Non-HIV Associated Kaposi’s Sarcoma: A case report / Sarcoma de Kaposi no asociado a VIH: un caso clínico

Kaposi sarcoma (KS), first described in 1872, is an angioproliferative neoplasm that often presents with red-purple macules in the skin. This report is of a case ofclassic/iatrogenic form of KS in a 79-year-old male, that had a prolonged hospitalization due to surgical complications. After discharge, he presented a red-purple macule. A biopsy was made and KS was confirmed. He was HIV negative. The patient did not require any other treatment asides from the total removal of thelesion. Kaposi’s sarcoma is an uncommon disease, still very associated with HIV. This case demonstrates the importance of recognition of KS in non-HIV patients.There are four types of Kaposi’s sarcoma and the importance of its recognition in non-HIV patients. (AU)

An unusual case of pyogenic granuloma-like Kaposi sarcoma.

Kaposi sarcoma (KS) is not typically included in the differential diagnosis of lesions with clinical characteristics of pyogenic granuloma. However, cases of pyogenic granuloma-like Kaposi sarcoma have been reported in the literature. This variant is extremely rare and possesses clinical and histological findings consistent with both conditions. We report an elderly, immunocompetent man with pyogenic granuloma-like Kaposi sarcoma, which was clinically consistent with a pyogenic granuloma and possessed histological findings consistent with Kaposi sarcoma and pyogenic granuloma.

Update on Kaposi sarcoma-associated herpesvirus (KSHV or HHV8) - review.

Human herpesvirus 8 (HHV8), also known as Kaposi sarcoma-associated herpesvirus (KSHV), is one of the few pathogens recognized as direct carcinogen, being involved in the pathogenesis of Kaposi sarcoma, primary effusion lymphoma and multicentric Castleman disease. KSHV is a relatively recently discovered virus, with still limited possibilities for diagnosis and treatment. Therefore, ongoing studies are trying to answer the main issues related to the management of KSHV infection and its associated diseases. This review updates the current knowledge of the KSHV infection, discussing aspects related to epidemiology, virological features, clinical manifestations, diagnosis and treatment.

Update on oncogenesis and therapy for Kaposi sarcoma.

PURPOSE OF REVIEW: This review is an update of the recent findings on pathophysiology of Kaposi sarcoma, the role of HHV-8 in Kaposi sarcoma pathogenesis and to summarize the recent advances in the treatment of Kaposi sarcoma and the role of immunity to control the disease. RECENT FINDINGS: The causal agent of Kaposi sarcoma is HHV-8 and the mechanism by which HHV-8 drives the tumor development is unique. HHV-8 is not a classic oncogenic virus and the disease is an opportunistic tumor responding to immune restoration when it is possible. SUMMARY: Five epidemiologic types of Kaposi are recognized and HHV-8 is associated to all epidemiologic forms of Kaposi. HHV-8 is a virus favoring both angiogenesis and cellular proliferation, which are the two main histological features of Kaposi sarcoma. Although in many cases, treatment of Kaposi sarcoma is not necessary, specific chemotherapy, immunomodulation and immune stimulation are the tools for treating Kaposi sarcoma. Monochemotherapy has been shown to be as efficient as polychemotherapy and less toxic. Immune checkpoint inhibitors gave some promising results, which should be confirmed by prospective studies.

Epidemiology of Kaposi sarcoma: review and description of the nonepidemic variant.

Kaposi sarcoma (KS) is a rare angioproliferative tumor whose etiology is associated with human herpesvirus 8 (HHV 8). KS lesions typically involve the skin or mucosal surfaces and are characterized by purplish, red-blue, or brown-black macules, papules, and nodules which are prone to bleeding and ulceration. Definitive diagnosis requires biopsy revealing characteristic angioproliferative features. There are four widely recognized types of KS, which are histologically indistinguishable but differ in epidemiology and prognosis. These include classic, endemic, iatrogenic, and epidemic. KS has been increasingly recognized in a new subgroup of patients: men who have sex with men (MSM) but who are HIV-seronegative human immuodeficiency virus-seronegative and have no identifiable immunodeficiency. This fifth variant of KS, termed nonepidemic KS, resembles classic KS in presentation and prognosis. In this literature review, we report the characteristics of nonepidemic KS based on all published cases and highlight the need for clinicians to recognize this new clinical variant.

Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment. / Sarcoma de Kaposi y angiosarcoma cutáneo: directrices para el diagnóstico y tratamiento.

Kaposi sarcoma is a vascular sarcoma with 4 clinical variants: classic Kaposi sarcoma, which mainly affect the extremities of elderly patients and follows a chronic, generally indolent course; African Kaposi sarcoma; immunosuppression-associated Kaposi sarcoma; and AIDS-associated Kaposi sarcoma. Type8 human herpesvirus is the etiologic agent in all 4variants. Cutaneous angiosarcoma is a cutaneous neoplasm with a very poor prognosis. It carries a high probability of local relapse and has a 10% to 15% survival rate at 5years. There are 3 main variants of cutaneous angiosarcoma: idiopathic angiosarcoma of the face and scalp; Stewart-Treves syndrome; and postradiation angiosarcoma. The only potentially curative treatment is surgery with or without radiotherapy. However, its indistinct borders and multicentric nature mean that treatment is often palliative with chemotherapy, radiotherapy, or both.

Kaposi's sarcoma: a population-based cancer registry descriptive study of 57 consecutive cases diagnosed between 1977 and 2009.

BACKGROUND: There are few epidemiological data available on rare skin cancer, including Kaposi's sarcoma (KS), which is a multifocal illness affecting the skin, mucosa, and viscera. Four different types of KS have been described: classic, AIDS-associated, iatrogenic, and African. The purpose of this study was to describe the epidemiology and evolution of the different types of KS in the Doubs region of France. PATIENTS AND METHODS: A retrospective population-based study was conducted, including 57 patients with KS from the Doubs region between 1977 and 2009. These patients were identified by the tumor registry of the Doubs region. RESULTS: A larger proportion of AIDS-associated KS (61%) was observed compared to classic KS (30%) and iatrogenic KS (9%). No cases of African KS were observed. Most new cases were observed between 1987 and 1996 due to the AIDS explosion. The patients were predominantly male, with a male/female ratio at 10 : 4 (34 for AIDS-associated KS). The mean age of patients was 50.2 (higher in classic and iatrogenic KS and lower in AIDS-associated KS). Mucosal and visceral forms were more common in AIDS-associated KS, explaining the higher rate of mortality due to KS (21%). CONCLUSION: KS, which used to be a rare illness, as it existed only in the classic form, has become more common since the appearance of HIV and, to a lesser extent, of immunosuppressive treatment.

Kaposi's sarcoma: clinical and pathological aspects in patients seen at the Hospital Universitário Cassiano Antônio Moraes - Vitória - Espírito Santo - Brazil.

BACKGROUND: Kaposi's sarcoma is a neoplasm of endothelial origin that is divided into four distinct types according to the clinical characteristics and the affected population: Classic (in elder men of Jewish or Mediterranean origin); Epidemic (in patients affected by AIDS); Endemic (in black African men) and Iatrogenic (in patients under immunosuppressive regimens). Human herpesvirus 8 infection is essential but not sufficient for the sarcoma development. OBJECTIVE: To describe the epidemiological, clinical and histopathological aspects of patients with KS seen at the Dermatology Clinic -Cassiano Antônio Moraes University Hospital - Federal University of Espirito Santo, Vitória - ES. METHODS: A descriptive and retrospective study based on clinical charts of patients with KS seen at the Dermatology Clinic from 1986 to 2009. RESULTS: The majority of the 15 cases were male patients (93,3%) and white (60%). Epidemic Kaposi's sarcoma occurred in 80%, and the Classic form in 20%, with no cases in the Endemic or Iatrogenic groups. All the histopatho logical exams of the cutaneous lesions were reviewed and a proliferation of fusiform cells, extravasated erythrocytes and vascular rifts among the largest vessels, assuming the "vessels in vessels" typical aspect, were seen. CONCLUSION: The number of cases of Kaposi's Sarcoma was linear throughout the years of the study, especially of the epidemic form, although the incidence and prevalence of AIDS increased in the state of Espírito Santo. Therefore, if we consider the relation between KS and AIDS, a decreasing line of Kaposi's sarcoma could be seen, especially after the introduction of HAART.

Kaposi's Sarcoma: clinical and pathological aspects in patients seen at the Hospital Universitário Cassiano Antônio Moraes - Vitória - Espírito Santo - Brazil / Sarcoma de Kaposi: achados clínico-patológicos nos pacientes atendidos no Hospital Universitário Cassiano Antônio Moraes - Vitória - Espírito Santo - Brazil

BACKGROUND: Kaposi's sarcoma is a neoplasm of endothelial origin that is divided into four distinct types according to the clinical characteristics and the affected population: Classic (in elder men of Jewish or Mediterranean origin); Epidemic (in patients affected by AIDS); Endemic (in black African men) and Iatrogenic (in patients under immunosuppressive regimens). Human herpesvirus 8 infection is essential but not sufficient for the sarcoma development. OBJECTIVE: To describe the epidemiological, clinical and histopathological aspects of patients with KS seen at the Dermatology Clinic -Cassiano Antônio Moraes University Hospital - Federal University of Espirito Santo, Vitória - ES. METHODS: A descriptive and retrospective study based on clinical charts of patients with KS seen at the Dermatology Clinic from 1986 to 2009. RESULTS: The majority of the 15 cases were male patients (93,3%) and white (60%). Epidemic Kaposi's sarcoma occurred in 80%, and the Classic form in 20%, with no cases in the Endemic or Iatrogenic groups. All the histopatho logical exams of the cutaneous lesions were reviewed and a proliferation of fusiform cells, extravasated erythrocytes and vascular rifts among the largest vessels, assuming the "vessels in vessels" typical aspect, were seen. CONCLUSION: The number of cases of Kaposi's Sarcoma was linear throughout the years of the study, especially of the epidemic form, although the incidence and prevalence of AIDS increased in the state of Espírito Santo. Therefore, if we consider the relation between KS and AIDS, a decreasing line of Kaposi's sarcoma could be seen, especially after the introduction of HAART.

FUNDAMENTOS: O Sarcoma de Kaposi é neoplasia de origem endotelial, dividida em quatro formas clínicas: clássica (homens idosos de origem judaica e mediterrânea), epidêmica (associada ao HIV), endêmica (negros africanos) e iatrogênica (relacionada à imunossupressão). A infecção pelo herpes vírus humano tipo 8 (HHV-8) é necessária, mas insuficiente para que todas as formas possam ocorrer. OBJETIVOS: Avaliar os aspectos epidemiológicos, clínicos e características histopatológicas das lesões dos pacientes com Sarcoma de Kaposi consultados no Serviço de Dermatologia do Hospital Universitário Cassiano Antônio Moraes - Universidade Federal do Espírito Santo, Vitória - ES. MÉTODOS: Estudo retrospectivo, descritivo, realizado pela análise dos prontuários dos pacientes diagnosticados com Sarcoma de Kaposi, durante janeiro de 1986 a dezembro de 2009, no Serviço de Dermatologia. RESULTADOS: Dos 15 pacientes estudados, houve maioria do sexo masculino (93,3%) e predomínio da raça branca (60%). A forma epidêmica foi a mais freqüente (80%), seguida pela clássica (20%). Não foram observadas as formas: endêmica e iatrogênica. A revisão das lâminas das biópsias cutâneas foi feita nos 15 casos, e demonstrou derme com proliferação de células fusiformes, extravasamento de hemácias e fendas vasculares em torno de vasos maiores, com aspecto clássico de "vasos em torno de vasos". CONCLUSÕES: O número de casos de Sarcoma de Kaposi foi linear ao longo do estudo, especialmente da forma epidêmica. Por outro lado, a incidência e a prevalência da AIDS no Espírito Santo foram crescentes. Portanto, considerando-se a relação entre o sarcoma de Kaposi e a AIDS houve decréscimo do primeiro, mais acentuado após a era HAART.

Marathon of eponyms: 11 Kaposi sarcoma.

The use of eponyms has long been contentious, but many remain in common use, as discussed elsewhere (Editorial: Oral Diseases. 2009: 15; 185). The use of eponyms in diseases of the head and neck is found mainly in specialties dealing with medically compromised individuals (paediatric dentistry, special care dentistry, oral and maxillofacial medicine, oral and maxillofacial pathology, oral and maxillofacial radiology and oral and maxillofacial surgery) and particularly by hospital-centred practitioners. This series has selected some of the more recognized relevant eponymous conditions and presents them alphabetically. The information is based largely on data available from MEDLINE and a number of internet websites as noted below: the authors would welcome any corrections. This document summarizes data about Kaposi sarcoma.

Kaposi's sarcoma and mTOR: a crossroad between viral infection neoangiogenesis and immunosuppression.

The incidence of Kaposi's sarcoma (KS) among the recipients of solid organ transplants is about 500 times the rate in the general population, suggesting a role for immunosuppression in the development of the disease. The drugs used for the induction and maintenance of immunosuppression and the length of treatment with these agents influence both the incidence and the type of cancer development. The clinical presentation of KS in transplant recipients is often limited to the skin. The risk of death from KS is related to the form and extent of the lesions. The main approach to managing transplant-associated KS is to reduce or even discontinue immunosuppressive therapy; this strategy carries a risk of acute rejection of the graft. KS is a multicentric tumor composed of endothelium-lined vascular spaces and spindle-shaped cells. Its pathogenesis is unclear. Recent evidence suggests that vascular endothelial growth factor (VEGF) is likely to be a growth factor for KS cells: blocking the interaction between VEGF and Flk-1/KDR can abolish VEGF-induced growth of the tumor. Recently, Sirolimus, a drug used in kidney-transplant recipients, has been suggested to reduce KS progression in transplant recipients. This unexpected effect of the drug confirms previous experimental information on KS pathogenesis and may shed light on an array of molecular mechanisms, modulated by Sirolimus, of potential clinical interest in the transplantation scenario.

Endothelial cell- and lymphocyte-based in vitro systems for understanding KSHV biology.

Kaposi sarcoma (KS), the most common AIDS-associated malignancy, is a multifocal tumor characterized by deregulated angiogenesis, proliferation of spindle cells, and extravasation of inflammatory cells and erythrocytes. Kaposi sarcoma-associated herpesvirus (KSHV; also human herpesvirus-8) is implicated in all clinical forms of KS. Endothelial cells (EC) harbor the KSHV genome in vivo, are permissive for virus infection in vitro, and are thought to be the precursors of KS spindle cells. Spindle cells are rare in early patch-stage KS lesions but become the predominant cell type in later plaque- and nodular-stage lesions. Alterations in endothelial/spindle cell physiology that promote proliferation and survival are thus thought to be important in disease progression and may represent potential therapeutic targets. KSHV encodes genes that stimulate cellular proliferation and migration, prevent apoptosis, and counter the host immune response. The combined effect of these genes is thought to drive the proliferation and survival of infected spindle cells and influence the lesional microenvironment. Large-scale gene expression analyses have revealed that KSHV infection also induces dramatic reprogramming of the EC transcriptome. These changes in cellular gene expression likely contribute to the development of the KS lesion. In addition to KS, KSHV is also present in B cell neoplasias including primary effusion lymphoma and multicentric Castleman disease. A combination of virus and virus-induced host factors are similarly thought to contribute to establishment and progression of these malignancies. A number of lymphocyte- and EC-based systems have been developed that afford some insight into the means by which KSHV contributes to malignant transformation of host cells. Whereas KSHV is well maintained in PEL cells cultured in vitro, explanted spindle cells rapidly lose the viral episome. Thus, endothelial cell-based systems for studying KSHV gene expression and function, as well as the effect of infection on host cell physiology, have required in vitro infection of primary or life-extended EC. This chapter includes a review of these in vitro cell culture systems, acknowledging their strengths and weaknesses and putting into perspective how each has contributed to our understanding of the complex KS lesional environment. In addition, we present a model of KS lesion progression based on findings culled from these models as well as recent clinical advances in KS chemotherapy. Thus this unifying model describes our current understanding of KS pathogenesis by drawing together multiple theories of KS progression that by themselves cannot account for the complexities of tumor development.

Case report: classic Kaposi's sarcoma.

Classic KS usually appears between ages 50 and 70 years and has a relatively benign, indolent course for 15 years or longer. Treatment usually controls the disease quite well.