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Neoplastic Syndromes, Hereditary

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Definition: The condition of a pattern of malignancies within a family, but not every individual's necessarily having the same neoplasm. Characteristically the tumor tends to occur at an earlier than average age, individuals may have more than one primary tumor, the tumors may be multicentric, usually more than 25 percent of the individuals in direct lineal descent from the proband are affected, and the cancer predisposition in these families behaves as an autosomal dominant trait with about 60 percent penetrance.  do not use /blood supply /chem /second /secret /ultrastruct; coord IM with specific hereditary organ/neopl term (IM) or specific hereditary histol type (IM)   
Examples Adenomatous Polyposis Coli; Basal Cell Nevus Syndrome; Birt-Hogg-Dube Syndrome; Colorectal Neoplasms, Hereditary Nonpolyposis; Dysplastic Nevus Syndrome; Exostoses, Multiple Hereditary; Hamartoma Syndrome, Multiple; Hereditary Breast and Ovarian Cancer Syndrome; Li-Fraumeni Syndrome; Multiple Endocrine Neoplasia; Tuberous Sclerosis; Wilms Tumor; Neurofibromatoses; Peutz-Jeghers Syndrome 		Other names Cancer Syndromes, Hereditary; Syndromes, Hereditary Neoplastic; Syndromes, Hereditary Cancer; Syndrome, Hereditary Neoplastic; Syndrome, Hereditary Cancer; Neoplastic Syndrome, Hereditary; Hereditary Neoplastic Syndrome; Hereditary Cancer Syndrome; Cancer Syndrome, Hereditary; Hereditary Neoplastic Syndromes; Hereditary Cancer Syndromes
 
SubstanceCAS Registry & nameCategoriesSource
Meningioma, familial  0   *Meningioma *Neoplastic Syndromes, Hereditary.
Leiomyomatosis and renal cell cancer, hereditary  0   *Kidney Neoplasms *Neoplastic Syndromes, Hereditary *Leiomyomatosis.
Turcot syndrome  0   *Brain Neoplasms *Neoplastic Syndromes, Hereditary *Colorectal Neoplasms.
Thyroid cancer, familial medullary  0   *Neoplastic Syndromes, Hereditary *Thyroid Neoplasms.
Familial cylindromatosis  0   *Neoplastic Syndromes, Hereditary *Skin Neoplasms.
Trichoepithelioma multiple familial  0   *Neoplastic Syndromes, Hereditary.
Diaphyseal medullary stenosis with malignant fibrous histiocytoma  0   *Bone Diseases, Developmental *Neoplastic Syndromes, Hereditary *Histiocytoma, Benign Fibrous.
Myelocytic leukemia-like syndrome, familial, chronic  0   *Leukemia, Myeloid *Neoplastic Syndromes, Hereditary.
Hemangioma, capillary infantile  0   *Neoplastic Syndromes, Hereditary *Hemangioma, Capillary.

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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