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"Hereditary Leiomyomatosis and Renal Cell Cancer  HLRCC  is an autosomal dominant syndrome caused by heterozygous pathogenic germline variants in the fumarate hydratase   FH   gene  It is characterized by cutaneous and uterine leiomyomas and an increased risk of developing renal cell carcinoma  RCC   which is usually adult-onset  HLRCC-related RCC tends to be aggressive and can metastasize even when the primary tumor is small  Data on children and adolescents are scarce  Herein  we report two patients from unrelated Dutch families  with HLRCC-related RCC at the ages of 15 and 18  years  and a third patient with an FH mutation and complex renal cysts at the age of 13  Both RCC   s were localized and successfully resected  and careful MRI surveillance was initiated to monitor the renal cysts  One of the patients with RCC subsequently developed an ovarian Leydig cell tumor  A review of the literature identified 10 previously reported cases of HLRCC-related RCC in patients aged younger than 20  years  five of them presenting with metastatic disease  These data emphasize the importance of recognizing HLRCC in young patients to enable early detection of RCC  albeit rare  They support the recommendations from the 2014 consensus guideline  in which genetic testing for FH mutations  and renal MRI surveillance  is advised for HLRCC family members from the age of 8   10  years onwards  Electronic supplementary material The online version of this article  10 1007 s10689-019-00155-3  contains supplementary material  which is available to authorized users "
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