Case Report
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World J Gastroenterol. Nov 21, 2014; 20(43): 16359-16363
Published online Nov 21, 2014. doi: 10.3748/wjg.v20.i43.16359
Giant primary angiosarcoma of the small intestine showing severe sepsis
Mizuna Takahashi, Masanori Ohara, Noriko Kimura, Hiromitsu Domen, Takumi Yamabuki, Kazuteru Komuro, Takahiro Tsuchikawa, Satoshi Hirano, Nozomu Iwashiro
Mizuna Takahashi, Masanori Ohara, Hiromitsu Domen, Takumi Yamabuki, Kazuteru Komuro, Nozomu Iwashiro, Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate 041-8512, Japan
Noriko Kimura, Pathology, National Hospital Organization Hakodate Hospital, Hakodate 041-8512, Japan
Takahiro Tsuchikawa, Satoshi Hirano, Department of Gastroenterological Surgery II, Hokkaido University Hospital, Hokkaido 060-8648, Japan
Author contributions: Takahashi M wrote the manuscript; Tsuchikawa T and Hirano S drafted the manuscript; Domen H, Yamabuki T and Ohara M edited the manuscript; Kimura N contributed to the pathological diagnosis and discussion; and Komuro K and Iwashiro N checked the references.
Correspondence to: Mizuna Takahashi, MD, Departments of Surgery, National Hospital Organization Hakodate Hospital, 18-16 Kawahara-cho, Hakodate 041-8512, Japan. mi.takahashi@sepia.plala.or.jp
Telephone: +81-138-516281 Fax: +81-138-516288
Received: March 17, 2014
Revised: May 14, 2014
Accepted: July 15, 2014
Published online: November 21, 2014
Abstract

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.

Keywords: Angiosarcoma, Small intestine, Prognosis, Sepsis, Immunohistological marker

Core tip: Angiosarcoma represents 1%-2% of soft-tissue sarcomas and most frequently occurs in the subcutis. This tumor may affect internal organs such as the heart, liver, and spleen, and only rarely emerges in the gastrointestinal tract. Details of angiosarcoma of the small intestine are thus particularly scarce. We encountered a case of small intestinal angiosarcoma showing rapid tumor growth. We speculate that the giant tumor became hypoxic due to ischemia, resulting in tumor necrosis and perforation. Postoperative prognosis was very poor, and a multidisciplinary approach to establishing optimal treatment is warranted.