A 53 years old man presented with anemia (hemoglobin 4.5 g/dl), hypalbuminemia, elevated
CRP and leukocytosis. He reported of a normal gastroscopy and colonoscopy 2 weeks
earlier. Repeat gastroscopy showed multiple ulcerations of the whole duodenum with
oozing bleeding. CMV and EBV PCR were negative; sCD25 was normal. After initial stabilization
by red blood cell transfusion, recurrent bleeding enforced repeated enteroscopies
revealing dozens of small oozing ulcerations with polypoid proliferations suspicious
for vasculitis. Argon plasma coagulation (APC) was repeatedly performed on all reachable
lesions.
The CT scan and angiography confirmed the clinical suspicion of vasculitis or histiocytosis
of the small intestine and immunosuppressive treatment was initiated. In spite of
that the patient showed successive clinical decline with steadily increasing daily
transfusion needs (167 blood bags, 17 platelet bags) up to hemorrhagic shock which
lead to an emergency laparotomy. He underwent partial small intestine resection with
intraoperative enteroscopy showing the highest lesion density in the duodenum and
jejunum.
Meanwhile, histopathological analysis of duodenal biopsies revealed epithelioid angiosarcoma,
which was also confirmed in the resected intestinal segment. Since the patient was
unfit for chemotherapy, best supportive care was initiated. The patient died a few
days later. Angiosarcoma is a very rare neoplasm, mostly located in the skin and soft
tissues. Only a few cases have been reported in the intestine but it represents a
critical differential diagnosis of hemorrhagic shock due to intestinal bleeding.
