AUA 2016: Spectrum of radiological findings of hereditary leiomyomatosis and renal cell cancer (HLRCC) - Session Highlights

San Diego, CA USA ( Individuals with hereditary leiomymatosis (smooth muscle tumors) have a 15% lifetime risk of developing renal cell carcinoma, and thus are of special interest in the urological field. Small aggressive papillary carcinoma type II tumors with an increased metastatic potential are the typical tumors classified under hereditary leiomyomatosis and renal cell cancer (HLRCC).

In this study, the authors sought to classify the heterogeneous imaging nature of HLRCC in order to improve screening and diagnosis.

HLRCC lesions from a total of 30 patients were evaluated with CT and/or MR for cystic, enhancement, homogeneity, heterogeneity, nodules/septations, margin definition, and maximal axial diameter. On CT three distinct categories of lesions were classified: 53% of lesions were found to be heterogeneous cystic, 35% were found to be heterogeneous solid, and 12% were found to be homogenous solid. On MRI, the homogenous solid lesions were found to be small (2.4cm), appear hypo-intense on T2 imaging and non-hemorraghic. Heterogenous cyst and heterogeneous solid lesions tended to be larger (4.5cm and 6.7cm, respectively), appear hyper-intense on T2, and be hemorraghic. All three lesions were associated with metastasis and with a restricted diffusion feature.

Although highly varied in size and appearance, HLRCC manifests as heterogenous lesions with cystic and solid components. The authors stress the importance of a thorough family history for identification possible ties to HLRCC since 80% of patients will present with cutaneous or uterine leiomymatosis. In addition, continuous careful screening of even small lesions to monitor for changes in appearance in light of the high metastatic potential of HLRCC.


Presented By: Jana Lovell, MD

Written By: Blanca Morales; Department of Urology, University of California, Irvine at the 2016 AUA Annual Meeting - May 6 - 10, 2016 – San Diego, California, USA