Sarcoma de Kaposi anoperineal: uma rara apresentação / Perineal Kaposi's Sarcoma: a rare presentation

O sarcoma de Kaposi é uma neoplasia maligna associada à infecção pelo herpes vírus humano 8 em doentes imunossupressos. O sarcoma de Kaposi Epidêmico é o tipo epidemiológico mais frequente e afeta indivíduos VIH-positivos. A região anoperineal é raramente envolvida e as lesões suspeitas devem ser biopsiadas para confirmação histológica. A base do tratamento é a restauração imune do doente. Relatamos o caso de um jovem, com diagnóstico recente de infeção pelo VIH, sem tratamento, que foi admitido no serviço de infectologia apresentando sintomas constitucionais, adenomegalias inguinais e extensa lesão verrucosa e ulcerada na região anoperineal. As biópsias confirmaram o diagnóstico de sarcoma de Kaposi e o doente iniciou terapia antirretroviral e quimioterapia. Houve recuperação clínica, regressão das lesões e desaparecimento das adenomegalias. Este relato objetiva alertar as equipes médicas no sentido de se incluir o sarcoma de Kaposi no diagnóstico diferencial das lesões que afetam a região anoperineal.

Kaposi's sarcoma is a malignant neoplasm associated with human herpesvirus 8 infection in immunocompromised patients. Epidemic Kaposi's sarcoma is the most common epidemiological type and affects HIV-positive patients. Perineal involvement is rare, and suspicious lesions should be biopsied to confirm histological diagnosis. Treatment consists of restoring the patient's immune system. We report the case of a young patient recently diagnosed with HIV, without treatment, who was admitted to the Department of Infectious Diseases with nonspecific symptoms, inguinal lymphadenopathy, and an extensive verrucous ulcerated lesion in the perineal region. Biopsy confirmed the diagnosis of Kaposi's sarcoma, and the patient was started on antiretroviral therapy and chemotherapy. Clinical recovery was achieved, with lesion reduction and inguinal adenopathy resolution. This case report aims to encourage physicians to include Kaposi's sarcoma in the differential diagnosis of perineal lesions.

Sarcoma de Kaposi oral en paciente seropositivo, fase sintomática avanzada. Presentación de caso / Oral Kaposi Sarcoma, advanced symptomatic phase, in a seropositive patient. Case presentation

RESUMEN Se presentó el caso clínico de un paciente seropositivo, en fase sintomática avanzada. Fue atendido en el Servicio de Medicina Interna del Hospital Clínico Quirúrgico Docente "León Cuervo Rubio", de la ciudad de Pinar del Río, por presentar disnea, astenia, anorexia y pérdida de peso. Al examen oral se constató lesión tumoral de la lengua que dificultaba la masticación y deglución. La biopsia mostró sarcoma de Kaposi asociado al VIH/sida. La evolución tórpida y el estadio tan avanzado de la enfermedad, propiciaron el deceso del paciente (AU).

ABSTRACT The authors presented the clinical case of a seropositive patient, in advanced symptomatic phase. The patient attended the Internal Medicine Service of the Teaching Clinical Surgical Hospital Leon Cuervo Rubio of Pinar del Rio, presenting dyspnea, asthenia, anorexia and weight loss. On the oral examination, a tumor lesion was found making difficult to chew and swallow. A biopsy showed Kaposi sarcoma associated to HIV/AIDS. The torpid evolution and disease's advanced stage propitiated the patient's death (AU).

Diagnosis of elephantiasis nostras verrucosa as a clinical mani-festation of Kaposi's sarcoma / Diagnóstico de elefantiasis nostras verrucosa como mani-festación clínica del Sarcoma de Kaposi

Elephantiasis nostras verrucosa, a rare manifestation of Kaposi's sarcoma, is a progressive cutaneous hypertrophy caused by chronic non-filarial lymphedema secondary to obstruction of the lymphatic system that can lead to severe disfigurement of parts of the body that have gravity-dependent blood flow, due to edema, fibrosis, and hyperkeratosis, especially lower extremities. Among the various conditions that can induce chronic lymphedema are tumors, trauma, radiotherapy, obesity, hypothyroidism, chronic venous stasis, and AIDS-related Kaposi's sarcoma. Kaposi's sarcoma is a vascular tumor associated with the presence of human gammaherpesvirus 8 that is predominantly cutaneous, locally aggressive, with metastasis, and is associated with the production of factors that favor inflammation, lymphatic obstruction, and lymphedema.

Sarcoma de Kaposi epidémico en África Subsahariana / Epidemic Kaposi sarcoma in Sub-Saharan Africa

El sarcoma de Kaposi se ha convertido en uno de los tumores más prevalentes en África tras la epidemia de VIH, que afecta de una manera similar a hombres y mujeres. El retraso diagnóstico y el limitado acceso a tratamiento antirretroviral o quimioterapia condicionan el pronóstico de los pacientes que lo padecen. En este artículo se realiza una revisión sobre la referida enfermedad, con el objetivo de describir sus aspectos más relevantes en los últimos años en África, como son su epidemiología, caractéristicas clínicas y opciones terapéuticas existentes. Este tumor es provocado por la infección por virus herpes humano tipo 8, que resulta más prevalente en las zonas rurales del continente africano. Se postula la transmisión a través de la saliva como la vía más importante de contagio en África. La inmunodepresión que causa el VIH favorece el efecto oncogénico del virus. La forma epidémica de SK se manifiesta inicialmente como lesiones hiperpigmentadas o violáceas en la piel, que pueden extenderse a linfáticos o mucosas y a nivel sistémico, principalmente a pulmón o aparato digestivo. El síndrome de reconstitución inmune sistémica puede complicar la evolución del paciente. El inicio temprano de la terapia antirretroviral resulta imprescindible. Además, el pronóstico de los pacientes mejora con la suma de tratamiento quimioterápico con doxorrubicina, vincristina, etopóxido o bleomicina principalmente(AU)

Kaposi sarcoma (KS) has become one of the most prevalent tumors in Africa after the HIV epidemic. KS affects both men and women. Diagnostic delay and limited access to antiretroviral treatment or chemotherapy have an impact on the prognosis of KS patients. A review was conducted about KS with the purpose of describing its most outstanding characteristics in recent years in Africa, such as its epidemiology, clinical features, and existing therapeutic options. This tumor is caused by infection with human herpesvirus 8, which is more prevalent in rural areas of the African continent. Transmission via saliva was found to be the most important transmission route in Africa. HIV-related immunosuppression fosters the oncogenic effect of the virus. The epidemic form of KS initially presents as hyperpigmented or violet-colored skin lesions which may extend to lymph nodes or mucosae, or systemically, mainly to the lungs or the digestive tract. Systemic immune reconstitution syndrome may complicate the patient's evolution. Early start of antiretroviral therapy is indispensable. Additionally, prognosis improves with chemotherapy with doxorubicin, vincristine, etoposide or bleomycin, mainly(AU)

Kaposi's sarcoma in persons living with HIV/AIDS: a case series in a tertiary referral hospital

Abstract: Background: Kaposi's sarcoma (KS) is a rare neoplasm with indolent progression. Since 1981, the Kaposi's sarcoma epidemic has increased as co-infection with HIV. Objectives: The study aimed to identify the clinical and demographic characteristics and therapeutic approaches in HIV/AIDS patients in a regional referral hospital. Methods: We analyzed the medical records of 51 patients with histopathological diagnosis of Kaposi's sarcoma hospitalized at Hospital Universitário João de Barros Barreto (HUJBB) from 2004 to 2015. Results: The study sample consisted of individuals 15 to 44 years of age (80.4%), male (80.4%), single (86.3%), and residing in Greater Metropolitan Belém, Pará State, Brazil. The primary skin lesions identified at diagnosis were violaceous macules (45%) and violaceous papules (25%). Visceral involvement was seen in 62.7%, mainly affecting the stomach (75%). The most frequent treatment regimen was 2 NRTI + NNRTI, and 60.8% were referred to chemotherapy. Study limitations: We assumed that more patients had been admitted to hospital without histopathological confirmation or with pathology reports from other services, so that the current study probably underestimated the number of KS cases. Conclusion: Although the cutaneous manifestations in most of these patients were non-exuberant skin lesions like macules and papules, many already showed visceral involvement. Meticulous screening of these patients is thus mandatory, even if the skin lesions are subtle and localized.

Clinical and laboratory characteristics, staging, and outcomes of individuals with AIDS-associated Kaposi's sarcoma at an university hospital

Abstract: Background: Kaposi's sarcoma continues to be the most common human immunodeficiency virus - associated neoplasm with considerable morbidity and mortality. Objective: To describe the clinical and laboratory characteristics, initial staging, and outcomes of aids patients with Kaposi's sarcoma at an university hospital of Recife, Pernambuco. Methods: This is a descriptive study with analytic character, retrospective, of a case series between 2004 and 2014. Results: Of the 22 patients included in the study, 20 were aged <40 years (72.7%). The majority had CD4+ T lymphocyte counts of <200 cells/mm3 (77.3%) and human immunodeficiency virus loads of <100,000 copies/mL (78.9%). Lesions were most commonly observed on the skin (90%), and internal organs were affected in 11 of the 22 patients. Only 7 (31.8%) of the 22 patients were undergoing antiretroviral therapy (ART) at the time of Kaposis sarcoma diagnosis, and the initial disease staging classification was high risk (Aids Clinical Trials Group Oncology Committee) in 19 of the 22 patients (86.4%). Regarding Kaposi's sarcoma treatment, 17 of 22 patients (77.3%) underwent systemic chemotherapy + ART and 5 were treated exclusively with ART. Eight of the 22 patients died (36.5%); of these, 87.5% had died within one year of Kaposi's sarcoma diagnosis. Limitation of the study: Without a control group, this study cannot be used to generate hypotheses. Conclusions: Despite the association between aids and late Kaposi's sarcoma diagnosis in the study population, including an unfavorable risk at the time of staging, a lower mortality rate was observed relative to other studies; this might be related to access to a specialized health service.

Pulmonary Kaposi's sarcoma in a female patient: Case report / Sarcoma de Kaposi pulmonar em paciente do sexo feminino: Relato de caso

SUMMARY Kaposi’s sarcoma (KS) is a multicentric lymphoproliferative malignancy. Most of the time this tumor is confined to the skin and subcutaneous tissue, but it can present with widespread visceral involvement, such as in the lung. Pulmonary KS is the most frequent form in young adult males, in a ratio of 15:1. The disease usually affects individuals with low CD4 lymphocyte counts (<150-200 cells/mm3). We report a case of a female patient aged 35 years, with the presence of skin lesions, self-limiting episodes of diarrhea and weight loss of 15 kg for nearly 9 months, progressing to persistent fever. AIDS was diagnosed and biopsy of the lesions revealed Kaposi’s sarcoma. Computed tomography of the chest showed peribronchovascular thickening, areas of ground glass opacity, condensations with air bronchograms surrounded by ground glass opacity (halo sign) and bilateral pleural effusion. The diagnosis of pulmonary KS is still a challenge, especially due to the occurrence of other opportunistic diseases that may also occur concurrently. Therefore, suspecting this diagnosis based on clinical and laboratory manifestations, and even more with CT findings, is fundamental, especially in patients who already have the cutaneous form of the disease.

RESUMO O sarcoma de Kaposi (SK) é uma neoplasia angioproliferativa maligna multicêntrica. Na maioria das vezes, esse tumor restringe-se à pele e ao tecido subcutâneo, mas pode cursar com comprometimento visceral amplamente disseminado, como é o caso do pulmão. O SK pulmonar é a forma mais frequente em homens adultos jovens, em uma proporção de 15:1. A doença geralmente acomete indivíduos com baixa contagem de linfócitos CD4 (<150-200 células/mm3). Relatamos um caso de uma paciente do sexo feminino, 35 anos, com presença de lesões cutâneas, episódios de diarreia autolimitada e perda de peso de 15 kg havia aproximadamente 9 meses, evoluindo para quadro febril persistente. Foi dado o diagnóstico de aids e foi realizada biópsia das lesões evidenciando sarcoma de Kaposi. A tomografia computadorizada do tórax evidenciou imagens de espessamento peribroncovascular, áreas de vidro fosco, condensações com broncogramas aéreos circundados por área de vidro fosco (sinal do halo) e derrame pleural bilateral. O diagnóstico da forma pulmonar de SK ainda é um desafio, principalmente pela ocorrência de outras doenças oportunistas que, inclusive, podem ocorrer de forma concomitante. Portanto, a suspeição desse diagnóstico pelas manifestações clínicas, laboratoriais e, principalmente, tomográficas é de fundamental importância, sobretudo em pacientes que já apresentam a forma cutânea da doença.

Intralesional 3% Sodium Tetradecyl Sulfate for Treatment of Cutaneous Kaposi's Sarcoma

No abstract available.

Sarcoma de Kaposi clásico en un paciente joven e inmunocompetente: reporte de un caso / Classic Kaposi's sarcoma in a young, immunocompetent patient: a case report

El sarcoma de Kaposi (SK) es un cáncer que se desarrolla a partir de las células de los vasos sanguíneos o linfáticos. Fue descrito, en 1872, por Moriz Kaposi, y por muchos años se consideró una neoplasia de curso poco agresivo, lo cual cambió con la epidemia del sida. Se han descrito clásicamente cuatro grupos de SK: clásico, endémico, iatrogénico y epidémico. El SK clásico, aparece con mayor frecuencia en pacientes de 60 a 70 años y de origen mediterráneo. Se presenta el caso de un paciente joven de 27 años, inmunocompetente que desarrolla un SK clásico con inicio inusual.

Kaposi's sarcoma is a type of cancer that develops from blood cells or lymphatic vessels. It was described in 1872 by Moriz Kaposi, and for many years was considered an aggressive neoplasm, which changed with the AlDS epidemic. lt has been classically described into four groups of Kaposi's sarcoma: classic, endemic, iatrogenic and epidemic. The classic Kaposi sarcoma occurs more frequent in patients from 60 to 70 years old and Mediterranean origin. We report the case of a young patient of 27 years old, immunocompetent who developed classic Kaposi sarcoma with an un usual start.

Sarcoma de Kaposi en SIDA. Tratamiento con HAART y paclitaxel en tres casos de SK/SIDA con buena respuesta / AIDS-KAPOSI`S SARCOMA: treatment with HAART and paclitaxel on three cases of SK/AIDS with good response

El sarcoma de Kaposi (SK), descripto en 1872 por Moritz Kaposi, constituye en la actualidad la enfermedad maligna más frecuente asociada con la epidemia de SIDA. Generalmente se comporta con gran agresividad y, dejada a su libre evolución, produce la muerte en semanas o meses. Su incidencia ha disminuido drásticamente con la introducción de la terapia antirretroviral de alta actividad (HAART). Cuando hay afectación sistémica está indicada la quimioterapia. Las antraciclinas constituyen las drogas de primera línea y el paclitaxel, de segunda. Presentamos tres pacientes de sexo masculino, de entre 25 y 45 años. Dos de ellos refieren hábitos homosexuales y uno heterosexual, con SK asociado con SIDA. Todos presentaron lesiones mucocutáneas (dos orales y uno conjuntival) y extensión visceral. En los tres casos observamos una respuesta altamente satisfactoria y rápida al tratamiento con paclitaxel en combinación con HAART.

Sarcoma de Kaposi postrasplante renal, evolución favorable al tratamiento / Post-transplantation Kaposi sarcoma: a favorable treatment course

Se presenta el segundo reporte en Cuba de un paciente que se le realizó trasplante renal, quien recibió esquema inmunosupresor a base de Imurán, ciclosporina y prednisona. A los 20 meses de haber realizado el trasplante se consulta por presentar lesiones de tipo máculo, nodulares, en placas de color rojo violáceo distribuidas en todo el cuerpo menos en la cara y el cuello, además de lesiones nodulares en el paladar blando. Se le realizó una biopsia de piel donde se le diagnosticó un sarcoma de Kaposi y se efectuó tinción de Warthin Starry. Se le retiró el tratamiento con ciclosporina y se le redujo la dosis de Imurßn. Las lesiones desaparecieron paulatinamente hasta alcanzar casi un 100 por ciento de recuperación a los 3 meses aproximadamente de haber disminuido la dosis de los inmunosupresores. Las cifras de creatinina no presentaron un aumento significativo a pesar de la reducción de la inmunosupresión. La reducción de la inmunosupresión y la disminución de las dosis de los anticalcineurínicos mejoraron la evolución de las lesiones dermatológicas de dicho paciente.

This is the second report in Cuba from a patient undergoes a renal transplantation who received an immunosuppressive scheme based on Imuran, Cyclosporine and Prednisone. Twenty months post-transplantation is seen in consulting room from macular, nodular, in red-violet plaques lesions distributed in whole body except in the face and neck as well as nodular lesions in soft palate. We obtained a skin biopsy with a diagnosis of Kaposi's sarcoma and Warthin Starry dye was performed. Cyclosporine treatment was stopped and Imuran was decreased. Lesions disappeared gradually until achieving almost a 100 percent of recovery at three months approximately after decrease the immunosuppressive dose. Creatinine figures have not a significant increase despite immunosuppression reduction and decrease of anticalcineurinic dose improved dermatologic lesions course of such patient.

Recurrencia de sarcoma de Kaposi clásico post amputación de pierna derecha: una presentación inusual / Classic Kaposi sarcoma recurrence after right leg amputation: an unusual presentation

La presentación clínica característica del sarcoma de Kaposi (SK) clásico suele involucrar sólo los miembros inferiores, con un curso progresivo y benigno. A continuación reportamos la inusual presentación de un paciente de 52 años de edad con recurrencia de SK clásico posterior a la amputación del miembro inferior derecho. Dentro de los posibles mecanismos implicados se encuentran el trauma, la inflamación y la hipoxia. Son diversos los factores que influyen en la evolución de los pacientes con SK clásico, por lo que una intervención y diagnóstico tempranos son necesarios.

Sarcoma de Kaposi y VIH: tratamiento antirretroviral y quimioterapia en 32 pacientes / Kaposi sarcoma in HIV patients: Response to antiretroviral treatment and chemotherapy

BACKGROUND: Kaposi sarcoma is the most common tumor in HIV infected patients. AIM: To describe a cohort of patients with Kaposi sarcoma. MATERIAL AND METHODS: Retrospective review of HIV patients with Kaposi sarcoma seen at a public hospital and at a HIV clinic. Detailed description of patients subjected to antiretroviral treatment and chemotherapy. RESULTS: Between 1995 and 2002, 1127 HIV infected patients were seen. Of these, 78 had Kaposi sarcoma, 15 were treated with high activity antiretroviral therapy (HAART) and 17 with HAART and chemotherapy. These 32 patients were male, and their mean age was 33 +/- 6 years. The initial CD4 count in 27 of these was 95 +/- 105 cells/ml. The initial viral load in 18 of these was 370,246 +/- 768,693 RNA copies/ml. Of the 15 patients treated with HAART, eight had a complete remission, one had a partial response, one remained stationary and five died. Of the 17 patients treated with HAART and chemotherapy, seven had complete remission, five had a partial response, 2 remained stationary and three died. The lapse of complete remission was 272 days for those treated with HAART and 292 days for those treated with HAART and chemotherapy. CONCLUSIONS: In HIV patients with Kaposi sarcoma, high activity antiretroviral therapy, associated or not to chemotherapy, induces complete remission in 46 per cent of patients and partial remission in 16 per cent.

Treatment of post transplantation kaposi's sarcoma with interferon

Kidney transplanted patients under the immunosuppressive therapy are at risk of acquiring Kaposi's sarcoma [KS]. A 26 years old Somali male patient developed KS [10 months after renal transplantation] whilst on cyclosporin A, azathioprine and prednisolone. The clinical diagnosis of KS was confirmed by the biopsy. There was no lymphadenopathy nor visceral involvement. The patient received recombinant alpha 2a interferon in an subcutaneous injection of 6 million units three times weekly for three months [36 injections]. The KS regressed gradually and disappeared completely with brownish pigmented skin. Now, after more than 20 months, the patient is well with a well functioning graft and there is no recurrence of the KS inspite of the patient is still on the same immunosuppressive therapy

Estudo de fase II do uso da associaçäo de interferon alfa e isotretinoína no tratamento de tumores sólidos avançados / Study of phase II of the use and association of interferon-alpha and isotretinoin in treatment of several solid tumors

Estudos laboratoriais e clínicos sugerem a eficácia e sinergismo da associaçäo de interferon e retinóides na induçäo de regressäo tumoral em uma gama variada de neoplasias. No presente estudo prospectivo de fase II, trinta pacientes portadores de tumores sólidos avançados e já refratários à modalidades terapêuticas usuais receberam ácido 13-cisretinóide, 1mg/Kg/dia, por via oral e interferon alfa 2a, 6MUI/m25 dias por semana, por via subcutânea, por um período de 2 a 6 meses. Dezoito pacientes eram portadoras de carcinoma espinocelular de colo uterino; seis, portadores de carcinoma espinocelular de cabeça e pescoço; cinco de sarcoma de kaposi mais SIDA e resistência secundária à interferonterapia e um, portador de xeroderma pigmentoso e múltiplas lesöes espino e baso-celulares em pele e mucosas. Houve um total de 5(16,6 por cento) respostas objetivas, incluindo 4 remissöes parciais de 1 resposta completa. Cinco pacientes experimentaram estabilizaçäo de sua doença. Somente pacientes com sarcoma de Kaposi näo responderam ao tratamento. A sobrevida mediana livre de progressäo foi de 6 meses (variaçäo de 3 a 11 meses). A toxicidade foi baixa e tolerável, constando principalmente de febre baixa, calafrios, mialgia, ressecamento de pele e adinamia. A presente associaçäo surpreendente consegue induzir respostas tumorais objetivas mesmos em pacientes com tumores escamosos avançados e refratários. Estudos utilizando-a com terapêutica inicial, principalmente na forma neoadjuvante, devem ser realizados