Long-term response of metastatic hereditary leiomyomatosis and renal cell carcinoma syndrome associated renal cell carcinoma to bevacizumab plus erlotinib after temsirolimus and axitinib treatment failures.

Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is a rare hereditary kidney cancer syndrome in which affected individuals are at risk of skin and uterine leiomyomatosis and kidney cancer. HLRCC-associated kidney cancer is a lethal disease with a highly aggressive behavior, and there is no standard treatment option for metastatic disease.

Here, we report a 29-year-old patient with a locally advanced HLRCC-assiciated RCC. He was administrated temsirolimus initially, then underwent surgical removal of kidney, retroperitoneal lymph nodes, inferior vena cava and tumor thrombi. Unfortunately, multiple liver metastases were confirmed 1 month after surgery, so axitinib was given but failed immediately. We tried bevacizumab plus erlotinib, which achieved long-term good response lasting more than 18 months. He is alive with disease and maintains bevacizumab plus erlotinib treatment.

The promising results obtained in this patient suggest that combined bevacizumab plus erlotinib may offer a valid treatment option for advanced HLRCC-associated kidney cancer, even after failures of mTOR inhibitor and/or VEGFR TKI based therapies.

BMC urology. 2019 Jun 10*** epublish ***

Inkeun Park, Young Sup Shim, Heounjeong Go, Bum Sik Hong, Jae Lyun Lee

Division of Medical Oncology, Department of Internal Medicine, Gachon University Gil Medical Center, 1198 Guwol-dong, Namdong-gu, Incheon, 21565, Republic of Korea. ., Department of Radiology, Gachon University Gil Medical Center, Incheon, Republic of Korea., Department of Pathology, Ulsan University Asan Medical Center, Seoul, Republic of Korea., Department of Urology, Ulsan University Asan Medical Center, Seoul, Republic of Korea., Department of Oncology, Ulsan University Asan Medical Center, Seoul, Republic of Korea.

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