title: Giant primary angiosarcoma of the small intestine showing severe sepsis.
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.
authors.affiliation: Mizuna Takahashi, Masanori Ohara, Hiromitsu Domen, Takumi Yamabuki, Kazuteru Komuro, Nozomu Iwashiro, Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate 041-8512, Japan.
authors.forename: Mizuna
authors.initials: M
authors.lastname: Takahashi
authors.affiliation: Mizuna Takahashi, Masanori Ohara, Hiromitsu Domen, Takumi Yamabuki, Kazuteru Komuro, Nozomu Iwashiro, Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate 041-8512, Japan.
authors.forename: Masanori
authors.initials: M
authors.lastname: Ohara
authors.affiliation: Mizuna Takahashi, Masanori Ohara, Hiromitsu Domen, Takumi Yamabuki, Kazuteru Komuro, Nozomu Iwashiro, Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate 041-8512, Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Kimura
authors.affiliation: Mizuna Takahashi, Masanori Ohara, Hiromitsu Domen, Takumi Yamabuki, Kazuteru Komuro, Nozomu Iwashiro, Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate 041-8512, Japan.
authors.forename: Hiromitsu
authors.initials: H
authors.lastname: Domen
authors.affiliation: Mizuna Takahashi, Masanori Ohara, Hiromitsu Domen, Takumi Yamabuki, Kazuteru Komuro, Nozomu Iwashiro, Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate 041-8512, Japan.
authors.forename: Takumi
authors.initials: T
authors.lastname: Yamabuki
authors.affiliation: Mizuna Takahashi, Masanori Ohara, Hiromitsu Domen, Takumi Yamabuki, Kazuteru Komuro, Nozomu Iwashiro, Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate 041-8512, Japan.
authors.forename: Kazuteru
authors.initials: K
authors.lastname: Komuro
authors.affiliation: Mizuna Takahashi, Masanori Ohara, Hiromitsu Domen, Takumi Yamabuki, Kazuteru Komuro, Nozomu Iwashiro, Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate 041-8512, Japan.
authors.forename: Takahiro
authors.initials: T
authors.lastname: Tsuchikawa
authors.affiliation: Mizuna Takahashi, Masanori Ohara, Hiromitsu Domen, Takumi Yamabuki, Kazuteru Komuro, Nozomu Iwashiro, Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate 041-8512, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Hirano
authors.affiliation: Mizuna Takahashi, Masanori Ohara, Hiromitsu Domen, Takumi Yamabuki, Kazuteru Komuro, Nozomu Iwashiro, Department of Surgery, National Hospital Organization Hakodate Hospital, Hakodate 041-8512, Japan.
authors.forename: Nozomu
authors.initials: N
authors.lastname: Iwashiro
mesh_terms: D000369:Aged, 80 and over; D001344:Autopsy; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D046152:Gastrointestinal Stromal Tumors / Q000737:chemistry / Q000150:complications* / Q000556:secondary / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D007414:Intestinal Neoplasms / Q000737:chemistry / Q000150:complications* / Q000473:pathology / Q000601:surgery; D007421:Intestine, Small / Q000737:chemistry / Q000473:pathology / Q000601:surgery*; D018805:Sepsis / Q000175:diagnosis / Q000209:etiology*; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D047368:Tumor Burden
chemical_list: D014408:Biomarkers, Tumor
keywords: Angiosarcoma; Immunohistological marker; Prognosis; Sepsis; Small intestine