Resection and Re-resection for a Clear Cell Renal Carcinoma with Sarcomatoid Features in a Patient with Birt-Hogg-Dubé Syndrome

Abstract

Background: Birt-Hogg-Dubé syndrome (BHDS) is a rare monogenic autosomal dominant disorder which is, among other disease manifestations, associated with an increased life-time risk of developing renal cell carcinoma (RCC). Sarcomatoid RCC (sRCC) is a subtype of RCC associated with poor prognosis, high recurrence rates, and a significant resistance to a variety of systemic treatments. Case Report: Here, it is reported that the first case of a sRCC with local recurrence in a 62-year-old female patient with BHDS. This patient was successfully treated with a partial robotic nephrectomy and a re-resection of the tumor recurrence resulting in good short-term oncological outcomes. Conclusions: Robotic partial nephrectomy for sRCC, and robotic re-resection for locally recurrent sRCC in BHDS patients is a feasible treatment strategy associated with good short-term oncological outcomes. Due to the high risk of local and distant recurrences in sRCC cases, a lifelong follow-up with regular imaging is recommended.