Risk of cancer in patients with polycystic kidney disease

Violo, Leano; Francesco, M. De; Schoenholzer, Carlo

doi:10.1016/s1470-2045(16)30526-5

Cited by 2 publications

(2 citation statements)

References 5 publications

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“…3 There is more than twofold higher risk of developing renal cell carcinoma (RCC) in patients with ADPKD in large national databases over a 14year follow-up period. 4 Surgical specimens of ADPKD patients on dialysis showed 5 to 12% harboring RCC. 5,6 A possible direct association of ADPKD with RCC is, however, controversial, and is more often deemed a coincidence except in those with end-stage renal failure where genetic mutations may trigger malignant transformation.…”

Section: Introductionmentioning

confidence: 99%

Renal Cell Carcinoma in the Background of Autosomal Dominant Polycystic Kidney Disease: Report of Two Cases and Review of Literature

Vias

Goyal

Madan

et al. 2023

Indian J Med Paediatr Oncol

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Patients with autosomal dominant polycystic kidney disease (ADPKD), especially those with renal failure, carry a higher risk of developing renal cell carcinoma (RCC) compared to the general population. Genetic mutations associated with ADPKD are known but a direct link associated with RCC is still controversial. We discuss the clinical course of two such patients. The first patient was diagnosed with ADPKD at the age of 10 years with an unreported tubulocystic RCC focus on his renal biopsy that was picked up on review 16 years later when he presented with vertebral metastases determined to have originated from the RCC. He was doing well on multikinase inhibitors till 4 years of diagnosis with metastatic disease when he succumbed to progressive disease after 3 lines of systemic therapy. The second patient was diagnosed with ADPKD in middle age and papillary RCC 3 years later. Within 3 months of cancer diagnosis, there was progression to metastatic disease and rapid decline despite systemic therapies. We surmise that the diagnosis of RCC may be missed in ADPKD till the advanced stages. Patients with ADPKD should be monitored regularly with imaging and biopsy if needed. Histology may be varied but once diagnosed, systemic therapies may help disease control.

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Section: Introductionmentioning

confidence: 99%

Renal Cell Carcinoma in the Background of Autosomal Dominant Polycystic Kidney Disease: Report of Two Cases and Review of Literature

Vias

Goyal

Madan

et al. 2023

Indian J Med Paediatr Oncol

View full text Add to dashboard Cite

show abstract

“…The prevalence of renal cell carcinoma (RCC) in patients with ADPKD is known to be high. [ 3 ] Whether this is due to chronic dialysis or the underlying disease is still speculative, but attending physicians should be aware of the malignant potential of ADPKD, especially in patients receiving concomitant dialysis. [ 4 ] The most common histological types of kidney tumors are clear cell RCC (ccRCC, 75%), type I papillary RCC (5%), type II papillary RCC (10%), and chromophobe RCC and oncocytoma (10%).…”

Section: Introductionmentioning

confidence: 99%