Small intestinal angiosarcoma on clinical presentation, diagnosis, management and prognosis: A case report and review of the literature

Table 1 The basic clinicopathological factors of 82 collected cases

Characteristics	Number of patients	%
Gender
Male	55	67.07
Female	27	32.93
Age
≤ 65	34	41.46
> 65	47	57.32
NA	1	1.22
Race
North America	35	42.68
European	17	20.73
Asia	26	31.71
other	4	4.88
Year
≤ 2000	20	24.39
> 2010	62	75.61
Tumor origin
Primary	62	75.61
Secondary	14	17.07
NA	6	7.32
Radiation history
With	21	25.61
Without	61	74.39

NA: Not available.

Table 2 Age distribution characteristics of patients

Gender	Number	Mean age	SD	Median age	Range of age
Female	26	60.85	22.73	70	20-92
Male	55	64.44	14.92	68	25-87
All	82	63.28	17.74	68	20-92

Table 3 Patients’ symptoms during the disease

Symptoms	Number	Percentage (%)
Gastrointestinal bleeding	51	62.20
Anemia	47	57.32
Abdominal pain	31	37.80
Weak	19	23.17
Loss of weight	15	18.29
Short of breath	13	15.85
Nausea	11	13.41
Abdominal distention	10	12.20
Loss of appetite	8	9.76
Dizziness	5	6.10
Fever	3	3.66
Constipation	3	3.66
Chest pain	3	3.66
Back pain	3	3.66
Diarrhea	2	2.44
Syncope	2	2.44
Drowsiness	1	1.22
Peripheral edema	1	1.22
Lower limb weakness	1	1.22

Table 4 Characteristics of immunohistochemistry results

Pathological markers	Total	Positive	Negative
CD31	49	49	0
CD34	40	30	10
Vimentin	28	28	0
VIII	29	26	3
ERG	10	10	0
FLI-1	6	6	0
von Willebrand factor	2	2	0
EMA	17	1	16
SMA	10	0	10
CD117	10	0	10
Desmin	11	0	11
S100	28	1	27

VIII: Factor VIII-related antigen; ERG: ETS-related gene; FLI-1: Friend leukemia integration 1; EMA: Epithelial membrane antigen; SMA: Smooth muscle actin.

Table 5 Location of small intestinal angiosarcoma

Tag	Location	Number	Percentage (%)
A	Jejunum	23	28.0
B	Ileum	16	19.5
C	Duodenum	10	12.2
D	Duodenum, Jejunum and Ileum	10	12.2
E	Duodenum and Jejunum	9	11.0
F	Jejunum and Ileum	5	6.1
G	Unspecified small intestine	9	11.0

Table 6 Characteristics of small intestinal angiosarcoma lesions

Characteristics	Number	%
Size
≤ 40 mm	38	45.78
> 40 mm	14	16.87
Diffuse	2	2.41
NA	29	34.94
Small intestinal lesions
Single focal	26	35.6
Multifocal	47	64.4
NA	9
Pathological morphology
Ulcerative	13	15.66
Protuberant	41	49.4
Superficial	8	9.64
Diffuse infiltrating	6	7.23
NA	15	18.07
Infiltration depth
Mucosa	3	3.61
Submucosa	17	20.48
Muscularis propria	7	8.43
Serosa	17	20.48
Under the serosa	1
NA	39	46.99

NA: Not available.

Table 7 Distant metastatic site of primary small intestinal angiosarcoma

Distant metastasis	Number	%
No metastasis	23	37.1
Lung	14	22.6
Liver	13	21.0
Large intestine	13	21.0
Spleen	5	8.1
Bone	5	8.1
Pleural	4	6.5
Stomach	4	6.5
Bladder	3	4.8
Kidney	2	3.2
Vein	2	3.2
Abdominal wall	2	3.2
Gallbladder	2	3.2
Pancreas	1	1.6
Heart	1	1.6
Adrenal gland	1	1.6
Pelvic cavity	1	1.6
Brain	1	1.6
Oropharynx	1	1.6
Diaphragm	1	1.6
NA	4	6.5
All	62	100%

NA: Not available.

Table 8 Analysis of risk factors for small intestinal angiosarcoma

Gender/age (yr)	Time1 (yr)	Cause of radiation	Radiation area	Dose (Gray)
F/66	20	Uterus cancer	Pelvis	NA
F/69	7	Adenocarcinoma of uterus	Pelvis	NA
F/50	14	Adenocarcinoma of uterine body	Pelvis	55.6
F/76	7	Adenocarcinoma of uterine body	Pelvis	45.1
F/78	10	Endometrial cancer	Pelvis	55.5
F/51	9	Adenocarcinoma of cervix	Pelvis	50
F/80	20	Squamous cell carcinoma of cervix	Pelvis	55
F/61	20	Squamous cell carcinoma of cervix	Pelvis	NA
F/NA	8	Cervical cancer	Pelvis	NA
F/72	24	Leiomyosarcoma of uterus	Pelvis	NA
F/26	14	Dysgerminoma of ovary	Pelvis	48
F/66	8	Ovarian cancer	Pelvis	60
F/88	18	Breast cancer	Chest	NA
F/37	NA	Breast cancer	Chest	NA
F/92	12	Colon cancer	Pelvis	NA
M/82	NA	Prostate cancer	Pelvis	NA
M/80	NA	Prostate cancer	Pelvis	NA
M/73	NA	Squamous cell carcinoma of tonsil	Neck	NA
M/63	45	Left lower abdominal lymphoma	Abdomen	15
M/57	8	Chondrosarcoma of right hemipelvis	Pelvis	NA
M/68	30	Occupational exposure	NA	Heavy

¹Time: Duration from exposure to risk factors to diagnosis of small intestinal angiosarcoma.

M: Male; F: Female; NA: Not available.

Table 9 Results of univariate COX regression analysis

Factors	HR (98%CI)	P value
Gender (male/female)	1.395 (0.772-2.521)	0.270
Age (> 65/≤ 65)	1.803 (1.007-3.227)	0.047
Tumor origin (secondary/primary)	1.708 (0.789-3.696)	0.174
Tumor size(> 40 mm/≤ 40 mm)	1.265 (0.578-2.767)	0.557
Tumor lesion (multifocal/single focal)	1.365 (0.722-2.579)	0.338
Distant metastases (yes/no)	1.140 (0.573-2.269)	0.708
Acute abdominal disease (yes/no)	0.780 (0.424-1.435)	0.424
Surgery (with/without)	0.570 (0.296-1.100)	0.094
Chemotherapy (with/without)	0.473 (0.238-0.940)	0.032
Gastrointestinal bleeding (yes/no)	1.076 (0.604-1.915)	0.803
Tumor distribution
Two segments/one segment	2.116 (0.975-4.593)	0.058
Whole intestine/one segment	2.473 (1.156-5.289)	0.020

Table 10 Results of multivariate COX regression analysis

Factors	HR (98%CI)	P value
Chemotherapy (with/without)	0.442 (0.205-0.956)	0.038
Surgery (with/without)	0.407 (0.182-0.908)	0.028
Age (> 65/≤ 65)	1.944 (0.969-3.902)	0.061
Tumor distribution
Two segments/one segment	0.434 (0.194-0.969)	0.042
Whole intestine/one segment	0.820 (0.323-2.086)	0.677