposi's sarcoma. Ultrastructural study in four patients.

    The clinically uninvolved skin of 4 patients with well-developed AIDS was investigated by electron microscopy. All biopsy specimens had vascular abnormalities: protruding endothelial cells, vascular channels reduced to slits, gaps within the vascular walls, and extravasated erythrocytes. These features are similar to those described in early lesions of Kaposi's sarcoma. These findings suggest that blood vessels of the clinically uninvolved skin of AIDS patients are potential sites of Kaposi's sarcoma lesions.
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7/8. Bacillary angiomatosis: presentation of six patients, some with unusual features.

    Bacillary angiomatosis (BA) is an unusual systemic vascular proliferation seen predominantly in patients with the acquired immunodeficiency syndrome. These vascular lesions are probably due to infection with a bartonella species, most often B. henselae and, in some patients, B. quintana. BA is treatable and often curable, but without therapy, may be life-threatening. Clinically, the lesions, when superficial, are said to often resemble pyogenic granulomas, appearing polypoid histologically with an epidermal collarette. We now report six patients, three of whom showed lesions of BA morphologically and histologically distinct from the other patients reported to date. Two patients lesions appeared clinically as violaceous plaques and tumours resembling Kaposi's sarcoma; one of them had lesions histologically reminiscent of a papular angiokeratoma; and the other had lesions histologically suggestive of a combination of Kaposi's sarcoma and BA. Another patient presented with soft subcutaneous nodules which histologically showed extensive acute inflammation characteristic of an acute abscess, but which also displayed proliferating dilated small blood vessels with bulbous endothelial cells adjacent to numerous bacteria and also containing them. The Grocott-methenamine silver stain and the Warthin-Starry stain showed the organisms to better advantage in lesions of all six patients, although bacteria were also evident with the haematoxylin and eosin, periodic acid-Schiff and alcian blue stains.
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8/8. Bullous lesions in Kaposi's sarcoma: case report.

    Bullous lesions have been only rarely described in Kaposi's sarcoma (KS), and their histopathologic features have never been described in detail. We report a case of bullous lesions of KS in an 82-year-old Italian woman. The patient had typical smooth pale reddish-grey slightly-raised KS plaques on the legs, present for at least 10 years. Several dull grayish-pink blisters (0.5 to 2 cm in diameter) affected both dorsa of her feet and ankles symmetrically. Two punch biopsies were taken, one from an infiltrated KS plaque on the right buttock and the other from a bullous lesion on the right foot. Histopathologically, the late KS plaque on the buttock showed typical features of KS, with an increased number of spindle cells arranged in short bundles and extravasation of erythrocytes. The bullous lesion on the foot showed a full-thickness vascular neoplasm involving the upper and lower dermis and the subcutaneous fat. The upper portion of the lesion contained many newly formed, highly-dilated blood vessels, touching the overlying epidermis and separated from it by a narrow band of collagen and endothelial cells; wide, empty spaces characterized the superficial dermis, in which preexisting venules and bands of collagen associated with non-atypical endothelial cells floated. All these findings would suggest a lymphangiomatous lesion, if the presence of specific diagnostic criteria of KS were not recognizable at a deeper level of the lesion. Various criteria actually suggest that the bullous lesion may be regarded as an epiphenomenon of a KS plaque lesion: (a) full-thickness involvement of the reticular dermis and, in this case, also of the subcutaneous fat; (b) dense and patchy lymphoplasmocytic infiltrate typical of plaque lesions and, much less frequently, of patch lesions; (c) presence of ectatic blood vessels, filled with plasma and erythrocytes (pseudoangiomatous findings), a nonpathognomonic but highly characteristic finding of the plaque lesion; and (d) as in the KS plaque lesions, in the bullous lesion as well the reticular dermis was characterized by an increased number of anastomosing bizarrely shaped vascular spaces lined by non-atypical endothelial cells. We hypothesize that the prevalence of lymphangiomatous differentiation in the upper dermis represents one of the many features of KS lesions. When present, it may correlate with the clinical feature of a blister.
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