Epithelioid angiosarcoma of the small intestine presenting with intractable gastrointestinal bleeding: An uncommon tumor with diagnostic challenge and poor prognosis

Abstract

Introduction: Angiosarcoma of the gastrointestinal tract is a rare, aggressive malignant neoplasm of vascular origin. Commonly, this tumor occurs due to metastases from other sites like the head and neck region and the soft tissue. When occurring as a primary tumor of the intestines, this entity poses a diagnostic dilemma due to its non-specific clinical presentation, coupled to its infiltrative features that may occur without an obvious lesion amenable to routine gastrointestinal interventions. The findings from an autopsy and surgical case with a brief review of its diagnostic features and relevant literature are discussed.

Methods: We present a 73-year-old male with a history of a recent onset of gastrointestinal bleeding who was referred to our facility for persisting bleeding. He underwent endoscopy without an identifiable source of bleeding and subsequently received a colectomy at our facility, with repeated blood transfusions for persisting gastrointestinal bleeding. He was eventually transitioned to hospice and died shortly after presentation. A complete autopsy was performed.

Results: There was anasarca with hemoperitoneum and intestinal intraluminal blood. Present in the small intestinal mucosa were three hemorrhagic nodules. Histologically, the nodules demonstrated medium to large epithelioid vasoformative neoplastic cells infiltrating through the wall of the small intestine and extending to the omentum. The findings from the gross and histology examination, as well as the immunohistochemical stains were consistent with an epithelioid angiosarcoma.

Conclusion: Primary epithelioid angiosarcoma of the intestine is an uncommon aggressive tumor that poses a diagnostic challenge. Although rare, this diagnosis needs to be considered in patients with intractable gastrointestinal bleeding. Due to its morphologic overlap with other benign and malignant neoplasms, the use of ancillary testing during histologic examination is key in arriving at an accurate diagnosis.