A Case of Metastatic Fumarate Hydratase-Deficient–like Renal Cell Carcinoma Successfully Managed by Ipilimumab plus Nivolumab

Sekito, Takanori; Takamoto, Atsushi; Kobayashi, Yasuyuki; Mitsui, Masao; Watari, Shogo; Kubota, Risa; Sadahira, Takuya; Iwata, Takehiro; Nishimura, Shingo; Edamura, Kohei; Sako, Tomoko; Araki, Motoo; Watanabe, Masami; Watanabe, Toyohiko; Shibata, Rei; Ennishi, Daisuke; Nasu, Yasutomo

ID	62237
JaLCDOI	10.18926/AMO/62237
FullText URL	75_3_397.pdf 5.98 MB
Author	Sekito, Takanori Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Takamoto, Atsushi Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Kobayashi, Yasuyuki Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Mitsui, Masao Department of Urology, Kurashiki Medical Center Watari, Shogo Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Kubota, Risa Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Sadahira, Takuya Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Iwata, Takehiro Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Nishimura, Shingo Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Edamura, Kohei Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Sako, Tomoko Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Araki, Motoo Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Watanabe, Masami Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Watanabe, Toyohiko Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences Shibata, Rei Department of Pathology, Okayama University Hospital Ennishi, Daisuke Department of Hematology and Oncology, Okayama University Hospital Nasu, Yasutomo Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences
Abstract	We report a 62-year-old male with metastatic fumarate hydratase-deficient renal cell carcinoma (FH-deficient RCC) without fumarate hydratase (FH) mutation (FH-deficient–like RCC). The International Metastatic RCC Database Consortium risk score was intermediate, and immunotherapy with nivolumab and ipilimumab (Ipi/ Nivo) was initiated. Four cycles of Ipi/Nivo and 5 cycles of nivolumab resulted in a complete response of the metastases. Hypophysitis occurred as an immune-related adverse event after four cycles of Ipi/Nivo. The prognosis of patients with FH-deficient RCC is generally poor. Few reports of FH-deficient RCC successfully treated with Ipi/Nivo have been published. Ipi/Nivo can be effective for treating FH-deficient RCC.
Keywords	fumarate hydratase fumarate hydratase-deficient renal cell carcinoma renal cell carcinoma ipilimumab nivolumab
Amo Type	Case Report
Publication Title	Acta Medica Okayama
Published Date	2021-06
Volume	volume75
Issue	issue3
Publisher	Okayama University Medical School
Start Page	397
End Page	402
ISSN	0386-300X
NCID	AA00508441
Content Type	Journal Article
language	English
Copyright Holders	CopyrightⒸ 2021 by Okayama University Medical School
File Version	publisher
Refereed	True
PubMed ID	34176946
Web of Science KeyUT	000667147700017
NAID	120007089822