Primary angiosarcoma of the small intestine with metastasis to the liver: a case report and review of the literature

Abstract

Angiosarcoma is a rare disease with a poor prognosis; significantly, patients with intestinal angiosarcomas who survive over 1 year after diagnosis are extraordinarily rare. This article describes the case of a 33-year-old gentleman who presented with abdominal pain of 4 months duration, which had increased in severity 2 weeks prior to presentation. After a complicated diagnostic and therapeutic process, the diagnosis of primary angiosarcoma of the small intestine with metastasis to the liver was made by pathological and immunohistochemical examinations. We reviewed previous cases of angiosarcoma described in the English literature to determine their risk factors, diagnosis and treatment, and we found that angiosarcoma is extremely rare, especially in the small intestine. To the best of our knowledge, this may be the youngest case of primary angiosarcoma of the small intestine with metastasis to the liver reported in the English literature.

Primary Angiosarcoma of Small Intestine Presenting With Intestinal Perforation: A Case Report

“…These tumors have predilection for skin and superficial soft tissue, breast, bone, liver; they are rarely seen in deep soft tissue [3]. They are associated with chronic lymphedema, previous radiotherapy, and arteriovenous fistulas in renal transplant patients [1,4]. In our case report, we present a 78-year-old woman presented with acute abdomen and intestinal perforation.…”

“…In the case report and review of the literature about primary angiosarcoma of the small intestine made by Qingqiang et al, they reported that 27 articles were found about angiosarcomas and only 21 of them were primary small intestinal angiosarcoma cases [1].…”

“…Angiosarcoma may be similar to a gastrointestinal stromal tumor, leiomyosarcoma, a metastatic melanoma, a lymphoma, a poorly differentiated carcinoma, neuroendocrine tumor, Crohnsdisease, mesothelioma [1,2,5,9]. Microscopically anastomosing, delicate vascular channels lined by atypical endothelial cells mixed with solid sheets of spindled, epithelioid or undifferentiated cells are seen [2].…”

“…The pathogenesis of intestinal angiosarcoma is unclear; several etiologic factors have been suggested such as previous radiation, chronic lymphedema, exogenous toxins like thorotrast, vinyl chloride and arsenic, long-term peritoneal dialysis, intra-abdominal foreign body, visceral metastasis from Kaposi's sarcoma and familial syndromes such as neurofibromatosis Type 1, mutated BRCA1 and BRCA2, Mafucci syndrome and Klippel-Trenaunay syndrome [1,3,4]. In the case report and review of the literature about primary angiosarcoma of the small intestine made by Qingqiang et al, they reported that 27 articles were found about angiosarcomas and only 21 of them were primary small intestinal angiosarcoma cases [1].…”

“…Primary gastrointestinal angiosarcomas are very rare and angiosarcoma of small intestine is even rarer, with poor prognosis [1]. Angiosarcomas which account for only 1% to 2% of all soft tissue sarcomas are rare malignant tumors of endothelial origin [2].…”