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.

Jejunal angiosarcoma: a rare cause of obscure gastrointestinal bleeding with successful resection after enteroscopic localization

“…The aetiology of small intestinal angiosarcoma is not clear; however, vinyl chloride and radiation exposure have been implicated in disease development . The clinical manifestations of small intestinal angiosarcoma are non‐specific and include abdominal pain (41.3%), anaemia (34.8%) and gastrointestinal bleeding (26.1%) . There is a slight male predominance (male to female ratio of 1.6:1) reported in the literature with a median age of 68.5 years at diagnosis.…”

“…It occurs mainly in the skin, soft tissues and breast. The gastrointestinal tract is rarely involved with less than 50 cases of primary angiosarcoma of the small intestine reported between 1970 and 2017 . The aetiology of small intestinal angiosarcoma is not clear; however, vinyl chloride and radiation exposure have been implicated in disease development .…”

“…The gastrointestinal tract is rarely involved with less than 50 cases of primary angiosarcoma of the small intestine reported between 1970 and 2017 . The aetiology of small intestinal angiosarcoma is not clear; however, vinyl chloride and radiation exposure have been implicated in disease development . The clinical manifestations of small intestinal angiosarcoma are non‐specific and include abdominal pain (41.3%), anaemia (34.8%) and gastrointestinal bleeding (26.1%) .…”

Primary small intestinal epithelioid angiosarcoma: a potential diagnostic pitfall due to abnormal expression of cytokeratin

“…Small intestinal AS is very rare. To the best of our knowledge,only 66 cases of small intestinal angiosarcoma including primary or metastatic were reported in the current literature,most had only been found in scattered case reports and small series [3][4][5][6][7][8]. Pathologically AS can be classified into well-differentiated, poorly differentiated,and epithelioid AS.…”

“…Therefore, unless early finding and appropriate treatment are given, the prognosis is usually poor. At present, the etiology of AS is not clear [2][3][4]. Most pathologists lack knowledge of AS and are easily misdiagnosed as poorly differentiated adenocarcinoma, especially when tumor cells express epithelial markers abnormally in small biopsy specimens.…”