Angiosarcoma is a rare malignancy arising from the endothelial lining of blood vessels and lymphatics, accounting for approximately 2% of all soft tissue sarcomas. It can be seen in all age groups, with maximum incidence in the age group 60–80 years. It commonly affects the skin, liver, heart, and spleen and poses diagnostic challenges due to its nonspecific clinical presentation. Risk factors include chronic lymphedema, prior radiation exposure, chemical carcinogens, and certain genetic mutations. Histologically, angiosarcoma displays variable differentiation with aggressive infiltration. Diagnosis relies on clinical suspicion supported by imaging, histopathology, and immunohistochemistry. Management typically involves wide surgical excision and adjuvant radiotherapy for localized disease, while systemic chemotherapy is used for advanced or metastatic cases. Emerging therapies, including immunotherapy, have shown promising early results but remain under investigation.

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Angiosarcoma in Older Adults

  • Bijoy Shah,
  • McCall Torpey,
  • Tobi Klar

摘要

Angiosarcoma is a rare malignancy arising from the endothelial lining of blood vessels and lymphatics, accounting for approximately 2% of all soft tissue sarcomas. It can be seen in all age groups, with maximum incidence in the age group 60–80 years. It commonly affects the skin, liver, heart, and spleen and poses diagnostic challenges due to its nonspecific clinical presentation. Risk factors include chronic lymphedema, prior radiation exposure, chemical carcinogens, and certain genetic mutations. Histologically, angiosarcoma displays variable differentiation with aggressive infiltration. Diagnosis relies on clinical suspicion supported by imaging, histopathology, and immunohistochemistry. Management typically involves wide surgical excision and adjuvant radiotherapy for localized disease, while systemic chemotherapy is used for advanced or metastatic cases. Emerging therapies, including immunotherapy, have shown promising early results but remain under investigation.