Revisión
Sarcoma de Kaposi y angiosarcoma cutáneo: directrices para el diagnóstico y tratamientoKaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment

https://doi.org/10.1016/j.ad.2018.06.013Get rights and content

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 tipo 8 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 5 años 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.

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. Type 8 human herpesvirus is the etiologic agent in all 4 variants. 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 5 years. 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.

Section snippets

Sarcoma de Kaposi

El sarcoma de Kaposi (SK) es un tumor angioproliferativo asociado a la infección por virus herpes humano tipo 8 (VHH-8)1, 2. Se describen 4 variantes (tabla 1):

Sarcoma de Kaposi clásico. Es un tumor infrecuente que afecta a hombres1, 3 del área mediterránea o centroeuropea, con una incidencia entre 0,18-13,2 casos/1064, siendo más frecuente en hombres con edema crónico de piernas, diabetes mellitus y usuarios de corticoides. Se presenta como placas o nódulos eritematovioláceos, únicos o

Angiosarcoma cutáneo

Los angiosarcomas representan entre el 1 y el 2%25, 26 de todos los sarcomas, pero al menos la mitad de ellos son cutáneos. De los sarcomas cutáneos, el angiosarcoma es el cuarto en frecuencia, por detrás del sarcoma de Kaposi, el dermatofibrosarcoma y el leiomiosarcoma. El angiosarcoma cutáneo es una de las neoplasias cutáneas de peor pronóstico, con supervivencia a los 5 años de entre el 10% en las series más antiguas27 y el 30 y el 50% en las más modernas25, 28, 29. Existen 3 grandes

Conflicto de intereses

Los autores declaran no tener ningún conflicto de intereses.

Bibliografía (62)

  • T. Mentzel et al.

    Postradiation cutaneous angiosarcoma after treatment of breast carcinoma is characterized by MYC amplification in contrast to atypical vascular lesions after radiotherapy and control cases: Clinicopathological, immunohistochemical and molecular analysis of 66 cases

    Mod Pathol.

    (2012)
  • W. Shon et al.

    MYC amplification and overexpression in primary cutaneous angiosarcoma: A fluorescence in-situ hybridization and immunohistochemical study

    Mod Pathol.

    (2014)
  • A.M. Udager et al.

    MYC immunohistochemistry in angiosarcoma and atypical vascular lesions: Practical considerations based on a single institutional experience

    Pathology.

    (2016)
  • H.J. Kahn et al.

    Monoclonal antibody D2-40, a new marker of lymphatic endothelium, reacts with Kaposi's sarcoma and a subset of angiosarcomas

    Mod Pathol.

    (2002)
  • S. Breiteneder-Geleff et al.

    Angiosarcomas express mixed endothelial phenotypes of blood and lymphatic capillaries: Podoplanin as a specific marker for lymphatic endothelium

    Am J Pathol

    (1999)
  • J.E. Grundahl et al.

    Cutaneous angiosarcoma of head and neck: A new predictive score for locoregional metastasis

    Transl Oncol.

    (2015)
  • T. Dettenborn et al.

    Prognostic features in angiosarcoma of the head and neck: A retrospective monocenter study

    J Craniomaxillofac Surg.

    (2014)
  • A. Lindford et al.

    Surgical management of radiation-associated cutaneous breast angiosarcoma

    J Plast Reconstr Aesthet Surg.

    (2011)
  • M. Veliz et al.

    Durable response of angiosarcoma of the face and scalp to docetaxel

    Clin Oncol (R Coll Radiol).

    (2007)
  • E. Pasquier et al.

    Effective management of advanced angiosarcoma by the synergistic combination of propranolol and vinblastine-based metronomic chemotherapy: A bench to bedside study

    EBioMedicine.

    (2016)
  • E. Martro et al.

    Risk factors for human Herpesvirus 8 infection and AIDS-associated Kaposi's sarcoma among men who have sex with men in a European multicentre study

    Int J Cancer.

    (2007)
  • B. Brenner et al.

    Tailoring treatment for classical Kaposi's sarcoma: Comprehensive clinical guidelines

    Int J Oncol.

    (1999)
  • M. Geddes et al.

    Kaposi's sarcoma in Italy before and after the AIDS epidemic

    Br J Cancer.

    (1994)
  • PDQ Adult Treatment Editorial Board. Kaposi Sarcoma Treatment (PDQ®): Health Professional Version. En: PDQ Cancer...
  • D. Raeisi et al.

    Kaposi's Sarcoma after kidney transplantation: A 21-years experience

    Int J Hematol Oncol Stem Cell Res.

    (2013)
  • S. Grabar et al.

    Differential impact of combination antiretroviral therapy in preventing Kaposi's sarcoma with and without visceral involvement

    J Clin Oncol

    (2006)
  • F. Lanternier et al.

    Kaposi's sarcoma in HIV-negative men having sex with men

    AIDS

    (2008)
  • S.E. Krown et al.

    AIDS-related Kaposi's sarcoma: Prospective validation of the AIDS Clinical Trials Group staging classification AIDS Clinical Trials Group Oncology Committee

    J Clin Oncol.

    (1997)
  • G. Di Lorenzo et al.

    Pegylated liposomal doxorubicin as second-line therapy in the treatment of patients with advanced classic Kaposi sarcoma: A retrospective study

    Cancer.

    (2008)
  • L. Brambilla et al.

    Mediterranean Kaposi's sarcoma in the elderly. A randomized study of oral etoposide versus vinblastine

    Cancer.

    (1994)
  • M. Bower et al.

    Prospective stage-stratified approach to AIDS-related Kaposi's sarcoma

    J Clin Oncol.

    (2014)
  • Cited by (29)

    • Skin lesions in a patient recently diagnosed with HIV

      2023, FMC Formacion Medica Continuada en Atencion Primaria
    • Emerging therapies for rare cutaneous cancers: A systematic review

      2021, Cancer Treatment Reviews
      Citation Excerpt :

      Of note, however, response rate to treatment were below 60% in clinical trials and neutropenia was a concern for all studies [7]. Pegylated liposomal doxorubicin (PLD) is commonly used for disseminated disease in patients with or without HIV, while pomalidomide is preferred for refractory therapy [46]. Anti-proliferative agents, including pomalidomide and lenalidomide, have clinical trials with the most active recruitment, aligning with NCCN guidelines for systemic therapy and clinical trial enrollment.

    View all citing articles on Scopus
    View full text