Cases reported "Sarcoma, Kaposi"

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1/24. 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).
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ranking = 1
keywords = angiomatosis
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2/24. Bacillary angiomatosis affecting the oral cavity. Report of two cases and review.

    Bacillary angiomatosis (BA) is an infectious disease characterized by proliferative vascular lesions; it mainly affects HIV-positive patients. Multiple cutaneous nodular lesions together with fever, chills, malaise, anorexia, vomiting and headache are the most important clinical manifestations. It may also involve the heart, liver, spleen, bones, lung, muscles, lymph nodes, central nervous system and other organs. erythromycin, 500 mg four times a day, is the drug of choice. The importance of this lesion lies in its clinical and histological similarity with other diseases. Cutaneous and oral lesions of BA clinically resemble Kaposi's sarcoma (KS). Histopathologically, BA may be confused with angiosarcoma, pyogenic granuloma and epithelioid hemangioma. We report two HIV-positive men with BA lesions in the oral mucosa. diagnosis was confirmed by biopsy and Warthin-Starry silver staining.
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ranking = 5
keywords = angiomatosis
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3/24. Spontaneously regressed Kaposi's sarcoma and human herpesvirus 8 infection in a human immunodeficiency virus-negative patient.

    Kaposi's sarcoma occurring in a 78-year-old woman, with the absence of the human immunodeficiency virus infection, was correctly diagnosed by immunohistochemistry using anti-human herpesvirus 8 (HHV8) antibody (PA1-73N) for the first time. The patient suffered from chronic respiratory failure and was treated with a low dose of steroids for 2.5 years. After her medication dosage was increased for the exacerbation of the respiratory failure, multiple skin tumors in her feet and legs suddenly developed. Histopathologically, skin tumors were suspected as Kaposi's sarcoma at the first biopsy and reactive angiomatosis at the second biopsy. polymerase chain reaction and immunohistochemistry, however, revealed the presence of HHV8 dna fragment and positive staining in the majority of spindle cells in the skin tumors. Serological examination confirmed the positivity of anti-HHV8 antibodies. HHV8 infection and steroid-induced immunosuppression, as well as environmental factors played a role in the development of Kaposi's sarcoma in this patient, because she was born in Okinawa, which is a well-known endemic area of Kaposi's sarcoma in japan. As her general condition improved, the skin lesions regressed without any specific treatment, and disappeared completely 8 months later, in which regression may be associated with evidence of numerous CD8 cell infiltration in the second biopsy tissues. No recurrence was observed during the following 6 month follow up.
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ranking = 1
keywords = angiomatosis
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4/24. Kaposiform hemangioendothelioma in adult. Report of a case with amianthoid-like fibrosis and angiectases.

    A rare case of kaposiform hemangioendothelioma in adult is reported. The 11 x 7 x 5 cm tumor was excised from deep subcutis of the abdominal region in 37-year-old man. No signs of kasabach-merritt syndrome or lymphangiomatosis were present. Besides typical pattern of kaposiform hemangioendothelioma, following unusual features were found: dilated vessels producing a gross impression of spindle cell hemangioma, areas of amianthoid-like fibrosis, and diffuse immunoreactivity for CD99. The differential diagnosis included mainly spindle cell hemangioma (hemangioendothelioma), hemangiopericytoma-like solitary fibrous tumor, and Kaposi's sarcoma.
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ranking = 1
keywords = angiomatosis
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5/24. AIDS presenting with cutaneous Kaposi's sarcoma and bacillary angiomatosis in the bone marrow mimicking Kaposi's sarcoma.

    Kaposi's sarcoma (KS) and bacillary angiomatosis (BA) may be histologically similar. A precise diagnosis is required because of the different management of these diseases. KS or BA involving bone marrow is rare in patients with and without acquired immune deficiency syndrome (AIDS). We report the case of a 40-year-old human immunodeficiency virus (HIV)-positive homosexual male who presented with small KS lesions in the skin and BA in the bone marrow that histologically were similar. Laboratory evaluation revealed anemia and thrombocytopenia; CD4 count was 103/mm3, and the viral load was 750,000 hiv-1 mRNA copies per milliliter in plasma. bartonella henselae, the etiologic agent of BA, was isolated from a blood culture. dna sequences of human herpesvirus-8 (HHV-8), the putative etiologic agent of KS, were identified by polymerase chain reaction (PCR) in skin and bone marrow specimens, but antibody anti-HHV-8-encoded protein ORF73, localized signals only in the skin-KS lesion. The patient received clarithromycin and cefotetan for the BA, and antiretroviral therapy for the HIV infection. The skin lesions gradually regressed, the hiv-1 mRNA copy number decreased to less than 400 per milliliter and the cd4 lymphocyte count increased to 665/mm3. In conclusion, vascular lesions of BA and KS may be clinically and histologically similar, both may be associated with advanced AIDS, and an accurate diagnosis is required because of their different management.
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ranking = 5
keywords = angiomatosis
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6/24. Upper gastrointestinal bacillary angiomatosis causing hematemesis: a case report.

    A 38-year-old HIV-positive woman presented with massive hematemesis on initial admission to hospital. endoscopy revealed ulcerated nodular lesions in the esophagus, stomach, and duodenum. The clinical impression was of Kaposi's sarcoma. The stomach was biopsied when the patient re-presented, and another endoscopy was performed. The biopsy showed mucosal ulceration with a proliferation of vascular channels associated with neutrophils and clumps of purplish, granular bacterial colonies, which were highlighted by a Warthin-Starry stain. The histopathological features were typical of bacillary angiomatosis. This case highlights bacillary angiomatosis involving the gastrointestinal tract at multiple sites, the cause of massive upper gastrointestinal hemorrhage that was the initial presentation of an HIV-positive patient, and the occurrence of visceral bacillary angiomatosis in the absence of cutaneous lesions.
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ranking = 7
keywords = angiomatosis
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7/24. Polypoid endobronchial lesions. A manifestation of bacillary angiomatosis.

    Polypoid endobronchial lesions occurred in a patient with acquired immunodeficiency syndrome (AIDS) with recent fever, skin lesions, lymphadenopathy, lung infiltrates, and pleural effusions. His condition improved with antimicrobials and vincristine. After therapy ceased, skin lesions recurred and gastroesophageal mucosal lesions developed. Bacillary angiomatosis was identified during retrospective analysis of skin and endobronchial biopsy specimens.
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ranking = 5
keywords = angiomatosis
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8/24. Bacillary epithelioid angiomatosis involving the liver, spleen, and skin in an AIDS patient with concurrent Kaposi's sarcoma.

    The simultaneous findings of bacillary epithelioid angiomatosis and Kaposi's sarcoma of the skin with visceral hepatosplenic bacillary epithelioid angiomatosis is reported in a patient with acquired immune deficiency syndrome. liver biopsy showed periportal peliotic spindle cell foci that initially were misinterpreted as Kaposi's sarcoma. After antibiotic therapy induced rapid clinical improvement, repeated liver biopsy showed resolution of the previously noted lesions. Although the violaceous skin lesions all appeared similar clinically, some resolved completely and some progressed. One of the latter was biopsied and had the histologic features of Kaposi's sarcoma. The differential diagnosis is discussed.
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ranking = 6
keywords = angiomatosis
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9/24. Localized bacillary angiomatosis in the oral cavity: observations about a neoplasm with atypical behavior. Description of a case and review of the literature.

    Bacillary angiomatosis is a rather frequent infectious pathology appearing mainly in the skin but can also affect the liver, spleen, heart, bones, lungs, muscles, central nervous system and other organs. The localization of the lesion in the oral cavity is rather rare, as it is evident in the literature. Bacillary angiomatosis can be clinically similar to the Kaposi's sarcoma and histologically confused with angiosarcoma, epitheloid hemangioma and pyogenic granuloma. A case of bacillary angiomatosis of the oral cavity in an immuno-competent patient is described. The high tendency to relapse, the capability in migration and to involve several localizations at the same time have induced the authors to deepen the research to exclude the possibility that it could be a Kaposi's sarcoma or a pyogenic granuloma and to get to an accurate diagnosis in order to resolve the disease.
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ranking = 7
keywords = angiomatosis
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10/24. Epithelioid angiomatosis or cat scratch disease with splenic and hepatic abnormalities in AIDS: case report and review of the literature.

    Cat scratch disease (CSD) in the setting of HIV infection is associated with lesions of epithelioid angiomatosis but not with granulomatous lesions seen in the normal host. We report a case of CSD in a patient with AIDS and Kaposi's sarcoma with epithelioid angioma of skin, thrombocytopenia, and abnormalities of liver, spleen, lymph node, and pleura that responded to antimicrobial therapy. We also review reported cases of epithelioid angiomatosis in hiv infections. 12 of these resolved, including 3 without antimicrobial therapy; 18 demonstrated pleomorphic organisms with Warthin-Starry silver stain. Six involved visceral or bony as well as skin lesions. CSD should be considered in the setting of HIV infection with skin nodules even in the presence of biopsy-proven Kaposi's sarcoma. CSD may in these patients be responsible for a variety of disseminated lesions which respond to antimicrobial therapy.
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ranking = 6
keywords = angiomatosis
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