A 21-year-old female presented with a retroperitoneal mass in the right side and a centrally located renal mass in the left kidney with an aberrant vascular anatomy of a double inferior vena cava. The 2 tumors were radically excised by a transperitoneal laparoscopic approach in one session. Histopathologic examination revealed the right retroperitoneal lesion to be a schwannoma and the renal mass to be a cluster of monotypic neoplasms with perivascular epithelioid cell differentiation (PEComas). The latter is a rare tumor.
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