Metastatic Bilateral Clear Cell Renal Carcinoma in Tuberous Sclerosis Complex: A Case Report

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Résumé

Malignant tumors of the kidney, particularly clear cell renal carcinoma (CCRC), are fearful entities due to their aggressiveness
and metastatic potential. They occasionally arise in predisposing hereditary syndromes such as Tuberous Sclerosis Complex
(TSC). Herein, we present a case of A 33-year-old nulligravida woman presented with intermittent right-sided low back pain
evolving for approximately 15 years. Imaging, including contrast-enhanced abdominal CT scan, revealed the presence of
bilateral poorly defined heterogeneous solid-cystic masses with enhancement after contrast injection suggestive of bilateral renal
tumors. Bilateral renal biopsy was performed, and histological examination revealed bilateral clear cell renal carcinoma (CCRC)
with International Society of Urological Pathology (ISUP) nuclear grades of 2. Further staging with thoracic CT scan revealed
bilateral pulmonary lymphangioleiomyomatosis (Figure 5) associated with multiple bone metastases. The tumor was classified
as intermediate risk according to Heng prognostic criteria, and the case was discussed in a multidisciplinary tumor board
meeting. The specific treatment of metastatic CCRC in the setting of TSC has not been reported in the literature to our
knowledge. Subsequently, the patient was referred to medical oncology for initiation of tyrosine kinase inhibitor therapy. We
reviewed the patient at 6 months, 12 months and 18 months and the evolution was satisfactory, she expressed no complaints and
weighed 58kg.

Mots-clés

Phakomatosis, Renal Cell Carcinoma, Tuberous Sclerosis Complex, Kidney Biopsy