Medical Case Reports

Significant Response to Sunitinib in an Adult with Advanced clear cell Sarcoma of the Kidney: Report of a Rare Case and Literature Review

Clear Cell Sarcomas of the Kidney (CCSK) is an exceedingly rare neoplasm that predominantly affects the pediatric age group. Primary localized disease is treated with radical nephrectomy and adjuvant chemotherapy and/ or radiotherapy. Optimal therapy at relapse is controversial. We report a 22-year-old male who presented with abdominal pain following a prior radical right nephrectomy for a kidney tumor. Imaging studies showed local recurrence and peritoneal metastasis. Pathology review confirmed a diagnosis of CCSK. Three months after starting sunitinib, Computed Axial Tomography (CT) scan of the chest, abdomen, and pelvis showed significant regression of his diseased consistent with partial response. Clear cell sarcoma of the kidney is extremely rare in adults. To our knowledge, this is the first report of advanced CCSK in an adult responding to sunitinib. Clinical trials are needed to better define the orle of systemic treatments in patients with relapsed CCSK.

Author(s): Samer Salah, Jakub Khzouz, Mais Zmaily, Omar Jaber, Fawzi Abuhijla and Ramiz Abu Hijlih

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