Intestinal Sarcoma in Two Simultaneous Intestinal Segments: Case Report

Case Presentation A 71-year-old male patient presented with abdominal pain, hematochezia, and a 15 kg weight loss over 4 months. He was referred to a tertiary hospital in Greater São Paulo, where enterotomography revealed irregular segmental parietal thickening with a neoplastic appearance in the proximal jejunal loop, beginning about 10 cm after the Treitz angle, extending 10 cm, and signs of fistulization extending into another jejunal loop, approximately 80 cm from the Treitz angle, with 4.0 cm of involvement. There was also thickening in the ileal loop in the right hypogastrium, measuring 4.0 cm, without mesenteric lymphadenopathy. Following staging, the patient underwent exploratory laparotomy requiring 3 enterectomies and the creation of 3 enteroenterostomies: the first 4 cm from the Treitz angle, the second 70 cm from the Treitz angle, and the third at the site of the ileal tumor resection. The patient had a good postoperative recovery and was discharged on the 6th postoperative day. Histopathological examination revealed poorly differentiated malignant neoplasia with ulceration in both resected lesions, and immunohistochemistry (IHC) was consistent with angiosarcoma due to the following positive markers: CD-34, CD-31, ERG, Factor VIII, and FL1.

Discussion Sarcomas are a rare and heterogeneous group of malignant mesenchymal tumors that account for less than 1% of all adult malignant neoplasms. The histopathological spectrum of sarcomas is broad, presumably because the embryonic mesenchymal cells from which they arise have the capacity to differentiate into skeletal and smooth muscle, adipose tissue, and fibrous tissue, among others. The reported incidence is between 10 and 15 cases per million inhabitants per year. Symptoms may include weight loss, intestinal subocclusion, and hematochezia, if the tumor is intestinal. Treatment is surgical, and the diagnosis of sarcoma is retrospective, as it is made through IHC, which detects tumor cells positive for vascular markers such as CD31 and Factor VIII.

Conclusion The incidence of intestinal sarcoma is rare, and its diagnosis is made through immunohistochemical results after resection. Therefore, treatment, when possible, should be surgical, similar to other intestinal neoplasms. It is important to pay attention to the pathological results and request immunohistochemistry for differentiation.

No conflict of interest has been declared by the author(s).

Publication History

Article published online:
25 April 2025