The role of gastrointestinal endoscopy in Kaposi sarcoma / El papel de la endoscopia gastrointestinal en el sarcoma de Kaposi

We are writing to make endoscopists aware of the paramount of a prompt diagnosis of gastrointestinal Kaposi sarcoma (GI-KS). Patients with GI involvement have a two to five times higher risk of death and will benefit from chemotherapy to improve their survival. However, current evidence found that one out of three patients might have a false negative result even with HHV-8 since other entities such as gastrointestinal stromal tumors, angiosarcoma, and lymphoma shared macroscopic and histopathological characteristics. These cause a delay in treatment and significantly worsen the prognosis. We observed a trend for a positive diagnosis from ulcers and nodules. To our knowledge, this is the largest cohort of patients with GI-KS in the world. Our study suggests that in cases where a complete immunochemistry panel for KS is not available, HHV-8 remains as a bare minimum. However, other gastrointestinal lesions shared histopathological characteristics. Therefore, we suggest taking biopsies from nodular and ulcer-type lesions to increase the probability to establish a histopathological diagnosis.(AU)

Posttransplant Kaposi sarcoma: Analysis of a series of 13 patients / Sarcoma de Kaposi en pacientes trasplantados: análisis de una serie de 13 casos

Introduction and objectives: This study reflects our experience in the management of posttransplant Kaposi Sarcoma (KS) and assesses the clinical relevance of monitoring HHV-8 DNA viral load in peripheral blood by qPCR.Patients and methodsRetrospective study of all patients diagnosed with posttransplant KS during the period 1995–2019. In 8 patients, we performed a qPCR in serum for HHV-8 DNA detection at diagnosis and/or during follow-up.ResultsData from 13 organ transplant recipients with a diagnosis of iatrogenic KS were collected. Reduction and/or discontinuation of one or more immunosuppressive agent(s) along with switching to an mTOR inhibitor was part of the treatment approach in 12 (92%) patients. Overall response rate (including complete response, partial response, and stable disease) was observed in 9 patients. At diagnosis, HHV-8 qPCR in serum was positive in 2 out of 5 patients. During follow-up, both positive cases turned negative, as a clinical response.ConclusionsOur work highlights the critical role of reduction of immunosuppression and conversion to an mTOR inhibitor in the management of posttransplant KS. (AU)

Introducción y objetivos: Este estudio aporta nuestra experiencia en el manejo del sarcoma de Kaposi (SK) en pacientes trasplantados, y evalúa la relevancia clínica de la monitorización por qPCR de la carga viral del VHH-8 en sangre periférica.Pacientes y métodosEstudio retrospectivo de los pacientes diagnosticados de SK postrasplante en el periodo de 1995-2019. En 8 pacientes, realizamos qPCR en suero para la detección de ADN del VHH-8 en el momento del diagnóstico o durante el seguimiento.ResultadosSe recogieron datos de 13 trasplantados de órgano sólido con diagnóstico de SK. La reducción o discontinuación de uno o más fármacos inmunosupresores junto con el cambio a un inhibidor de mTOR constituyó una parte del tratamiento en 12 (92%) pacientes. Se observó respuesta al tratamiento (incluyendo respuesta completa, parcial o estabilidad) en 9 pacientes. En el diagnóstico, la qPCR en suero de VHH-8 fue positiva en 2 de 5 pacientes. Durante el seguimiento, ambos casos positivos se negativizaron al responder los pacientes al tratamiento.ConclusionesNuestro trabajo muestra el papel esencial de la reducción de la inmunosupresión y la conversión a un inhibidor de mTOR en la estrategia terapéutica de SK en pacientes trasplantados. (AU)

Sarcoma de Kaposi subcutáneo primario, una rara variante clínica / Primary Kaposi Sarcoma of the Subcutaneous Tissue: A Rare Clinical Variant

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Placas infiltradas de rápida evolución en paciente trasplantado / Rapidly progressive infiltrated plaques in a transplant recipient

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Sarcoma de Kaposi de tipo linfangiomatoso / Lymphangioma-like Kaposi sarcoma

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Non-AIDS Kaposi sarcoma in the external ear / Sarcoma de Kaposi del oído externo, no asociado con SIDA

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Derrame linfomatoso primario bilateral, no asociado al herpesvirus humano 8, en un paciente con hipervolemia crónica / Human Herpes Virus 8 Unrelated Bilateral Primary Effusion Lymphoma in a Patient With Chronic Fluid Overload

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Diagnóstico y tratamiento actual de la enfermedad de Castleman / Current diagnosis and treatment of Castleman's disease

La enfermedad de Castleman no es una única enfermedad. Bajo este epónimo se reúne un heterogéneo grupo de trastornos linfoproliferativos no clonales, muy infrecuentes, con un amplio espectro de expresión clínica. Se han descrito 3 tipos histológicos, junto con varias formas clínicas, según la forma de presentación, el sustrato histológico y las enfermedades asociadas. La interleucina 6, los polimorfismos del receptor de esta interleucina, el virus de la inmunodeficiencia humana y el virus herpes humano tipo 8 están implicados en la etiopatogenia y su estudio ha aportado luz al conocimiento de un síndrome cuya incidencia es desconocida. A pesar de avances recientes e importantes en su conocimiento y de la progresiva experiencia terapéutica con rituximab, tocilizumab y siltuximab, aún existen preguntas difíciles de contestar con los factores etiológicos, el abordaje terapéutico óptimo y el pronóstico (AU)

Castleman's disease is not just a single disease but rather an uncommon, heterogeneous group of nonclonal lymphoproliferative disorders, which have a broad spectrum of clinical expression. Three histological types have been reported, along with several clinical forms according to clinical presentation, histological substrate and associated diseases. Interleukin-6, its receptor polymorphisms, the human immunodeficiency virus and the human herpes virus 8 are involved in the etiopathogenesis of Castleman's disease. The study of this disease has shed light on a syndrome whose incidence is unknown. Despite recent significant advances in our understanding of this disease and the increasing therapeutic experience with rituximab, tocilizumab and siltuximab, there are still difficult questions concerning its aetiology, prognosis and optimal treatment (AU)

Neumonía bilateral y tumoraciones cutáneas / Bilateral pneumonia and cutaneous tumours

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Síndrome de Parsonage-Turner durante el tratamiento de la enfermedad de Castleman multicéntrica asociada a VIH y herpes humano 8 / Parsonage-Turner syndrome during the treatment of HIV/HHV8-related multicentric Castleman disease

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Nódulo sésil en mucosa gingival / Sessile Nodule on the Gingival Mucosa

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Herpesvirus / Herpesvirus

El Herpes virus (HSV) destaca por ser el principal responsable de un gran número de infecciones de la región orofacial, así como de la región genital. El virus del herpes simple es el prototipo de una gran familia de virus de doble cadena de ADN, los herpes viridiae, que causan una gran morbilidad en humanos. La infección en las células de la mucosa epitelial da lugar a una serie de signos clínicos y a la infección latente a nivel de las neuronas sensoriales. Durante la fase de infección productiva se expresan múltiples proteínas virales mientras que en fases latentes apenas se expresan dichas proteínas. La reactivación del virus da lugar a infecciones recurrentes, desencadenando en lisis celular y múltiples cuadros con manifestaciones clínicas bien definidas y que desarrollaremos en esta revisión. Por otro lado analizaremos la evidencia disponible que relaciona ciertos virus de esta familia con la progresión de la enfermedad periodontal tanto en adultos como en niños (AU)

The HSV is a virus that causes of a great number of infections both in the orofacial and in the genital area. The herpes simple virus is the prototype of a big family of double DNA strand viruses, the herpes viridiae, which causes a great morbidity in humans. The infection of epithelial cells of the oral mucosa gives rise to a series of clinical signs and symptoms and to a latent infection of the sensitive neurons. During the active phase of infection, multiple viral proteins are expressed while in the latent phase they are barely expressed. When the virus is reactivated, recurrent infection starts, producing cell lisis and different clinical manifestations that we are will reviewed in this article. We will explain the. The fact that certain virus of the herpesvirus family might be related with the progression of periodontal diseases in adults and children which is gaining interest lately (AU)

Fifty-one Kaposi sarcoma patients

INTRODUCTION: Kaposi sarcoma (KS) is a mesenchymal tumor originating from lymphatic endothelial cells. Immunosuppressive patients have higher risk for KS. HHV-8 has a role in immunopathogenesis of KS. Aim Evaluation of demographical properties with tumor characteristics and treatment modalities of KS. MATERIAL AND METHOD: Histopathologically documented KS patients were evaluated retrospectively. Anti-HIV seroprevalence was also evaluated with patient and tumor characteristics besides treatment regimens. RESULTS: Fifty-one patients were included between September 1998 and February 2009. Male/female ratio was 3.25 (39/12). Median age was 68 (31-94). Lower extremity was the most common site whereas excisional biopsy was the most common diagnostic procedure. Smoking rate was 42.8%. Twenty percent had family history for cancer. Anti- HIV seropositivity rate was 1.9%. Thirty eight percent had local monotherapy, and radiotherapy was most common (26%). Multidisciplinary approach rate was 44%. Most of them had surgery and radiotherapy combination. Two-third of the patients had radiotherapy alone or with other modalities. Rates were as 12% for chemotherapy and 6% for interferon. Vincristine-bleomycin-doxorubicin combination was the most preferred regimen (60%). CONCLUSION: Male patients in the sixth decade seem to have higher risk for KS. Smoking rate was almost as high. Local therapy might be sufficient in most of the patients. However, we may also consider systemic chemotherapy for selected patients, including vincristine, bleomycin and doxorubicin (AU)

Sarcoma de Kaposi clásico localizadoen pene / Penile classic Kaposi’s sarcoma

El Sarcoma de Kaposi es un tumor vascular de origen multifocal que se relaciona con el virus herpes humano tipo 8. La afectación genital en el sarcomade Kaposi es rara sobretodo en pacientes VIH negativo. Las lesiones en este área muestran rasgos clínicos e histológicos similares a otras localizaciones,sin embargo, es interesante el diagnóstico diferencial con otras enfermedades que presentan lesiones genitales. Presentamos un caso de sarcoma deKaposi clásico localizado exclusivamente en pene (AU)

Kaposi’s sarcoma is a multifocal vascular tumor which is related to human herpes virus type 8. The genital involvement in Kaposi’s sarcoma is infrequentmostly in HIV - patients. Lesions in this area show similar clinical and histological features as Kaposi’s sarcoma lesions in other locations. However,the differential diagnosis is interesting taking into account other dermatological conditions than present with genital lesions. We report a case ofclassic Kaposi’s sarcoma located in the penis (AU)

Classic Kaposi’s sarcoma presenting in the oral cavity of two HIV-negative Quechua patients

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Traditionally, classic KS lesions have a general distribution, often involving the skin of the feet and legs, and to a lesser extent, that of the hands, arms, and trunk. Oral involvement is a rare manifestation. Initial oral involvement is an even rarer occurrence. We report two unusual cases of classic KS presenting in the oral cavity of two patients from indigenous origin; the first patient with primary oral KS lesion on the hard palate, with no other signs of the condition in any other region of the body; the second patient with generalized dermal KS lesions with lymph node and lower lip involvement. In conclusion, clinicians and pathologists should be aware of the typical clinical, gross, and histologic features of KS. Moreover, we would like to emphasize that oral KS may affect patients without AIDS or exposure to immunosuppression. The awareness of oral classic KS as a diagnostic possibility is important in the work-up of vascular lesions in the oral cavity of non-immunosuppressed individuals

Enfermedad de Castleman subcutánea: un caso adicional / Castleman’s disease of the subcutis

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Disseminated classic Kaposi's sarcoma

Kaposi's sarcoma (KS) is characterised by proliferation of vascular endothelial and lymphoreticular cells, frequently with a multicentric expression developed from a single node and evolving to multiple cutaneous lumps or plaque-like appearance. Four types of KS with similar histological patterns have been described in terms of their clinical and epidemiological features: classic KS, endemic (African) KS, iatrogenic KS and epidemic (AIDS-related) KS. The differences in clinical features are quite relevant: classic KS is usually limited to the lower extremities; whereas immunodeficiency-related diseases frequently involve several organs. A case of a 67-year-old woman with metastatic KS and unproven immunodeficiency is presented (AU)

Dos enfermedades relacionadas con el VHH-8 en un paciente VIH-negativo: sarcoma de Kaposi y enfermedad de Castleman multicéntrica. Respuesta a tratamiento con rituximab y CHOP / Two HHV8-related illnesses in a HIV-negative patient: Kaposi’s sarcoma and multicentric castleman’s disease. Response to treatment with Rituximab and CHOP

El virus herpes humano tipo 8 (VHH-8) se descubrió en 1994 a partir de la biopsia de un sarcoma de Kaposi en un paciente con sida. Desde entonces se ha identificado en todas las variantes de sarcoma de Kaposi y en otros dos raros procesos: la enfermedad de Castleman multicéntrica y el linfoma primario de cavidades. Se presenta el caso de un paciente varón de 68 años de edad VIH-negativo con sarcoma de Kaposi de un año de evolución en seguimiento por Dermatología que consultó por astenia, anorexia y fiebre. A la exploración se detectaron múltiples adenopatías laterocervicales, axilares e inguinales. En la biopsia de una de dichas adenopatías se apreciaron hallazgos característicos de la variante de células plasmáticas de la enfermedad de Castleman. Se realizaron serologías para el VHH-8 y el VIH que resultaron positiva y negativa respectivamente (IgG anti-VHH-8 positivo, título 1/640, inmunofluorescencia indirecta). Mediante reacción en cadena de la polimerasa se amplificó VHH-8 en sangre periférica. El paciente recibió tratamiento con 8 ciclos de CHOP y rituximab con una completa resolución de las adenopatías y la sintomatología general sin observarse empeoramiento de su sarcoma de Kaposi. El paciente permanece en remisión completa 10 meses después de finalizado el tratamiento. Se presenta el caso de un paciente VIH-negativo y VHH-8-positivo, diagnosticado de sarcoma de Kaposi clásico que desarrolló una enfermedad de Castleman multicéntrica variante de células plasmáticas. La coincidencia de dos o más enfermedades relacionadas con el VHH-8 en un paciente VIH-negativo es un hecho descrito raramente en la literatura. El tratamiento con rituximab combinado con quimioterapia tipo CHOP ha demostrado ser eficaz en este caso sin que haya podido observarse un agravamiento de su SK

Human herpes virus 8 (HHV8) was discovered in 1994 in the biopsy of a Kaposi's sarcoma in a patient with AIDS. Since then it has been identified in all variants of Kaposi's sarcoma and in another two rare disorders: multicentric Castleman's disease and primary body-cavity based lymphomas. The case discusses a 68 year old, HIV-negative male patient, presenting Kaposi's sarcoma for one year and being monitored by dermatology, who presented for weakness, anorexia and fever. On examination, he was found to have adenitis of the lymph nodes in his neck, underarm and groin. A biopsy on one of the swellings led to findings characteristic of multicentric plasma cell variant Castleman's disease. Blood tests for HHV8 and HIV were carried out, resulting positive and negative respectively (IgG anti-HHV8 positive, title 1/640, indirect immunofluorescence). PCR amplification showed HHV8 in peripheral blood. Patient received 8 cycles of CHOP and rituximab, leading to complete disappearance of the adenitis and general symptoms, with no worsening of his Kaposi's sarcoma. Patient remained in complete remission for 10 months after treatment. This paper discusses the case of a HIV­, HHV8+ patient, diagnosed with classic Kaposi's sarcoma, who developed multicentric plasma cell variant Castleman's disease. The coincidence of two or more HHV8-related illnesses in a HIV-negative patient has rarely been described in medical literature. Treatment with rituximab combined with CHOP chemotherapy was effective in this case, and no worsening of the patient's KS was observed