Primary Small Intestinal Angiosarcoma: Epidemiology, Diagnosis and Treatment

Abstract

Angiosarcoma is an aggressive mesenchymal sarcoma of endothelial cell origin with high mortality. Its occurrence in the small intestine is exceedingly low. In addition to the rarity of small intestine angiosarcoma, the nonspecific early clinical symptoms obscure the suspicion of such tumors and thereby delay the diagnosis. In a hope to improve the knowledge of this rare but fatal neoplasm, we report one case of angiosarcoma of duodenum and jejunum in a 73-year-old man. Furthermore, we summarize and analyze the common clinical features, tumor markers, treatment, and survival of previous reported cases of this malignancy. Small bowel angiosarcoma occurs more often in men than women (1.6:1). The median age at diagnosis is 68.5 years. The overall median survival time is 150 days; the median survival time in female (300 days) is longer than that of male patients (120 days). Von Willebrand factor (vWF), CD31, CD34, vimentin, and Ulex europaeus agglutinin 1 appear to be the most useful markers for the diagnosis. The majority of the patients underwent surgical resection alone or surgery with subsequent chemotherapy. The patients treated with surgery plus chemotherapy survive longer than those underwent surgical resection only (median 420 days, n = 7 vs. 96.5 days, n = 26, respectively; P = 0.0275). Further studies of more cases are needed for a better understanding of this rare entity, as well as the development of effective strategies for prevention, early diagnosis, and treatment.

Keywords: Angiosarcoma; Diagnosis; Small intestine; Survival; 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.