J Clin Med Res

Journal of Clinical Medicine Research, ISSN 1918-3003 print, 1918-3011 online, Open Access

Article copyright, the authors; Journal compilation copyright, J Clin Med Res and Elmer Press Inc

Journal website http://www.jocmr.org

Review

Volume 10, Number 4, April 2018, pages 294-301

Primary Small Intestinal Angiosarcoma: Epidemiology, Diagnosis and Treatment

Figure 1. Age distribution in male and female patients of small intestinal angiosarcoma. There is no significant difference in age distribution between male and female genders (P = 0.677, Wilcoxon two-sample test, n = 28 and 18, respectively; the age of one male patient is missing).

Figure 2. (a) Survival analysis of patients with small intestinal angiosarcoma. (b) There is no gender difference in the survival times between male and female patients (0 = female, 1 = male; Log-rank test, z = 0.17, P = 0.86, n = 15 and 26, respectively).

Figure 3. Survival times in different treatment groups. The survival time is longer in patients received resection/chemotherapy compared to that in patients underwent surgery alone (*P = 0.0275; Log-rank test; n = 7 and 26, respectively). The survival times in other treatment groups are not compared due to the limited case numbers. *Compared to treatment with patients treated with surgery only. APC: argon plasma coagulation; Chemo: chemotherapy; NA: the other modality not available; Rad: radiation therapy; Surg: surgery.

**Table 1.** Age and Gender Characteristics of Patients With Small Intestinal Angiosarcoma
Gender	Range of age (years)	Median age (years)	Mean age (years)	n
Female	25 - 85	63.5	62.4	18
Male	25 - 87	69.5	64.7	29

Table 1. Age and Gender Characteristics of Patients With Small Intestinal Angiosarcoma

Gender

Range of age (years)

Median age (years)

Mean age (years)

Female

25 - 85

63.5

62.4

Male

25 - 87

69.5

64.7

**Table 2.** Symptoms and Signs in Patients With Small Intestine Angiosarcoma
Symptom	Number of cases	Percentage (%)
Abd: abdominal; GI: gastrointestinal.
Abd pain	19	41.3
Anemia	16	34.8
GI bleed	12	26.1
Fatigue/weakness	9	19.6
Weight loss	8	17.4
Dyspnea	8	17.4
Abd distension/bowel obstruction	6	13.0
Anorexia	5	10.9
Nausea/vomiting	4	8.7
Acute abdomen	4	8.7
Perforation	4	8.7
Diarrhea	2	4.3
Chest pain	1	2.2

Table 2. Symptoms and Signs in Patients With Small Intestine Angiosarcoma

Symptom

Number of cases

Percentage (%)

Abd: abdominal; GI: gastrointestinal.

Abd pain

41.3

Anemia

34.8

GI bleed

26.1

Fatigue/weakness

19.6

Weight loss

17.4

Dyspnea

17.4

Abd distension/bowel obstruction

13.0

Anorexia

10.9

Nausea/vomiting

8.7

Acute abdomen

8.7

Perforation

8.7

Diarrhea

4.3

Chest pain

2.2

**Table 3.** Risk Factor for Small Intestinal Angiosarcoma in Reported Cases
Risk factor	Number of cases	Percentage (%)
Hemodialysis	1	2.2
Radiation only	16	35.6
Radiation/Polyvinyl	1	2.2
Unspecified	27	60.0
Total	45	100.0

Table 3. Risk Factor for Small Intestinal Angiosarcoma in Reported Cases

Risk factor

Number of cases

Percentage (%)

Hemodialysis

2.2

Radiation only

35.6

Radiation/Polyvinyl

2.2

Unspecified

60.0

Total

100.0

**Table 4.** Location of Small Intestinal Angiosarcoma
Location	Percentage (%)
Duodenum	4.3
Duodenum/Jejunum	10.6
Duodenum/Jejunum/Ileum	2.1
Jejunum	27.7
Ileum	29.8
Jejunum/Ileum	2.1
Unspecified small intestine	23.4
Total	100.0

Table 4. Location of Small Intestinal Angiosarcoma

Location

Percentage (%)

Duodenum

4.3

Duodenum/Jejunum

10.6

Duodenum/Jejunum/Ileum

2.1

Jejunum

27.7

Ileum

29.8

Jejunum/Ileum

2.1

Unspecified small intestine

23.4

Total

100.0

**Table 5.** Sensitivity and Specificity of Some Markers for Angiosarcoma Diagnosis [2, 46-51]
Marker	Sensitivity	Specificity
ERG: erythroblast transformation specific related gene; UEA-1: Ulex europaeus agglutinin 1.
CD31	77-100%	High
CD34	40-100%	Low
vWF	50-84%	High
UEA-1	70-87%	Low
Fli-1	94%	high
ERG	100%	high

Table 5. Sensitivity and Specificity of Some Markers for Angiosarcoma Diagnosis [2, 46-51]

Marker

Sensitivity

Specificity

ERG: erythroblast transformation specific related gene; UEA-1: Ulex europaeus agglutinin 1.

CD31

77-100%

High

CD34

40-100%

Low

vWF

50-84%

High

UEA-1

70-87%

Low

Fli-1

94%

high

ERG

100%

high

**Table 6.** Markers Used for Immunohistochemical Studies in Reported Cases
Markers	Number of positive cases	Percentage (%)
UEA-1: Ulex europaeus agglutinin 1; ERG: erythroblast transformation specific related gene.
vWF	26	55.3
CD31	25	53.2
CD34	17	36.2
Vimentin	13	27.7
UEA-1	6	12.8
Keratin	4	8.5
Collagen IV	2	4.3
Anti-endothelin-1	1	2.1
WT-1	1	2.1
ERG	1	2.1

Table 6. Markers Used for Immunohistochemical Studies in Reported Cases

Markers

Number of positive cases

Percentage (%)

UEA-1: Ulex europaeus agglutinin 1; ERG: erythroblast transformation specific related gene.

vWF

55.3

CD31

53.2

CD34

36.2

Vimentin

27.7

UEA-1

12.8

Keratin

8.5

Collagen IV

4.3

Anti-endothelin-1

2.1

WT-1

2.1

ERG

2.1

**Table 7.** Markers Useful for Angiosarcoma Differentiation [2, 50, 56-58]
Tumors	Positive stain	Negative stain	Variable stain
CK: cytokeratin; HBME-1: Hector Battifora mesothelial-1; HHV-8 LNA-1: human herpesvirus type 8 latent nuclear antigen-1; EMA: epithelial membrane antigen; Fli-1: Friend leukemia integration 1; SMA: smooth muscle actin; WT-1, Wilms tumor-1.
Angiosarcoma	CD31, vWF, Fli-1, vimentin	S-100, EMA, SMA, HHV-8 LNA-1	CD34, CK, D2-40, Bcl-2
Kapasi sarcoma	HHV-8 LNA-1, D2-40	S-100, EMA, SMA, Bcl-2
Carcinoma	CK, EMA, mucin	CD31, CD34, vWF, Fli-1
Melanoma	S-100, HMB-45, melan-A, vimentin	CD31, CD34, vWF, FLI-1
Malignant mesothelioma	Calretinin, WT-1, CK5/6, HBME-1, vimentin	CD31, CD34, vWF, Fli-1
Epithelioid sarcoma	CK, EMA, vimentin	vWF, Fli-1, S-100	CD31, CD34
Hemangioendothelioma	CD31, CD34, vWF, Fli-1	EMA	CK, SMA
Anaplastic large cell lymphoma	CD30, CD45, pan–T-cell marker	CD31, CD34, vWF, Fli-1

Table 7. Markers Useful for Angiosarcoma Differentiation [2, 50, 56-58]

Tumors

Positive stain

Negative stain

Variable stain

CK: cytokeratin; HBME-1: Hector Battifora mesothelial-1; HHV-8 LNA-1: human herpesvirus type 8 latent nuclear antigen-1; EMA: epithelial membrane antigen; Fli-1: Friend leukemia integration 1; SMA: smooth muscle actin; WT-1, Wilms tumor-1.

Angiosarcoma

CD31, vWF, Fli-1, vimentin

S-100, EMA, SMA, HHV-8 LNA-1

CD34, CK, D2-40, Bcl-2

Kapasi sarcoma

HHV-8 LNA-1, D2-40

S-100, EMA, SMA, Bcl-2

Carcinoma

CK, EMA, mucin

CD31, CD34, vWF, Fli-1

Melanoma

S-100, HMB-45, melan-A, vimentin

CD31, CD34, vWF, FLI-1

Malignant mesothelioma

Calretinin, WT-1, CK5/6, HBME-1, vimentin

CD31, CD34, vWF, Fli-1

Epithelioid sarcoma

CK, EMA, vimentin

vWF, Fli-1, S-100

CD31, CD34

Hemangioendothelioma

CD31, CD34, vWF, Fli-1

EMA

CK, SMA

Anaplastic large cell lymphoma

CD30, CD45, pan–T-cell marker

CD31, CD34, vWF, Fli-1

**Table 8.** Therapeutic Modalities in Reported Cases
Therapeutic modalities	Number of cases	Percentage (%)
Surg: surgery; Chemo: chemotherapy; Rad: radiation therapy; APC: argon plasma coagulation. NA: the other modality not available.
Surg	31	66.0
Surg/Chemo	8	17.0
Surg/Chemo/Rad	2	4.3
Surg/NA	1	2.1
Chemo	1	2.1
Chemo/Rad	1	2.1
APC	1	2.1
Unspecified	2	4.3
Total	47	100.0

Table 8. Therapeutic Modalities in Reported Cases

Therapeutic modalities

Number of cases

Percentage (%)

Surg: surgery; Chemo: chemotherapy; Rad: radiation therapy; APC: argon plasma coagulation. NA: the other modality not available.

Surg

66.0

Surg/Chemo

17.0

Surg/Chemo/Rad

4.3

Surg/NA

2.1

Chemo

2.1

Chemo/Rad

2.1

APC

2.1

Unspecified

4.3

Total

100.0