Bilateral papillary renal cell carcinoma and angiomyolipoma in the patients with autosomal dominant polycystic kidney disease: of two cases and literature review

Konosu-Fukaya, Sachiko; Nakamura, Yasuhiro; Fujishima, Fumiyoshi; Kasajima, Atsuko; Takahashi, Yayoi; Joh, Kensuke; Ikeda, Yoshihiro; Ioritani, Naomasa; Watanabe, Mika; Sasano, Hironobu

doi:10.5114/pjp.2013.39340

Cited by 13 publications

(11 citation statements)

References 14 publications

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“…Despite this, some clinicians have continued to dispute any association between these two disease processes. Even in the recent literature, there is still no consensus regarding ADPKD and the associated risk for RCC [6] [11] [14]- [17].…”

Section: Discussionmentioning

confidence: 99%

“…With the literature equivocal regarding an increased risk for RCC in ADPKD, there is a lack of clear guidance regarding early nephrectomy in patients who develop new or growing soft tissue nodules within their kidneys. Table 3 provides an overview of the body of literature published on the topic "RCC in ADPKD" [11] [12] [18] [23]- [30]. A review of these studies shows that the majority of investigators and clinicians acknowledge a link between ADPKD and the risk for RCC beyond the current risk associated with ESRD.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

The Association between Autosomal Dominant Polycystic Kidney Disease and Renal Cell Carcinoma

Hansen¹,

Derrick²,

Warriach³

et al. 2015

OJU

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Objectives: The relationship between autosomal dominant polycystic kidney disease (ADPKD) and renal cell carcinoma (RCC) is investigated to determine a link that would guide management due to elevated RCC risk. Current literature is inconclusive on this topic. Methods: This study is a retrospective chart review of patients having undergone nephrectomy. Those with pathology and history consistent with ADPKD were reviewed for presence of RCC. Results: The review at this institution revealed RCC in 18% of ADPKD patients who underwent nephrectomy. These rates are significantly higher than those found in the general population, and even greater than those would be expected in patients suffering end-stage renal disease (ESRD). Conclusions: Due to the increased prevalence of RCC in ADPKD, clinicians managing patients with ADPKD should maintain a high index of suspicion. Our data suggest a link between ADPKD and RCC, most likely at the genetic level. In light of this, we feel that further genetic research is needed to potentially discover the link between these two disease processes.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

The Association between Autosomal Dominant Polycystic Kidney Disease and Renal Cell Carcinoma

Hansen¹,

Derrick²,

Warriach³

et al. 2015

OJU

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show abstract

“…In comparison to ADPKD, ESRD and its associated acquired renal cystic disease are well-established risk factors for RCC and have widely been accepted in the literature [11,14,15]. The prevalence of RCC in ESRD patients is significantly higher than the prevalence of sporadic RCC in the general population (by an estimated factor of 1000) [7].…”

Section: Commentarymentioning

confidence: 99%

Adult Dominant Polycystic Kidney Disease (ADPKD)

Riese¹

2017

J Med Surg Pathol

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Autosomal-dominant polycystic kidney disease (ADPKD) is the most common inherited renal cystic disease affecting up to 10% of patients requiring some form of renal replacement therapy such as hemodialysis or kidney transplant. An association between autosomal-dominant polycystic kidney disease (ADPKD) and renal cell carcinoma (RCC) has been suspected since the 1930s. Many cases of RCC in ADPKD patients have been observed and published in the meantime. The gene mutations responsible for ADPKD have been discovered in the 1990s, however a direct genetic link to RCC has not been discovered so far, and the debate about an association between these two diseases is still controversial and ongoing. Many clinical experts who have published about this issue suspect a genetic link and state that ADPKD patients are much more prone to develop RCC than patients with end-stage renal disease (ESRD) of other causes. Currently there are no clear clinical guidelines regarding early nephrectomy in ADPKD patients with growing soft tissue nodules within their native kidneys. This commentary/ review on this important clinical issue describes the experience with these patients in one institution and gives an overview of the literature.

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“…39 O angiomiolipoma e o Carcinoma de células renais (CCR), por sua vez, são tumores raros em indivíduos com DRPAD. 40 O papel da DRPAD como fator de risco para o CCR ainda está em discussão. 40,41 Embora o gene PKD1, na síndrome de genes contí-guos, tenha sido relacionado à múltiplos angiomiolipomas em rins com alterações policísticas, as alterações genéticas associadas ao CCR e DRPAD têm permanecido desconhecidas.…”

Section: O Gene Pkd1 E a Policistinaunclassified

Autosomal dominant polycystic kidney disease: an update on epidemiological and molecular aspects

Alves¹,

Borelli²,

Tsuneto³

2015

Medicina (Ribeirão Preto)

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Doença renal policística autossômica dominante (DRPAD) é a causa hereditária mais comum da doença renal crônica estágio 5 e representa de 3% a 10,3% entre os doentes em diálise no Brasil. A DRPAD é causada por mutações em um de dois genes, PKD1 ou PKD2, que codificam, consequentemente, as policistinas 1 e 2 envolvidas na formação dos cistos. A doença é caracterizada pelo progressivo crescimento e desenvolvimento de múltiplos cistos renais bilaterais que levam à perda da função dos rins. A DRPAD apresenta diversas manifestações clínicas renais e extrarrenais, além de comorbidades. Algumas estratégias diagnósticas são consideradas de primeira escolha para a investigação clínica e molecular da DRPAD. O conhecimento de aspectos moleculares em associação com as manifestações clínicas apresentadas pela epidemiologia da doença proporciona melhor compreensão dos mecanismos de cistogênese e maior acurácia diagnóstica

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Bilateral papillary renal cell carcinoma and angiomyolipoma in the patients with autosomal dominant polycystic kidney disease: of two cases and literature review

Cited by 13 publications

References 14 publications

The Association between Autosomal Dominant Polycystic Kidney Disease and Renal Cell Carcinoma

The Association between Autosomal Dominant Polycystic Kidney Disease and Renal Cell Carcinoma

Adult Dominant Polycystic Kidney Disease (ADPKD)

Autosomal dominant polycystic kidney disease: an update on epidemiological and molecular aspects

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