Giant abdominopelvic epithelioid angiomyolipoma associated with tuberous sclerosis: Report of a case

Tuberous sclerosis is a hereditary autosomaldominant disease characterized by hamartomas that can develop in any organ. We report herein the case of a 34-year-old female with tuberous sclerosis and a huge abdominopelvic mass that started growing quickly 2 years after its diagnosis. The patient had u ndergone several previous operations for hydrocephalus and cerebral tubers, and a nephrectomy for right renal angiomyolipoma. On admission, she was in poor general health with renal failure, severe anemia, and weight loss. A laparotomy revealed that the tumor occupied the pelvis, the lower and part of the upper abdomen, and was hypervascularized, with an extremely irregula r surface covered in nodules, vegetations, and areas of hemorrhagic necrosi s. The development of the mass and the impossibility of recognizing the int ernal genital organs led us to assume that the formation had originated fro m these. Frozen-section examination indicated an undifferentiated tumor tha t had not been completely resected, Her postoperative course was complicate d by bronchopneumonia and progressive renal failure. The patient died 10 da ys after surgery due to cardiorespiratory failure. A histological diagnosis of epithelioid angiomyolipoma was confirmed. Although it is presently impo ssible tb determine whether angiomyolipoma with predominant epithelioid cel ls is more aggressive than typical angiomyolipoma, it definitively demonstr ated local aggressive behavior in this patient.