Abstract
Angiomyolipomas (AMLs) are barely benign mesenchymal tumors that usually occur in the kidneys and may be associated with tuberous sclerosis complex (TSC). Extrarenal AMLs are markedly rare and infrequently observed in the duodenum. In the present case report, a 22-year-old female patient with duodenal AMLs presenting multiple systemic vascular malformations and aneurysms is described. The patient had a medical history of aneurysm rupture of the right subclavian artery and no other manifestation of TSC. Surgical intervention was performed. Following complete tumor resection, the patient declined to be treated further for vascular lesions. Pathological and immunohistochemical examination confirmed the diagnosis of duodenal AMLs. No tumor recurrence or progression of the vascular lesions was observed within 24 months of follow-up. This case report demonstrates the scarcity of duodenal AMLs with multiple systemic vascular malformations and aneurysms, which may be associated with novel gene mutations or TSC; however, further verification by gene sequencing is required.