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. 2013 Jan 24;8(1):e55209. doi: 10.1371/journal.pone.0055209

Table 1. Tumor types showing the strongest evidence for familial occurrence.

Topography Morphology Cluster score p-value* Number of patients in FCR Number of clusters** Number of patients in clusters** Suggestive predisposition gene
central nervous system hemangioblastoma 4.98 2.77E−14 141 5 13 VHL
thyroid gland medullary carcinoma 3.55 7.86E−21 335 9 22 RET
Skin Kaposi sarcoma 1.91 2.05E−13 537 9 19
Pancreas neuroendocrine carcinoma 1.40 2.75E−06 386 5 10 MEN1, RET
Kidney nephroblastoma 1.26 5.98E−06 428 5 10 WT1
small intestine neuroendocrine carcinoma 0.98 1.19E−09 1154 10 21
hematopoetic and reticuloendothelialsystem myelosclerosis 0.69 2.75E−09 2186 14 28
vulva and vagina squamous cell neoplasms and carcinoma 0.63 1.37E−10 3092 17 36
mesothelium mesothelioma 0.62 3.49E−06 1750 10 20
Breast mucinous and mucinous cystic tumor 0.57 2.45E−07 2564 13 27
thyroid gland papillary adenocarcinoma 0.56 2.77E−20 7863 41 82
central nervous system neurofibroma 0.55 1.02E−05 2062 9 21 NF1
central nervous system neoplasm malignant 0.51 1.89E−05 2346 11 22
hematopoetic and reticuloendothelialsystem polycythemia vera 0.50 6.07E−05 2173 10 20
hematopoetic and reticuloendothelialsystem chronic lymphatic leukemia, b-cell 0.48 3.37E−13 7478 31 66
Kidney neoplasm malignant 0.47 1.20E−05 3015 13 26
lymph node Hodgkin lymphoma 0.46 2.77E−09 5499 22 47
esophagus squamous cell neoplasms and carcinoma 0.45 8.37E−09 5514 23 46
Prostate epithelial neoplasm and carcinoma 0.44 2.82E−05 3187 13 26
Lip squamous cell neoplasms and carcinoma 0.35 1.04E−05 7167 22 46
*

Poisson distribution, two-sided test with 95% confidence level, p-values adjusted with FDR for multiple comparisons** CI-low ≥1.