Complete and incomplete forms of Beckwith-Wiedemann syndrome: Their oncogenic potential

Sotelo‐Avila, Cirilo; González-Crussí, Frank; Fowler, John W.

doi:10.1016/s0022-3476(80)80322-2

Cited by 283 publications

(133 citation statements)

References 22 publications

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“…3,10-14 A variety of other malignant and benign tumors have been reported. [12][13][14] The overall risk for tumor development in children with BWS has been estimated at 7.5% with a range of risk estimates between 4 and 21%. 3,[10][11][12]13,[15][16][17] Clinical findings associated with higher risks of tumor development include hemihyperplasia, nephromegaly, and nephrogenic rests.…”

Section: Introductionmentioning

confidence: 99%

“…[12][13][14] The overall risk for tumor development in children with BWS has been estimated at 7.5% with a range of risk estimates between 4 and 21%. 3,[10][11][12]13,[15][16][17] Clinical findings associated with higher risks of tumor development include hemihyperplasia, nephromegaly, and nephrogenic rests. 10,18 Although different molecular subgroups have been shown to be associated with different tumor rates and tumor profiles, 19,[20][21][22][23][24] further clinical studies are needed to validate these data before implementing stratified surveillance protocols.…”

Section: Introductionmentioning

confidence: 99%

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Beckwith–Wiedemann syndrome

2009

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In association withBeckwith-Wiedemann syndrome Beckwith-Wiedemann syndrome (BWS) is a model disorder for the study of imprinting, growth dysregulation, and tumorigenesis. Unique observations in this disorder point to an important embryonic developmental window relevant to the observations of increased monozygotic twinning and an increased rate of epigenetic errors after subfertility/assisted reproduction.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Beckwith–Wiedemann syndrome

2009

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“…A significant complication of this syndrome is the increased risk of developing malignant tumours. The reported incidence ranges from 7-10% [6]. Association with hemihypertrophy as in our case, increases the risk of malignancy to 40% [7].…”

Section: Discussionmentioning

confidence: 53%

Beckwith Weidemann Syndrome

Raju

Dhulia

Sharma

2004

Medical Journal Armed Forces India

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“…The risk of replicative error in DNA with cell division remains but the organ size governance structures of Hippo and Hippomediated cross-talk to mTOR are configured to ensure fidelity of organ size determination pathways. In a contrasting clinical and molecular scenario there are some conditions such as Beckwith Widemann syndrome in which a juxtaposition of increased organ size and an increased rate of malignancy does occur (17). One of the "other means" in which the Hippo pathway is controlled is by epigenetic regulation.…”

Section: Evolution Of Pathway Configuration: Core Axis Cluesmentioning

confidence: 99%

Oxford and the Savannah: Can the Hippo Provide an Explanation for Peto's Paradox?

Kelleher¹,

O’Sullivan²

2014

Clinical Cancer Research

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Peto's paradox is the counterintuitive finding that increasing body mass and thereby cell number does not correlate with an increase in cancer incidence across different species. The Hippo signaling pathway is an evolutionarily conserved system that determines organ size by regulating apoptosis and cell proliferation. It also affects cell growth by microRNA-29 (miR-29)-mediated cross-talk to the mTOR signaling pathway. Whether these pathways that decide organ size could explain this paradox merits consideration. Inactivation of most genes of the Hippo pathway in Drosophila melanogaster genetic screens causes excessive tissue-specific growth of developing tissues. Altered Hippo pathway activity is frequently found in diverse tumor types, but mutations of component pathway genes are rare. Most Hippo pathway components are encoded by tumor suppressor genes (TSG), but an exception is the downstream effector gene called YAP. Activity of the Hippo pathway causes deactivating phosphorylation of YES-associated protein (YAP) with nuclear exclusion. YAP can also be phosphorylated at a second site, S127, by AKT. YAP induces the expression of genes responsible for proliferation and suppression of apoptosis. Resolving Peto's paradox may serendipitously provide new insights into the biology and treatment of cancer. This article considers Hippo signaling and Peto's paradox in the context of TSG-oncogene computed models. Interspecies differences in dietary composition, metabolic rates, and anabolic processes are also discussed in the context of Hippo-mTOR signaling. The metabolically important LKB1-AMPK (liver kinase B1-AMP activated protein kinase) signaling axis that suppresses the mTOR pathway is also considered. Clin Cancer Res; 20(3); 557-64. Ó2013 AACR.

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Complete and incomplete forms of Beckwith-Wiedemann syndrome: Their oncogenic potential

Cited by 283 publications

References 22 publications

Beckwith–Wiedemann syndrome

Beckwith–Wiedemann syndrome

Beckwith Weidemann Syndrome

Oxford and the Savannah: Can the Hippo Provide an Explanation for Peto's Paradox?

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