Small intestinal angiosarcoma on clinical presentation, diagnosis, management and prognosis: A case report and review of the literature

Abstract

Background: Angiosarcoma is a highly malignant soft-tissue sarcoma derived from vascular endothelial cells that mainly occurs in the skin and subcutaneous tissues. Small-intestinal angiosarcomas are rare, and the prognosis is poor.

Case summary: We reported a case of primary multifocal ileal angiosarcoma and analyze previously reported cases to improve our understanding of small intestinal angiosarcoma. Small intestinal angiosarcoma is more common in elderly and male patients. Gastrointestinal bleeding, anemia, abdominal pain, weakness, and weight loss were the common symptoms. CD31, CD34, factor VIII-related antigen, ETS-related gene, friend leukemia integration 1, and von Willebrand factor are valuable immunohistochemical markers for the diagnosis of small-intestinal angiosarcoma. Small-intestinal angiosarcoma most commonly occurs in the jejunum, followed by the ileum and duodenum. Radiation and toxicant exposure are risk factors for angiosarcoma. After a definite diagnosis, the mean and median survival time was 8 mo and 3 mo, respectively. Kaplan-Meier survival analysis showed that age, infiltration depth, chemotherapy, and the number of small intestinal segments invaded by tumor lesions were prognostic factors for small intestinal angiosarcoma. Multivariate Cox regression analysis showed that chemotherapy and surgery significantly improved patient prognosis.

Conclusion: Angiosarcoma should be considered for unexplained melena and abdominal pain, especially in older men and patients with a history of radiation exposure. Prompt treatment, including surgery and adjuvant chemotherapy, is essential to prolonging patient survival.

Keywords: Angiosarcoma; Case report; Diagnosis; Pathological features; Prognosis; Small intestine.

Figure 1

Computed tomography showed segmental thickening of the small intestine (white arrow), with lesion enhancement in the arterial phase. A: Arterial phase; B: Venous phase; C: Balanced phase; D: Coronal plane; E: Adrenal masses.

Figure 2

Magnetic resonance imaging showed local abnormally enhanced nodules (white arrow) at the L1/L2 Level in the cauda equina. A: T1 phase; B: T2 phase.

Figure 3

Electronic double-balloon enteroscopy. A-C: Electronic double-balloon enteroscopy showed continuous periannulus ulcers 2.4-2.5 m above the ileocecal valve, covered with mucous moss.

Figure 4

During the operation, multiple grey-red ulcerative tumors were observed in the ileum mucosa covered with moss. In addition, an 8 cm × 6 cm ulcerative mass (white arrow) resulted in intestinal obstruction.

Figure 5

Pathologic findings. A and B: Microscopically, spindle cell infiltration was observed with round or spindle-shaped nuclei. In some areas, tumor cells formed vascular channels with red blood cells in the middle (× 100).

Figure 6

Age distribution of male and female patients.

Figure 7

Kaplan-Meier survival analysis. A: Sex; B: Age; C: Risk factor; D: Acute abdomen; E: Tumor origin; F: Tumor size; G: Infiltrating depth; H: Number of tumor lesions; I: Intestinal segments involvement; J: Distant metastases; K: Surgery; L: Chemotherapy.