Imaging appearance of renal epithelioid angiomyolipoma

Rationale: Epithelioid angiomyolipoma (EAML) is an extremely rare disease. It commonly occurs in middle-aged females and mainly involves the kidney. Histological and immunohistochemical examination play important roles in differentiating EAML from renal cell carcinoma (RCC) and poor-fat angiomyolipoma (AML). Patient concerns: Here, We report the imaging phenotype, as well as the pathological findings of a case of EAML in a 39-year-old female. Diagnoses: Preoperative noncontrast computed tomography (CT) scan revealed a 6.0 × 5.2 × 7.0 cm soft tissue mass with necrosis, located in the left kidney. On contrast-enhanced CT images, aprogressive enhancement pattern was observed. CT angiography did not show any enlarged vessels or vascular malformation. Abdominal MRI showed a well-circumscribed solid mass with a heterogeneous signal on T1-weighted and T2-weighted images. Ultrasonography of the abdomen demonstrated a hypoechoic mass with abundant blood flow. This patient underwent radical nephrectomy. The pathologic diagnosis was EAML. Interventions: This patient underwent operative resection of the tumor. The resection margins were negative for the neoplastic proliferation and no distant metastases were found. The patient did not receive advanced radiotherapy or chemotherapy. Outcomes: Four months after surgery, the follow-up CT scan did not reveal any local recurrence or distant metastases. Lessons: This case adds to the experience with EAML by summarizing its imaging characteristics as well as reviewing the literature. Additionally, we described the state-of-the-art management of the management of this rare tumor.