Clinical challenges and management of primary renal epithelioid angiomyolipoma of duplex kidney with paraneoplastic syndrome

Abstract

Giant renal epithelioid angiomyolipoma of a duplex kidney has rarely been reported, especially in patients with paraneoplastic syndrome. The present report describes a 33-year-old man of Miao nationality who presented with a 6-month history of intermittent dull pain in the left upper abdomen that occurred after eating. Ultrasonography, intravenous pyelography, and computed tomography revealed a mass lesion localized in the left kidney and connected to the left renal artery. Radical nephrectomy was successfully performed, and the postoperative histopathological examination verified the lesion as epithelioid angiomyolipoma. Inpatient treatment for paraneoplastic syndrome was also performed. The present case is discussed in the context of the patient’s clinical presentation and imaging findings, drawing attention to the challenges and management of this condition to assist clinicians in practice.

Advancements in the diagnosis and treatment of renal epithelioid angiomyolipoma: A narrative review

“…Some patients, depending on the location and size of the tumors, may also have clinical symptoms such as fatigue, fever, low back pain, abdominal pain, hematuria, bleeding, dysuria, and renal dysfunction. Recently, Benincasa et al 4 reported a case of spontaneous renal rupture caused by PEComa, and although this is relatively rare in clinical practice, it should receive attention as the tumor is also known to cause paraneoplastic syndromes such as a high erythrocyte sedimentation rate, elevated platelet count, and hormonal and immune system abnormalities 5 …”

“…reported a case of spontaneous renal rupture caused by PEComa, and although this is relatively rare in clinical practice, it should receive attention as the tumor is also known to cause paraneoplastic syndromes such as a high erythrocyte sedimentation rate, elevated platelet count, and hormonal and immune system abnormalities. 5 3 | ETIOLOGY AND PATHOGENESIS OF RENAL EPITHELIOID ANGIOMYOLIPOMA Tuberous sclerosis complex (TSC) is an autosomal dominant disorder characterized by hamartomas of the brain, heart, skin, lungs, and kidneys. 6 Studies have shown that a close relationship between renal AML and TSC exists.…”