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Fig 1.

Study design and testing strategy.

ACMG indicates American College of Medical Genetics and Genomics. Component cancers represent malignancies observed in PHTS patients.

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Table 1.

Demographic and clinical characteristics of 87 PTEN wildtype Cowden/Cowden-like syndrome and Bannayan-Riley-Ruvalcaba syndrome patients.

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Table 2.

Germline variants and copy number variation (CNVs) identified and associated syndromes.

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Table 3.

Clinicopathological characteristics of patients with identified germline alterations.

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Table 3 Expand

Fig 2.

Comparison of pathogenic and likely pathogenic germline variant spectra and frequencies in CS/CS-like/BRRS patients and TCGA apparently sporadic component cancer patients.

Solid-filled bars represent identified variant positive CS/CS-like and BRRS patients. Striped bars represent TCGA cancer patients with different colors indicating different cancer types. TCGA cancer types were selected because each is a PHTS component cancer. Abbreviations: CS, Cowden syndrome; BRRS, Bannayan-Riley-Ruvalcaba syndrome; OR, odds ratio; CI, confidence interval.

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