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Table 2 Cases of primary angiosarcoma involving the small intestine reported in the English literature

From: Primary angiosarcoma of the small intestine with metastasis to the liver: a case report and review of the literature

Authors

Sex/age (years)

Primary or secondary

Site

Immunohistochemical staining

History of prior radiation or other predisposing factor

Presentation

Treatment

Follow-up

Maeyashiki et al. [11]

M/72

Indeterminate

Small bowel

Positive for CD31, CD34 and factor VIII

None

Anemia, melaena

Resection and daily blood transfusions

Died on hospital day 103

Siderits et al. [12]

M/79

Primary

Small bowel

Strongly positive for CD31

None

Obstruction

Resection

Unknown

Taxy and Battifora [13]

M/64

Primary

Small bowel

Positive for Factor VIII, collagen type IV and vimentin

Not available

Gastrointestinal bleeding

Resection

Died 1 year after the initial diagnosis

Taxy and Battifora [13]

F/57

Primary

Small bowel

Positive for Factor VIII, collagen type IV

Not available

Not available

Resection

Died shortly after surgery

Chami et al. [14]

M/59

Primary

Small bowel

Weakly positive for factor VIII-related antigen, Ulex europaeus I antigen and cytokeratin

None

Gastrointestinal bleeding, bowel obstruction, anorexiaand weight loss

Resection and transfusions

Died on the 11th day after surgery

Ordonez et al. [15]

M/80

Primary

Small bowel

Positive immunoreaction for FVIII-RAG

None

Anemia, undue tiredness and weakness

Resection

Died on the 20th postoperative day

Hwang et al. [16]

F/60

Primary

Small bowel

Positive for Ulex europaeus agglutinin 1

History of radiotherapy

Diffuse abdominal pain

Resection

Died 2 months after discharge

Mohammed et al. [5]

F/25

Primary

Small bowel

Not available

None

Intermittent abdominal pain, weight loss, abdominal distension, hematemesis and malaena

Resection

Died on the 11th day after surgery

Fraiman et al. [17]

M/85

Primary

Small bowel

Strong positivity for vimentin and CD31; focal positivity for factor VIII and CD34

None

Weight loss, anemia, weakness and abdominal pain

Resection and thalidomide

Not available

Selk et al. [18]

M/57

Primary

Small bowel

Not available

History of radiation therapy

Progressive abdominal distention and shortness of breath

Resection

Died 4 months after surgery

Berry et al. [19]

M/51

Primary

Small bowel

Positive for Ulex europaeus and vimentin

History of 3-year irradiation

Peritonitis

Resection, adriamycin and dacarbazine

Died 5 months after initial presentation

Watanabe et al. [20]

M/64

Primary

Duodenum and upper jejunum

Positive for vimentin and anti-endothelin-1

Not available

Persistent gastrointestinal bleeding

Not available

Died of pulmonary metastasis 1 year after the operation

Al Ali et al. [1]

M/87

Indeterminate

Small bowel

Positive for CD31

None

Lethargy, weakness and anemia

Resection

Died 6 weeks after the initial diagnosis

Khalil et al. [21]

M/68

Primary

Small bowel

Strongly positive for CD31, CD34 and vimentin

30 year history of heavy occupational exposure to radiation and polyvinyl chloride

Gastrointestinal bleeding and melaena

Resection

Died 6 months after initial presentation

Suzuki et al. [22]

F/61

Primary

Ileum

Positive for factor VIII-related antigen and Ulex europaeus agglutinin 1

20 year history of radiotherapy

Abdominal pain

Resection and intra-abdominal cisplatin

Died 1 year after initial presentation

Cilursu [23]

F/74

Indeterminate

Small bowel

Not available

Not available

Melaena

Resection

Not available

Delvaux et al. [24]

M/67

Primary

Small bowel

Positive for CD 31, CD 34, factor VIII-related antigen and keratin

Not available

Weight loss, intermittent severe abdominal pain and melaena

Resection

Died 3 months after diagnosis

Policarpio-Nicolas et al. [25]

F/51

Primary

Small bowel

Positive for CD 31, CD 34 and factor VIII-related antigen

History of irradiation

Abdominal pain

Resection

Died 10 months after laparotomy

Hansen et al. [26]

F/76

Primary

Small bowel

Positive for factor VIII and vimentin

History of irradiation

Watery diarrhea, vomiting, weight loss and abdominal pain

Resection

Died 5 months after operation

Aitola et al. [27]

F/50

Primary

Small bowel

Positive for CD 31, CD 34 and factor VIII-related antigen

≥10 year history of radiotherapy

Intestinal obstruction

Resection followed by combination chemotherapy with doxorubicin

1 year and 9 months after diagnosis, she was alive

Aitola et al. [27]

F/78

Primary

Jejunum

Positive for factor VIII-related antigen, CD31, CD34 and Ulex europaeus

≥10 year history of radiotherapy

Intestinal obstruction

Resection

Died of sepsis 2 years after diagnosis

Knop et al. [28]

M/72

Primary

Small bowel

Not available

Not available

Gastrointestinal bleeding and anemia

Resection

Not available

Ogawa et al. [29]

M/36

Primary

Small bowel

Positive for factor VIII-related antigen

Not available

Abdominal pain and nausea

Surgical treatment

Not available

de Mascarenhas-Saraiva et al. [30]

M/82

Primary

Ileum

Not available

Not available

Melaena and increasing shortness of breath

Surgical treatment and transfusions

Not available

Turan et al. [31]

Not available

Indeterminate

Jejunum

Not available

Not available

Acute abdominal signs

Resection and chemotherapy

Not available

Liu et al. [32]

F/39

Primary

Terminal ileum

Positive for CD31 and CD34

None

Increasing right iliac fossa pain, abdominal bloating and vomiting

Resection and chemotherapy

Not available

Kelemen et al. [33]

M/76

Primary

Small bowel

Positive for CD31

None

Abdominal pain and fatigue

Resection

Died of cardiac arrest on the 9th day after surgery

Fohrding et al. [34]

M/84

Primary

Small bowel

Positive for CD31, cytokeratin and vimentin; slightly weaker for CD34; Focally positive for factor VIII

Not available

Gastrointestinal bleeding

Resection, adjuvant chemotherapy with paclitaxel and transfusion

Not available

Grewal et al. [35]

M/73

Primary

Small bowel

Positive for CD31

None

Gastrointestinal bleeding, weakness and melaena

Resection

Died within 4 months of the diagnosis

M, male; F, female