Cancer Prone Disease Section
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چکیده
Note Phenotypic spectrum variable; Growth: normal; Head: normal; Skin: normal; Hyperparathyroidism, jaw tumors, and kidney cysts; Hyperparathyroidism develops in about 95% of affected individuals due to parathyroid adenomas which can occur singley or multiply, with some patients undergoing several parathyroidectomies over the course of their lives; Parathyroid carcinoma develops in about 5% of patients (compare to 1 in 5 million in the general population); The jaw tumors consist of trabeculae of woven bone set in a cytologically bland fibrocellular stroma; About 50% develop fibro-osseous tumors of the maxilla or mandible, which may recur and are independent of the course of the parathyroid adenomas; Some families display an increased risk of developing kidney cysts (nephroblastomas or hamartomas) or adult Wilms tumors; Surgical removal of neoplastic tissues; Regular serum calcium level screening is a cost effective method to catch the development of parathyroid adenomas at an early stage; Dental x-rays are not recommended; may increase risk of development of jaw tumors.
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Cancer Prone Disease Section
Alias Multiple cutaneous and uterine leiomyomatosis (MCUL) Hereditary leiomyomatosis and renal cell cancer (HLRCC) Note Multiple cutaneous leiomyomatosis (MCUL) is characterized by multiple leiomyomas of the skin and uterus. When associated with renal cell cancer, this syndrome is referred to as hereditary leiomyomatosis and renal cell cancer (HLRCC). Inheritance Autosomal dominant with incompl...
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The definition of hereditary prostate cancer (HPC) is based on the family history (pedigree). The suggested criteria include 1) nuclear family with three (or more) cases of prostate cancer, 2) prostate cancer in three successive generations, or 3) at least two men diagnosed with the disease before the age of 55 years. Familial aggregation of cases that don't fulfill the HPC criteria are defined...
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