A giant renal angiomyolipoma (AML) in a patient with septo-optic dysplasia (SOD)

Abstract

Angiomyolipoma (AML) is a rare benign renal tumor occurring in about 0.3 to 3% of the general population. Most frequently it takes the form of small single tumors occurring sporadically or accompanying tuberous sclerosis (Bourneville-Pringle disease). In some cases the tumor may reach a very large size and be a cause of various serious complications. This case description concerns a 26-year-old female patient, suffering from hypopituitarism, hypothyroidism and binocular blindness during the course of septo-optic dysplasia, in whom a giant, left renal AML was diagnosed and treated surgically. According to the authors’ knowledge this was the first reported case of a huge size AML in a patient with de Morsier syndrome.

Successful robot-assisted partial nephrectomy for giant renal hilum angiomyolipoma through the retroperitoneal approach: A case report

“…Considering the large volume and deep location of the present mass, a robot-assisted LPN was planned to achieve complete removal of the tumor and adequate preservation of renal function. In terms of the surgical approaches for giant renal AMLs, the TP approach is more common as it provides a larger working space, better orientation and wider angles to reach tumors[ 10 , 12 , 13 ]. However, potential complications of the TP approach relate to the bowels caused by bowel mobilization which would prolong recovery time[ 3 ].…”

Familial genetic tuberous sclerosis complex associated with bilateral giant renal angiomyolipoma: A case report

“…TSC-associated renal manifestations include cysts, angiomyolipoma, epithelioid tumor, renal cell carcinoma and eosinophilic cell tumor, and may coexist or exist independently ( 30 – 33 ). The incidence of TSC-associated RAML is 20% of RAML and typically develops bilaterally ( 34 ). Imaging techniques enable TSC RAML to be distinguished from the classical type.…”