Bilateral giant renal angiomyolipomas associated with tuberous sclerosis represent a challenge in their treatment, especially if nephrectomy is used to treat one side.
Mohamed M. Salama, MD, Hebatallah G. Mohamed Mahmoud, MD, Mohamed Salama, MD, Amr Abdel Meguid Attia, MD; National Cancer Institute, Cairo University
OBJECTIVE: We report on a patient with bilateral giant renal angiomyolipomas, bilateral pulmonary leiomyomas, and bilateral periventricular brain calcifications associated with tuberous sclerosis, who presented to the surgical department of the National Cancer Institute, Cairo University.
We discuss the management of the case and the challenges in the surgical treatment of the patient, as well as the intraoperative and early postoperative complications and follow-up.
CASE REPORT: A 28-year-old female diagnosed with tuberous sclerosis presented clinically with a huge left abdominal mass. The patient was referred to our tertiary center after assessment of a CT-guided biopsy sample from the mass, which had a pathological diagnosis of renal angiomyolipoma. The CT scan showed left giant renal angiomyolipoma; the left side measured 22 × 17 × 15 cm, while the right side was the seat of small fatty masses not clearly evident on CT. Left nephrectomy was done, and the right kidney was explored after removal of the left kidney, which was masking the right angiomyolipoma. A right-sided hematoma resulted from manipulation intraoperatively, and a decision was made to monitor that side without surgical interference. The patient was followed up postoperatively every 3 months. Renal function in the postoperative period was preserved, and the follow-up with magnetic resonance imaging 9 months later showed a stationary course of the right-sided tumor, with good general condition of the patient.
CONCLUSION: Bilateral giant renal angiomyolipomas associated with tuberous sclerosis represent a challenge in their treatment, especially if nephrectomy is used to treat one side. Every effort should be made to follow up with the contralateral side if it is asymptomatic, to monitor its rate of growth.
Proceedings of the 98th Annual Meeting of the American Radium Society - americanradiumsociety.org