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Vol. 109. Num. 10.December 2018
Pages e1-e4Pages 851-940
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Vol. 109. Num. 10.December 2018
Pages e1-e4Pages 851-940
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DOI: 10.1016/j.adengl.2018.10.003
Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment
Sarcoma de Kaposi y angiosarcoma cutáneo: directrices para el diagnóstico y tratamiento
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C. Requenaa, M. Alsinab, D. Morgado-Carrascob, J. Cruzc, O. Sanmartína, C. Serra-Guilléna, B. Llombarta,
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beatriz.llombart@uv.es

Corresponding author.
a Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, Spain
b Servicio de Dermatología, Hospital Clínic de Barcelona, Universitat de Barcelona, Barcelona, Spain
c Servicio de Anatomía Patológica, Instituto Valenciano de Oncología, Valencia, Spain
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Table 1. Epidemiology and Clinical Characteristics of the Different Types of Kaposi Sarcoma.
Table 2. Staging Proposed by the AIDS Clinical Trials Groups Oncology Committee.
Table 3. Algorithm for Treatment of Kaposi sarcoma.
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Abstract

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.

Keywords:
Sarcoma
Cutaneous
Angiosarcoma
Kaposi sarcoma
Diagnosis
Treatment
Resumen

El sarcoma de Kaposi es un sarcoma vascular con cuatro variantes clínicas: el clásico, que asienta preferentemente en las extremidades de pacientes ancianos, de curso crónico y poco agresivo; el endémico de África central; el de pacientes inmunodeprimidos, y el asociado a SIDA. En todas las variedades se ha demostrado que el virus herpes tipo8 es el agente etiológico. El angiosarcoma cutáneo es una de las neoplasias cutáneas de peor pronóstico, con gran tendencia a la recidiva local y una supervivencia a 5años del 10-50%. Existen 3 grandes variedades de angiosarcomas cutáneos: los idiopáticos de cara y cuero cabelludo, los desarrollados sobre áreas de linfedema crónico y los que aparecen sobre áreas de piel irradiada. El único tratamiento potencialmente curativo es la cirugía asociada o no a radioterapia, pero su mala delimitación y su carácter multicéntrico obligan en muchos casos a emplear tratamientos paliativos con quimio y/o radioterapia.

Palabras clave:
Sarcoma
Cutáneo
Angiosarcoma
Sarcoma de Kaposi
Diagnóstico
Tratamiento

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