Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment

Actas Dermosifiliogr (Engl Ed). 2018 Dec;109(10):878-887. doi: 10.1016/j.ad.2018.06.013. Epub 2018 Sep 24.
[Article in English, Spanish]


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: Angiosarcoma; Cutaneous; Cutáneo; Diagnosis; Diagnóstico; Kaposi sarcoma; Sarcoma; Sarcoma de Kaposi; Tratamiento; Treatment.

Publication types

  • Review

MeSH terms

  • AIDS-Related Opportunistic Infections / diagnosis
  • AIDS-Related Opportunistic Infections / therapy
  • Age Distribution
  • Combined Modality Therapy
  • Female
  • Hemangiosarcoma / diagnosis*
  • Hemangiosarcoma / pathology
  • Hemangiosarcoma / therapy*
  • Herpesvirus 8, Human / isolation & purification
  • Humans
  • Lymphangiosarcoma / diagnosis
  • Lymphangiosarcoma / pathology
  • Lymphangiosarcoma / therapy
  • Male
  • Neoplasms, Second Primary / diagnosis
  • Neoplasms, Second Primary / etiology
  • Neoplasms, Second Primary / therapy
  • Practice Guidelines as Topic*
  • Radiotherapy / adverse effects
  • Sarcoma, Kaposi / classification
  • Sarcoma, Kaposi / diagnosis*
  • Sarcoma, Kaposi / therapy*
  • Sarcoma, Kaposi / virology
  • Skin Neoplasms / diagnosis*
  • Skin Neoplasms / pathology
  • Skin Neoplasms / therapy*
  • Skin Neoplasms / virology

Supplementary concepts

  • AIDS-related Kaposi sarcoma
  • Stewart Treves syndrome