Metastatic epitheloid angiosarcoma causing severe gastrointestinal bleeding

Angiosarcoma is a rare entity, comprising less than 1% of all sarcomas. Epithelioid angiosarcoma with the epithelioid appearance of their cells is a unique morphologic subtype, where the cells carry both the epithelial and the mesenchymal differentiation markers. Considering the location they arise predominantly in the deep soft tissue, but skin and visceral organs are also mentioned as primary sites. Epithelioid angiosarcoma spreads in an early phase to the lymphnodes and to the solid organs. Treatment modalities include surgical resection, radiotherapy and paclitaxel-based chemotherapy. Despite all therapeutic approaches more than 50% of the patients are dead within 2 to 3 years from diagnosis.

Case report: A 29-year-old male patient was admitted to the Emergency Department with severe microcytic anaemia. He has recently been experiencing recurrent tarry black diarrhoea, complained of weakness and dizziness, and had 6 kg of weight loss within a short time. Because of recurrent blood loss he received 15 units of packed red blood cells. As part of the diagnostic work up of anaemia, upper GI endoscopy was performed which detected in the descending part of the duodenum several flat, 4 – 5 mm polypoid, bleeding lesions that were biopsied. So as to determine the extent of small bowel affected by these lesions capsule endoscopy was done which displayed identical polypoid malformations in the jejunum as well. Colonoscopy did not demonstrate any bleeding source. He complained of a constant dull pain in the left leg, and the surface of the tibia felt irregular and painful on palpation. X-ray showed cortical erosions with lytic lesions of the tibia. A result of a previous investigation he has been scheduled for surgical removal of a swannoma-like lesion in the subclavian region revealed by MRI examination done to evaluate hypaesthesia and paraesthesia symptoms due to neuronal compression. Aspiration cytology samples were taken from the tibial mass and the subclavian lesion and were also sent for pathological studies.

Extensive immunohistochemical evaluation including staining for vimentin, CD31 and cytokeratin of all samples concluded the final diagnosis of epithelioid angiosarcoma. The patient was referred to a national oncology centre for further treatment.The diagnosis of angiosarcoma as a specific source of severe intestinal bleeding is difficult to make especially for its rarity.