Introduction: Angiosarcomas are rare tumors, comprising less than 1% of all sarcomas. However, they portend a poor prognosis, as they tend to metastasize early, being of uttermost importance a prompt diagnosis and treatment.
Case description: We present the case of a 55-year-old female with history of kidney transplantation, immunosuppressed with tacrolimus, prednisolone, and mofetil mycophenolate. Fifteen years after the transplant, she developed an ulcerated lesion on the site of a nonfunctioning arteriovenous graft, which was excised. Histology was compatible with a high grade angiosarcoma that invaded the margins, and immunosuppression was switched to everolimus. Staging imaging exams revealed lymph node, muscle, and lung metastases. Shortly after, nodular lesions appeared compatible with local recurrence of the disease, and the patient showed severe deterioration of her clinical condition, being proposed for palliative chemotherapy. However, the disease showed an explosive progression and the patient died before starting the treatment.
Conclusion: This case emphasizes the importance of including inspection of the vascular access (functioning or not) in regular post-transplant consultation and value any alterations in the attempt of a timely diagnosis. Although rare, angiosarcoma is an important entity that should be considered in the differential diagnosis of soft tissue masses arising from a vascular access, especially in immunocompromised patients. Aggressive treatment should be offered whenever possible.
Keywords: Prosthetic grafts; angiosarcoma; dialysis; dialysis access; renal transplant; tumor.