Primary Epithelioid Angiosarcoma of the Jejunal Mesentery Causing Abdominal Bleeding: Case Report and Literature Review

Figures & data

Figure 1 Abdominal computed tomography shows a slightly high-density heterogeneous mass of 9.3 * 5.9cm in the small intestine, with a density ranging from 34 to 57HU, indicated by yellow arrows (a). Enhanced scanning shows mild heterogeneous enhancement of the tumor, indicated by blue arrows (b).

Figure 2 During the operation, a dark red mass was found on the left side of the upper abdomen, with some of the greater omentum adhering to it. It was approximately 90 cm away from the Treitz ligament and was approximately 10 * 6cm in size (yellow arrows).

Figure 3 The lesion is located in the empty mesangium, with spindle shaped tumor cells and obvious atypia, indicated by yellow arrows (a). Tumor cells surround irregular blood vessel cavities, with green arrows (b and c). Some areas of the tumor show bleeding foci, indicated by blue arrows (d).

Figure 4 Immunohistochemical staining showed that tumor cells expressed CD31(a), CD34(b), Fli-1(c), MDM2(d), ERG(e), and INI1(f).

Table 1 A Retrospective Study Was Conducted on 38 Patients with Primary Gastrointestinal Angiosarcoma

Authors	Sex/Age (Years)	Radiation History	Symptoms	Site	Metastasis	Tumor Markers	Immunohistochemical	Treatment	Survival Time
Ni et al^Citation5	M/33	Not available	Abdominal pain, feeble, fever and weight loss	Small intestine	Liver and mesentery	None	Positive for CD31 and vimentin	Resection	Death 1 month after diagnosis
Tamura et al^Citation15	M/51	Not available	Hematochezia and weight loss	Small intestine	Bone	Not available	Positive for CD31, ERG, AE1/AE3 and CAM5.2	Resection, paclitaxel and adriamycin	Death 7 months after diagnosis
Policarpio et al^Citation25	F/51	History of cervical cancer radiotherapy	Abdominal distension	Small intestine	Mesentery	None	Positive for CD31, CD34 and factor VIII	Resection	Death 10 months after diagnosis
Mohammed et al^Citation6	F/25	Not available	Abdominal pain and weight loss	Small intestine	Colon	Not available	Positive for CD31	Resection	Death 3 months after diagnosis
Yu et al^Citation8	M/75	Not available	Hematochezia and feeble	Stomach	Small intestine	None	Positive for CD7, CD31, CD34, ERG, vimentin and P53	Resection	Death 1 month after diagnosis
Nai et al^Citation10	M/73	None	Black stool and feeble	Small intestine	None	Not available	Positive for CD34, vimentin, wilm’s tumor-1 and vWF	Resection	Death 1 month after diagnosis
Liu et al^Citation14	F/43	None	Abdominal pain, feeble and weight loss	Small intestine	Peritoneum	None	Positive for CD31 and CD34	Resection	Death 3 months after diagnosis
Kolli et al^Citation26	M/76	Not available	Abdominal pain, feeble and weight loss	Small intestine	Mesentery	Not available	Positive for CD31, vimentin and ERG	Resection and paclitaxel	Not available
Zacarias et al^Citation12	M/84	Not available	Gastrointestinal bleeding	Small intestine	None	Not available	Positive for CD34, vimentin and factor VIII	Resection	Not available
Kamocki et al^Citation27	M/68	Not available	Gastrointestinal bleeding	Rectum	None	Not available	Positive for CD31	Resection	Still alive 4 years after diagnosis
Takahashi et al^Citation28	F/85	None	Abdominal distension and fever	Small intestine	Lung and mesentery	None	Positive for CD31, vimentin and factor VIII	Resection	Death 2 months after diagnosis
Abdulfattach et al^Citation29	M/77	None	None	Small intestine	None	Not available	Positive for CD31 and ERG	Resection	Not available
Louie et al^Citation30	M/78	Not available	Cachexia	Colon	Liver and Bone	Not available	Positive for CD31 and ERG	None	Death 1 month after diagnosis
Fraiman et al^Citation11	M/85	Not available	Abdominal pain and weight loss	Small intestine	None	Not available	Positive for CD31, CD34, vimentin and factor VIII	Resection and thalidomide	Still alive 1 year after diagnosis after diagnosis
Khalil et al^Citation31	M/68	Thirty years of radiation history	Abdominal pain and black stool	Small intestine	None	Not available	Positive for CD31 and CD34	Resection	Death 3 months after diagnosis
Huntington et al^Citation32	M/69	Not available	Black stool and feeble	Small intestine	Lung	Not available	Positive for CD31, ERG, AE1/AE3, MNF116, vimentin and factor VIII	Resection	Not available
Delvaux et al^Citation33	M/67	Not available	Abdominal pain	Small intestine	None	None	Positive for CD31	Resection	Death 3 months after diagnosis
Brown et al^Citation13	M/77	Not available	Hematochezia and weight loss	Colon	None	Not available	Positive for CD31, CD34 and factor VIII	Resection	Death 6 months after diagnosis
Lu et al^Citation34	M/67	Not available	Abdominal pain and black stool	Small intestine	Rectum and bone	Not available	Positive for CD7, CD31, CD34, ERG and INI-1	Radiotherapy and chemotherapy	Not available
Wu et al^Citation24	F/78	None	Hematochezia and feeble black stool	Small intestine	None	Not available	Positive for CD31, CD34 and ERG	Resection	Not available

Table 2 A Retrospective Study Was Conducted on 38 Patients with Primary Gastrointestinal Angiosarcoma

Authors	Sex/Age (Years)	Radiation History	Symptoms	Site	Metastasis	Tumor Markers	Immunohistochemical	Treatment	Survival Time
Grewal et al^Citation35	M/73	History of radiation therapy for tonsil cancer	Black stool and feeble	Small intestine	Pharyngeal and mesentery	Not available	Positive for CD31 and CD34	Resection	Death 4 months after diagnosis
Ma et al^Citation36	M/70	None	Abdominal pain, black stool and feeble	Small intestine	Adrenal gland	None	Positive for CD31, CD34, vimentin, ERG and P53	Resection	Death 4 months after diagnosis
Navarro et al^Citation18	M/45	History of pelvic tumor radiotherapy	Abdominal pain, black stool and weight loss	Small intestine	None	Ca125 is 4 times higher than normal	Positive for CD31, AE1/AE3 and factor VIII	Resection	Not available
Watanabe et al^Citation37	M/70	Not available	Hematochezia	Colon	None	Not available	Positive for UEA-I, JC70, AE1, NCL5D3 and vimentin	Resection	Still alive 2 years after diagnosis
Watanabe et al^Citation37	M/64	Not available	Hematochezia	Small intestine	None	Not available	Positive for vimentin	Resection	Death 1 year after diagnosis
Lim et al^Citation38	F/44	Not available	Abdominal pain	Colon	None	None	Positive for CD31 and ERG	Resection and paclitaxel	Death 8 months after diagnosis
Hori et al^Citation39	F/20	Medical examination radiation history	Abdominal pain and hematochezia	Small intestine	Lung	None	Positive for CD31, CD141, ERG, CAM 5.2 and fli-1	Resection, carboplatin and paclitaxel	Still alive 2 years after diagnosis
Hui et al^Citation40	M/57	Not available	Hematochezia	Colon	None	Not available	Positive for CD31, CD34, fli-1, factor VIII, VEGR, EMA and ERG	Resection	Still alive 10 months after diagnosis
Tojo et al^Citation23	F/86	Not available	Oral swelling and feeble	Colon	Oral mucosa	Not available	Positive for CD31 and ERG	None	Death 2 months after diagnosis
Sadhu et al^Citation41	M/63	Not available	Hematochezia	Small intestine	None	Not available	Positive for CD31, PAX8, vimentin, fli-1 and ERG	Resection	Not available
Butrón et al^Citation42	M/59	Not available	Abdominal pain and hematochezia fever	Small intestine	Leg soft tissue	Not available	Positive for CD31, CD34 and fli-1	Resection, adriamycin and cyclophosphamide	Still alive 3 months after diagnosis
Ryu et al^Citation16	M/54	Not available	Abdominal pain and black stool	Small intestine	None	Not available	Positive for CD34, vimentin and factor VIII	Resection, ifosfamide and dacarbazine	Death 5 months after diagnosis
Zhang et al^Citation43	M/52	Not available	Abdominal pain and black stool	Small intestine	None	Not available	Positive for CD31 and CD34	Resection	Death 1 month after diagnosis
Kasper et al^Citation44	M/80	Not available	Leg pain	Colon	Bone	Not available	Not available	Resection	Death 9 months after diagnosis
Radić et al^Citation45	M/61	Not available	Abdominal pain, hematochezia and fever	Colon	Lumbar muscle	None	Positive for CD31, CD34, CD117, ERG and vimentin	Resection	Death 2 months after diagnosis
Castro et al^Citation17	F/11	Not available	Abdominal pain and weight loss	Small intestine	None	None	Positive for CD31	Resection, vincristine and adriamycin	Death 5 months after diagnosis
Hansen et al^Citation46	F/80	History of cervical cancer radiotherapy	Abdominal distension	Colon	Liver and small intestine	Not available	Positive for factor VIII	Resection	Death 1 month after diagnosis
Chahbouni et al^Citation47	M/25	Not available	Abdominal pain and weight loss	Small intestine	Mesentery	Not available	Positive for CD31, CD117 and factor VIII	Radiotherapy and adriamycin	Death 9 months after diagnosis

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