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.