INTRODUCTION
Epithelioid angiosarcoma (EA) is a rare, more aggressive form of angiosarcoma characterized by anaplastic cells originating from the vessel lining of irregularly blood-filled spaces. EA can resemble a bruise or present as specific or nonspecific nodules. A suspected carcinogen of EA is Dacron®, a polymer used in endografts for abdominal aortic aneurysm repairs. At present, three cases exist in the literature purporting Dacron® associated epithelioid angiosarcoma. We report a case of EA concurrent with placement and location of a Dacron® endograft.
CASE REPORT
A 64-year-old male was transferred from outside hospital for a 2 week history of fever, shortness of breath and a painful skin eruption. His recent history was significant for a type 2 endoleak of his abdominal aortic aneurysm repair that was to be revised. The endograft recently underwent expansion from 4.5 cm to 5.2 cm over the previous six months. He also reported a 15 pound weight loss during this period.
His physical examination was remarkable for numerous and scattered erythematous and purpuric papules and nodules (Figure 1). He had no other skin findings and no lymphadenopathy. The patient’s laboratory workup was significant for leukocytosis (white blood cell count of 16 g/dL) and anemia (hemoglobin 7 g/dL), along with an elevated ESR and CRP. Workup with transesophageal echocardiogram, upper endoscopy, and colonoscopy were unrevealing. A tagged white blood cell count showed no focus of infection but revealed possible osteopenia defect on L3 and a non-specific radiotracer uptake on L5 in proximity where the skin eruption and the endograft were located. MRI revealed interval enlargement of an abdominal aortic aneurysm (0.7 cm in 6 months), compression deformity at L3, two right lung nodules, and mediastinal lymphadenopathy. A punch biopsy was performed for histologic examination and stains. A bone marrow biopsy was also performed.
Microscopic evaluation revealed intradermal proliferation of atypical, epithelioid cells forming vascular channels in a sheet-like and infiltrative pattern. The lesion also showed massive necrosis and extravasated red blood cells with hemosiderin deposition. Endothelial cells were pleomorphic and contained vesicular nuclei with prominent nucleoli as is commonly seen with angiosarcoma; perineural infiltration was also noted. The patchy background harbored inflammatory infiltrate containing numerous eosinophils and lymphocytes (Figure 2). Immunohistological characterization of the tumor cells was unremarkable for S100, D2-40, CD1a, CD45, CD34, SMA, and HHV-8 antigens. Both vWF and FLI-1 antigens stained diffusely and CD31 antigen stained strongly. Factor VIII and AE1/AE3 were positive as well (Figures 3, 4, 5). Therefore, a diagnosis of epithelioid angiosarcoma was confirmed. A bone marrow biopsy performed subsequently revealed metastatic angiosarcoma.
The patient was started on palliative taxol chemotherapy and 3 month follow ultrasound revealed the aneurysm was 4.8 cm with no endoleak
