Sarcomatoid renal cell carcinoma: biology, natural history and management.
Kyle A BlumSounak GuptaSatish K TickooTimothy A ChanPaul RussoRobert J MotzerJose A KaramAbraham Ari HakimiPublished in: Nature reviews. Urology (2020)
Sarcomatoid dedifferentiation is an uncommon feature that can occur in most histological subtypes of renal cell carcinomas (RCCs) and carries a decidedly poor prognosis. Historically, conventional treatments for sarcomatoid RCCs (sRCCs) have shown little efficacy, and median survival is commonly 6-13 months. Despite being first described in 1968, the mechanisms driving sarcomatoid dedifferentiation remain poorly understood, and information and treatment options available to physicians and patients are limited. When diagnosed at an early stage, surgical intervention remains the treatment of choice. However, preoperative identification through routine imaging or biopsy is unreliable and most patients present with advanced disease and systemic symptoms. For these patients, the role of cytoreductive nephrectomy is disputed. The expansion of immunotherapies approved for RCCs has generated a search for biomarkers that might be indicative of treatment response in sRCCs, although a proven effective systemic agent remains elusive. PDL1 expression is increased in sarcomatoid dedifferentiated renal tumours, which suggests that patients with sRCCs could benefit from PD1 and/or PDL1 immune checkpoint blockade therapy. Treatment outcomes for sarcomatoid tumours have remained relatively consistent compared with other RCCs, but further investigation of the tumour-immune cell microenvironment might yield insights into further therapeutic possibilities.
Keyphrases
- poor prognosis
- end stage renal disease
- ejection fraction
- newly diagnosed
- early stage
- chronic kidney disease
- prognostic factors
- peritoneal dialysis
- stem cells
- long non coding rna
- healthcare
- radiation therapy
- mesenchymal stem cells
- patients undergoing
- high resolution
- bone marrow
- photodynamic therapy
- free survival
- cell therapy
- combination therapy
- minimally invasive
- ultrasound guided
- drug induced