Cancer Prone Disease Section
نویسندگان
چکیده
The penetrance for colon polyps is close to 100% and bi-allelic MUTYH mutation carriers generally develop 10-100’s adenomatous polyps/adenomas of the colon and the rectum. Approximately one third of patients also develop polyps/adenomas in the upper gastrointestinal tract. Other manifestations frequently seen in Familial Adenomatous Polyposis (FAP) are also present in minority of MAP patients: osteomas, pigmented retinal lesions (congenital hypertrophy of the retinal pigment epithelium; CHRPE) and tooth disorders. Recently also sebaceous gland tumors (Muir-Torre syndrome) and pilomatricomas have been reported in MAP-kindreds.
منابع مشابه
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...
متن کاملCancer Prone Disease Section
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...
متن کاملEvaluation of incidence of intestinal complications during radiation therapy in two supine and prone positions in patients with rectal cancer
Introduction: Radiation therapy is one of the main treatments for rectal cancer. In three-dimensional conformal radiotherapy, exposure of healthy tissue around the tumor is unavoidable. The small intestine is the most important organ at risk of rectal cancer radiotherapy. Intestinal complications are a major factor limiting the dose radiotherapy for rectal cancer. The most com...
متن کاملCancer Prone Disease Section
Stiff-person syndrome is a rare neurological disorder characterized by stiffness of skeletal muscles with superimposed spasms. The syndrome is a putative autoimmune disease occurring as an idiopathic or paraneoplastic condition. It is often associated with antibodies to glutamic acid decarboxylase (GAD) or, less commonly, to the 128 kD synaptic protein later amphiphysin (AMPH) and few other aut...
متن کاملCancer Prone Disease Section
Alias: Familial pancreatic cancer Inheritance It has been estimated that as many as 10% of pancreatic cancers have a hereditary basis; five genetic syndromes have been identified that are associated with the familial aggregation of pancreatic cancer; these include: The second breast cancer syndrome (BRCA2), the familial atypical multiple mole melanoma (FAMMM), the Peutz-Jeghers Syndrome, the he...
متن کامل