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Giant primary angiosarcoma of the small intestine showing severe sepsis

World Journal of Gastroenterology 2014年第43期20卷 16359-16363页

作者： Mizuna Takahashi Masanori Ohara Noriko Kimura Hiromitsu Domen Takumi Yamabuki Kazuteru Komuro Takahiro Tsuchikawa Satoshi Hirano Nozomu Iwashiro Department of Surgery National Hospital Organization Hakodate HospitalHakodate 041-8512Japan Pathology National Hospital Organization Hakodate HospitalHakodate 041-8512Japan Department of Gastroenterological Surgery II Hokkaido University HospitalHokkaido 060-8648Japan

Primary malignant tumors of the small intestine are rare, comprising less than 2% of all gastrointestinal tumors. An 85-year-old woman was admitted with fever of 40 °C and marked abdominal distension. Her medical history was unremarkable, but blood examination showed elevated inflammatory markers. Abdominal computed tomography showed a giant tumor with central necrosis, extending from the epigastrium to the pelvic cavity. Giant gastrointestinal stromal tumor of the small intestine communicating with the gastrointestinal tract or with superimposed infection was suspected. Because no improvement occurred in response to antibiotics, surgery was performed. Laparotomy revealed giant hemorrhagic tumor adherent to the small intestine and occupying the peritoneal cavity. The giant tumor was a solid tumor weighing 3490 g, measuring 24 cm × 17.5 cm × 18 cm and showing marked necrosis. Histologically, the tumor comprised spindle-shaped cells with anaplastic large nuclei. Immunohistochemical studies showed tumor cells positive for vimentin, CD31, and factor VIII-related antigen, but negative for c-kit and CD34. Angiosarcoma was diagnosed. Although no postoperative complications occurred, the patient experienced enlargement of multiple metastatic tumors in the abdominal cavity and died 42 d postoperatively. The prognosis of small intestinal angiosarcoma is very poor, even after volume-reducing palliative surgery.

关键词： Angiosarcoma Small intestine Prognosis Sepsis immunohistological marker

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