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).

Pseudosarcoma de Kaposi tipo Mali unilateral en paciente con insuficiencia venosa crónica / Unilateral Mali-type Pseudo-Kaposis sarcoma in a patient with chronic venous insufficiency

Introducción: La insuficiencia venosa crónica es el conjunto de síntomas y signos derivados de una hipertensión venosa en los miembros inferiores debido a un mal funcionamiento valvular de los sistemas venosos. Se han asociado varias complicaciones a esta entidad, una de ellas es el pseudosarcoma de Kaposi, enfermedad rara y caracterizada por una proliferación reactiva vascular, con lesiones cutáneas similares a las del sarcoma de Kaposi. Objetivo: Describir el proceso diagnóstico de una paciente con pseudosarcoma de Kaposi tipo Mali unilateral como complicación de la insuficiencia venosa crónica. Presentación del caso: Se presenta una paciente femenina, piel blanca, de 51 años de edad, con lesiones cutáneas de aproximadamente 9 años de evolución, tipo placas eritematomarronáceas y papuloescamocostrosas, en el miembro inferior derecho, que progresivamente fueron extendiéndose; la paciente presentaba además insuficiencia venosa crónica. Se diagnosticó pseudosarcoma de Kaposi tipo Mali unilateral, teniendo en cuenta, fundamentalmente, los antecedentes patológicos personales, el examen clínico realizado, la biopsia de la lesión, la inmunohistoquímica para anticuerpos CD 34 y el dúplex venoso de miembro inferior derecho. Se indicó terapia compresiva de la extremidad y ungüento esteroideo en lesiones, con mejoría de estas. Conclusiones: Una de las complicaciones asociadas a la insuficiencia venosa crónica es el pseudosarcoma de Kaposi. En esta rara entidad cutánea el estudio histopatológico y la inmunohistoquímica desempeñan un papel muy importante para su correcto diagnóstico y diferenciación del sarcoma de Kaposi, principal diagnóstico diferencial a tener en cuenta(AU)

Introduction: Chronic venous insufficiency is the set of symptoms and signs derived from venous hypertension in the lower limbs due to a valvular malfunction of the venous system. Several complications have been associated with this entity; one of them is Pseudo-Kaposi's sarcoma, a rare disease characterized by reactive vascular proliferation with skin lesions similar to those of Kaposi's sarcoma. Objective: To describe the diagnostic process of a patient with unilateral Mali-type Pseudo-Kaposi's sarcoma as a complication of chronic venous insufficiency. Case presentation: Fifty-one-year-old white female patient with about 9-year history of brownish erythematosus-type and squamous, crusty plaque-like skin lesions in the right lower limb that were progressively extending. The patient also presented chronic venous insufficiency. Unilateral Mali-type Pseudo-Kaposi's sarcoma was diagnosed, mainly considering the personal pathological antecedents, the clinical examination performed, the biopsy of the lesion, the immunohistochemistry for CD34 antibodies and the venous duplex imaging of the right lower limb. Compressive therapy of the limb and steroid ointment was indicated for the lesions, with improvement of the condition. Conclusions: Pseudo-Kaposi's sarcoma is one of the complications associated with chronic venous insufficiency. In this rare skin entity, the histopathological study and immunohistochemistry play a very important role in the correct diagnosis and differentiation of Kaposi's sarcoma, being this the main differential diagnosis to take into account(AU)

Etiopathogenic, epidemiologic and clinical-therapeutic comparison of non-hodgkins lymphoma and kaposis sarcoma / Comparação etiopatogênica, epidemiológica e clinicoterapêutica de linfoma não hodgkin e sarcoma de kaposi

ABSTRACT Background: Non-Hodgkin's lymphomas (NHL) are primary neoplasms derived from lymphocytes, and Kaposi's sarcoma (SK) is a multicentric disease of viral etiology and is associated with HIV. Aim: To study the etiopathogenesis and clinical characteristics of NHL and KS, describing their mutual factors. Methods: This retrospective investigation was performed on 101 medical charts. The patients were studied according to their age, gender, and HIV-positivity, following the PRISMA guidelines. The characteristics of the tumors and comorbidities were analyzed according to their age and lymphatic metastasis. Results: The mean age of the patients ranged between 15-87 years for NHL and between 25-54 for KS, but the age of patients with NHL associated with HIV did not surpass 34 years. The ratio male: female was 1,8:1 for NHL, but only men presented KS. HIV-positivity was found in five patients with NHL and in 14 with KS. The stages of NHL were: I (21%), II (18,4%), III (26,3%), and IV (34,2%), but KS were found only at III (40%) and IV (60%) stages. The lymphatic metastases were positive in 62 patients NHL and in four with KS. HIV-positivity occurred in 60% of patients with NHL and in 50% with KS. Conclusion: The HIV seropositivity was revealed for most of patients during the NHL and SK propaedeutic and none of them present clinical manifestations of AIDS. NHL associated with HIV was found only in young patients. NHL and KS patients have similar epidemiological, clinical, and therapeutic characteristics.

RESUMO Racional: Os linfomas não Hodgkin (LNH) são neoplasias primárias derivadas de linfócitos e o sarcoma de Kaposi (SK) é doença multicêntrica de etiologia viral, ambas associadas ao HIV. Objetivo: Avaliar características clínicas dos LNH e SK, relacionando fatores etiopatogênicos mútuos. Métodos: Foram avaliados retrospectivamente 101 prontuários. Os doentes foram analisados quanto a idade, sexo e soropositividade para o HIV, de acordo com o PRISMA guidelines. Os tumores foram classificados por estadiamento, presença de linfonodos regionais invadidos e tipo celular. Resultados: A idade variou entre 15 e 87 anos para o LNH e 25 a 54 anos para o SK, mas a idade dos pacientes com LNH associado com o HIV não ultrapassou 34 anos. A proporção homem: mulher foi de 1,8:1 para o LNH, enquanto SK foi registrado apenas em homens. A soropositividade para o HIV ocorreu em cinco pacientes com LNH e 14 com SK. A invasão de linfonodos regionais foi positiva em 62 com LNH e quatro com SK. Os linfomas foram 27,9% de baixo grau, 17,4% de grau intermediário e 12,8% de alto grau. A soropositividade para HIV, foi diagnosticada durante a propedêutica do tumor em 60% dos pacientes com LNH e 50% dos com SK. Conclusão: A maioria dos pacientes portadores de HIV descobriram a soropositividade durante propedêutica para LNH e SK, sem manifestações clínicas de AIDS. Todos os pacientes com LNH associado com o HIV eram jovens. Pacientes com LNH e com SK apresentam características epidemiológicas, clínicas e terapêuticas semelhantes entre si.

Extensive bulbar conjunctival Kaposi's sarcoma as initial symptom of human immunodeficiency virus / Sarcoma de Kaposi extenso na conjuntival bulbar como apresentação inicial do vírus da imunodeficiência humana

ABSTRACT Here, we present a case in which extensive bulbar conjunctival Kaposi's sarcoma was the initial presentation of human immunodeficiency virus in a 36-year-old man. The patient had a 3-month history of recurrent self-limited inferior conjunctiva hyperemia in the right eye, and presented with a painless bullous lesion in the right inferior bulbar conjunctiva persisting for 15 days. Surgical incision biopsy was performed at five locations and revealed a pattern compatible with Kaposi's sarcoma. Serologic testing was positive for human immunodeficiency virus; however, the patient had no other symptoms, or knowledge of human immunodeficiency virus infection. This case highlights the need to consider Kaposi's sarcoma as an early presentation of human immunodeficiency virus even if the patient denies infection.

RESUMO Este relato de caso apresenta um sarcoma de Kaposi extenso na conjuntival bulbar como a apresentação inicial do vírus da imunodeficiência humana em um homem de 36 anos de idade. O paciente tinha história de hiperemia na conjuntiva inferior do olho direito há 3 meses, autolimitada e recorrente e de surgimento de uma lesão bolhosa indolor no mesmo local 15 dias antes da sua apresentação. Uma biópsia incisional cirúrgica foi realizada e revelou um padrão compatível com sarcoma de Kaposi. Teste sorológico posterior revelou positividade para o vírus da imunodeficiência humana, no entanto, o paciente não apresentou outros sintomas, sinais ou conhecimento prévio sobre a infecção. Como conclusão deste caso, deve se ressaltar que a suspeita do diagnóstico do sarcoma de Kaposi deve ser levantada ainda que na apresentação inicial do vírus da imunodeficiência humana ou mesmo naqueles ainda sem este diagnóstico.

Sindrome de inmunodeficiencia adquirida: compromiso del corazón y de los vasos / AIDS: cardiovascular involvement

Las complicaciones cardiovasculares del SIDA, son a menudo asintomáticas, aunque en ocasiones pueden constituir la causa directa de muerte. Puede comprometerse el pericardio, miocardio, endocardio y los vasos, ya sea como manifestación de la enfermedad de base o como resultado de la terapia antirretroviral y su efecto sobre los factores de riesgo, en el contexto de una patología que es actualmente de evolución crónica y con mejores expectativas de sobrevida gracias a las nuevas drogas empleadas para su control y tratamiento. El objetivo de este artículo es presentar una revisión de los aspectos más relevantes del SIDA que comprometen al corazón y los vasos.

The cardiovascular complications of AIDS, are often asymptomatic, although some may be direct cause of death. Pericardium, myocardium, endocardium, and vessels may be involved as a result of illness or the adverse effects of antiretroviral therapy on risk factors, Today has become a chronic condition with improved life expectancy thanks to the development of new drugs for its treatment and control The aim of this article is to present a review of the most relevant aspects of AIDS involving the heart and vessels.

Manifestaciones dermatológicas asociadas a la infección por VIH/SIDA / Dermatologic manifestations associated with HIV/AIDS

The ongoing human immunodeficiency virus (HIV) infection epidemic coupled with more efficacious and available treatments has led to a larger number of patients living with HIV or AIDS. As a result, skin manifestations related to HIV/AIDS or its therapy have become increasingly more common and are reported to occur in as many as 95% of patients. Herein, we review the most common HIV/AIDS related cutaneous pathologies and classify them into inflammatory, HAART-associated, neoplastic, and infectious manifestations. Cutaneous manifestations should be promptly recognized and treated by physicians and health care personnel in order to provide optimal care.

La epidemia del virus de la inmunodeficiencia humana (VIH) sumado al mayor acceso a terapias antiretrovirales (TARV) han llevado a un aumento del número y la sobrevida de pacientes que viven con esta infección crónica. Se estima que hasta 95% de los pacientes con infección por VIH/SIDA presentarán alguna manifestación cutánea, por lo cual, debemos conocerlas. En la presente revisión se estudiarán las manifestaciones cutáneas de la infección por el VIH/SIDA clasificadas como: manifestaciones inflamatorias, manifestaciones asociadas a la TARV, manifestaciones neoplásicas y manifestaciones infecciosas asociadas a infección por VIH/SIDA (bacterianas, virales, fúngicas y parasitarias). Estas manifestaciones deben ser reconocidas por los médicos y el personal de salud a cargo del tratamiento y control de los pacientes con esta patología crónica.

Sarcoma de Kaposi gástrico en un paciente con virus de inmunodeficiencia humana (VIH) / Gastric Kaposi Sarcoma in a patient with HIV

Hombre de 38 años que consultó por sangrado digestivo de dos días de duración. La esofagogastroduodenoscopía demostró múltiples lesiones nodulares de color púrpura en el antro gástrico. La histopatología mostró un tumor formado por células fusiformes y proliferación vascular, hallazgos consistentes con sarcoma de Kaposi del estómago. Posteriormente se determinó la infección por virus de inmunodeficiencia (VIH) del paciente.

A 38 years old man with 48 hours of gastrointestinal bleeding was admitted to the hospital. The EGD revealed red-purple nodules in the gastric antrum. Histopathologically, there were spindle cells and capillary size vascular proliferation. These findings were consistent with Kaposi sarcoma of the stomach. Immediately after, the patient had a positive test for HIV.

Sarcoma, Kaposi; Gastric antrum; HIV.

Sarcoma de Kaposi por virus del herpes humano de tipo 8 en un receptor de trasplante hepático pediátrico: Caso clínico / Human herpesvirus 8 related Kaposi's sarcoma in a pediatric liver transplant recipient: Case report

Los pacientes que reciben tratamiento inmunosupresor están en riesgo de desarrollar tumores malignos. La infección primaria o reactivación del virus del herpes humano de tipo 8 (HHV-8) puede predisponer al sarcoma de Kaposi después del trasplante de un órgano sólido. En los receptores de trasplantes pediátricos, este sarcoma tiene baja incidencia y mal pronóstico. Se informa la presentación clínica de un sarcoma de Kaposi en un ganglio linfático luego de una infección por HHV-8 en un niño a los 4 meses del trasplante hepático. El paciente tuvo buena evolución con suspensión del tacrolimus y conversión a sirolimus un mes después del diagnóstico. En nuestro conocimiento, este es el primer caso de sarcoma de Kaposi en un receptor pediátrico de trasplante hepático informado en nuestro país y creemos que esta entidad debería considerarse como diagnóstico diferencial en las complicaciones postrasplante.

Patients under immunosuppressive treatment are at risk of developing malignant tumors. Primary infection or reactivation of human herpesvirus 8 (HHV-8) may predispose to Kaposi's sarcoma (KS) after solid organ transplantation. KS in pediatric liver transplant recipients has low incidence and poor prognosis. We report the clinical presentation of a KS in lymph node following HHV-8 infection in a pediatric patient presenting four months after liver transplantation. He had a good outcome with suspension of tacrolimus and conversion to sirolimus one month after diagnosis. To our knowledge, this is the frst KS reported case in our country after liver transplant in a pediatric recipient and we believe that this entity should be taken into consideration in the differential diagnosis of post-transplant complications.

Sarcoma de Kaposi perianal. Presentación de un caso y revisión de la literatura / [Perianal Kaposi’s sarcoma. A case report and a review of the literature].

Kaposi’s sarcoma is the most common cancer in men who have sex with men with AIDS. The estimated prevalence in the United States is 25

in patients with positive serology for the human immunodeficiency virus (HIV). The commitment of the gastrointestinal tract is seen in 40

of patients with Kaposi’s sarcoma related to AIDS but lesions can occur anywhere in the body and evolve rapidly. We present a 33-year-old patient who kept sex with men, with epidemiological history of hepatitis B and syphilis, who consulted the service of Coloproctology for perianal ulcer. He was studied according to the protocols of sexually transmitted diseases, was diagnosed HIV and an excision biopsy of the lesion was performed. The diagnosis of perianal Kaposi’s sarcoma was reached. Kaposi’s sarcoma-HIV was staged, no other lesions were found and the patient started antiretrovirals with poor response to therapy. He evolved with rapid progression of the disease and died with the presumptive diagnosis of Fournier’s syndrome at three months after the excision biopsy. We conclude that perianal ulcers are a relatively common pathology in the office of Coloproctology and differential diagnosis are different according to the positivity for HIV or not and the patient’s sexual practices. We consider that is important to publish and spread these cases.

Regression of conjunctival tumor during dietary treatment of celiac disease.

A 3-year-old girl presented with a hemorrhagic conjunctival lesion in the right eye. The medical history revealed premature cessation of breast feeding, intolerance to the ingestion of baby foods, anorexia, and abdominal distention. Prior to her referral, endoscopic small intestinal biopsy had been carried out under general anesthesia with a possible diagnosis of Celiac Disease (CD). Her parents did not want their child to undergo general anesthesia for the second time for the excisional biopsy. We decided to follow the patient until all systemic investigations were concluded. In evaluation, the case was diagnosed with CD and the conjunctival tumor showed complete regression during gluten-free dietary treatment. The clinical fleshy appearance of the lesion with spider-like vascular extensions and subconjunctival hemorrhagic spots, possible association with an acquired immune system dysfunction due to CD, and spontaneous regression by a gluten-free diet led us to make a presumed diagnosis of conjunctival Kaposi sarcoma.

Sarcoma de kaposi extratorácico: evidencias de una enfermedad multisistémica / Extra-thoracic kaposi's sarcoma: evidence of a multisystemic involvement

Kaposi's sarcoma is a low-grade vascular neoplasm that typically manifests as one of four variants, being the epidemic (or AIDS-related) the best known of them. The pulmonary involvement of this disease is well described and is expressed mainly by bilateral parenchymal nodules of characteristic morphology; however, this tumor produces a multisystemic compromise that is not widely known. Due to the increasing incidence and survival rates of patients with HIV, and the fact that Kaposi's sarcoma is one of its frequent complications, we sought to describe its staging and major imaging findings in computed tomography, given the importance of timely recognition of this neoplasm for a proper prognosis and management of the disease.

El sarco made Kaposies un tumor vascular de bajo grado, que manifesta típicamente cuatro formas diferentes, siendo la epidémica (o relacionada con el SIDA) la más conocida de ellas. El compromiso pulmonar de esta enfermedad está bien descrito y se manifiesta principalmente por nódulos parenquimatosos bilateral es de morfología característica ; sin embargo, esta neoplasia produce un compromiso multisistémico que no es ampliamente conocido. Debido a la creciente incidencia y sobrevida de los pacientes con VIH y el hecho de que el sarcoma de Kaposi es una de sus complicaciones frecuentes, decidimos describir su estadificación y los principales hallazgos imaginológicos en tomografía computada, por la importancia de su conocimiento para el adecuado pronóstico y manejo de esta enfermedad.

Sarkoma de Kaposi clássico / Classic Kaposi's sarcoma

Os autores relatam um caso clínico de sarcoma de Kaposi clássico (SKC) em paciente idoso, masculino, não-HIV-positivo, portador de laudo histopatológico incorreto, o que dificultou o diagnóstico. A conclusão final foi obtida por forte suspeita clínica e solicitação de nova biópsia da lesão com análise imuno-histoquímica.

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.

Kaposi's sarcoma occurring during short-term dialysis: report of two cases

Kaposi's sarcoma (KS) appears to develop in association with kidney transplantation, but unlikely with dialysis. We report two cases of classic KS that occurred in patients receiving short-term (less than 3 yr) dialysis. They have been suffering from chronic renal failure due to tuberculosis and diabetes mellitus, respectively. Several to multiple, reddened-violaceous patches, plaques and nodules were found on the hand and the lower extremities. Laboratory studies showed no evidence suggesting immunosuppressed state and there was no history of taking immunosuppressive agents. The biopsies of the two cases revealed proliferation of spindle-shaped cells focally arranged in bundles and multiple dilated vascular spaces outlined by an attenuated endothelium with intravascular and extravasated erythrocytes. The specimens expressed positivity with CD34 antigen. Human herpesvirus 8 (Kaposi's sarcoma-associated herpesvirus) was detected in one case by polymerase chain reaction method.