Bleeding "sarcomatosis" as a rare presentation of vascular graft-related angiosarcoma: case report and review of the literature

Abstract

Background: Angiosarcoma is a rare malignant tumor, originating from vascular endothelial cells, accounting for approximatively 1-2% of soft tissue sarcomas. It is characterized by a rapid proliferation and high metastatic potential. Some cases of angiosarcoma are described in association with vascular prosthesis, orthopedic devices and foreign bodies. Hereby, we report a case of a patient treated with the endovascular placement of a PTFE aorto bis-iliac prosthesis for aortic aneurysm, who developed a graft-related angiosarcoma with bone and peritoneal localizations. The peritoneal "sarcomatosis" led to an acute presentation with hemoperitoneum and anemia. We perform a thorough review of the literature summarizing the description of similar cases, their epidemiology and the possibilities for treatment.

Case presentation: An 84-year-old male with a history of abdominal aortic aneurysm endovascular repair presented to our emergency department complaining with low back pain radiating to the left limb. He underwent a type II endoleak embolization of the aneurysmal sac nine days before. During hospitalization he underwent a spine MRI which documented a vertebral alteration of non-univocal interpretation. Vertebral biopsy was performed revealing groups of cells of uncertain nature. He lately underwent percutaneous L2-L4 arthrodesis. Forty-two days after admission, he developed acute anemia. Emergency laparotomy revealed a massive hemoperitoneum and actively bleeding peritoneal nodules. Abdominal packing was performed, and several nodules were sent for definitive histological examination. After surgery, he developed progressive and severe hypovolemic shock and expired on postoperative day 5.

Conclusions: Angiosarcoma associated with foreign bodies, especially vascular prosthesis, is a very rare entity. In patients who have a history of prosthetic vascular graft placement that present with lumbar pain, osteolytic changes at radiologic imaging or the development of ascites, angiosarcoma should be considered in the differential diagnosis. Despite the poor prognosis, a prompt diagnosis might give access to an adequate treatment planning, with the aim for disease control and increased survival.

Keywords: Angiosarcoma; Case report; Sarcomatosis; Vascular-graft.

Fig. 1

Volume rendering CT reconstruction showing the endoprosthesis

Fig. 2

MRI imaging showing the vertebral alteration L3 and the inhomogeneous enhancement of the aortic sac

Fig. 3

CT imaging showing the contiguity between the aortic sac and the affected vertebra (after the L2–L4 arthrodesis)

Fig. 4

CT imaging showing the rapid evolution of the peritoneal localizations and ascites

Fig. 5

Histologic examination of the peritoneal samples. Hematoxylin and eosin (H&E) staining, original magnification Panel a: AX100, Panel b: X200, Panel c: X 400

Fig. 6

Histologic examination of the peritoneal samples. Immunoistochemistry, original magnification X200 Panel a: CD31, Panel b: ERG, Panel c: FLI1, Panel d: HHV8; Panel e: Ki67