Hereditary Neoplastic Syndromes (Cancer Syndrome, Hereditary)

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.

Also Known As:

Networked: 52 relevant articles (0 outcomes, 5 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Ovarian Neoplasms (Ovarian Cancer)
3.	Pheochromocytoma
4.	von Hippel-Lindau Disease (von Hippel Lindau Disease)
5.	Multiple Endocrine Neoplasia Type 2a (MEN 2)

Experts

1.	Lynch, Henry T: 3 articles (09/2014 - 08/2007)
2.	Kaelin, William G: 3 articles (01/2006 - 11/2003)
3.	Arun, Banu K: 2 articles (06/2014 - 09/2010)
4.	Lu, Karen H: 2 articles (06/2014 - 09/2010)
5.	Vadaparampil, Susan T: 2 articles (01/2014 - 02/2012)
6.	Quinn, Gwendolyn P: 2 articles (01/2014 - 02/2012)
7.	Merino, Maria J: 2 articles (01/2013 - 06/2007)
8.	Lynch, Patrick M: 2 articles (01/2008 - 08/2007)
9.	Lynch, Jane F: 2 articles (01/2008 - 08/2007)
10.	Kaelin, W G: 2 articles (09/2001 - 03/2001)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hereditary Neoplastic Syndromes:

1.	DNA (Deoxyribonucleic Acid)IBA 05/20/2013 - "In sequencing germline DNA, mutations may be encountered that are associated with increased susceptibility not only to hereditary cancer syndromes but also to other diseases; in those cases, disclosing germline data could be clinically relevant and even lifesaving. " 08/10/2007 - "We conclude that the physician's role in advising DNA testing is no small matter, given that a hereditary cancer syndrome's sequelae may be far reaching. " 07/01/1994 - "Due to the rapid developments in molecular genetics in HNPCC, it seems very likely that HNPCC will be the first common hereditary cancer syndrome, which can be identified by simple blood DNA tests. " 01/01/2008 - "Clearly, the family physician, oncology specialist, genetic counselor, and cancer geneticist must know fully the complexity of hereditary cancer syndromes, their differential diagnosis, in order to establish a diagnosis, direct highly-targeted surveillance and management, and then be able to communicate effectively with the molecular geneticist so that an at-risk patient's DNA can be tested in accord with the syndrome of concern. " 01/01/1997 - "In most hereditary cancer syndromes, finding a correspondence between various genetic mutations within a gene (genotype) and a patient's clinical cancer history (phenotype) is challenging; to date there are few clinically meaningful correlations between specific DNA intragenic mutations and corresponding cancer types. "
2.	Prostaglandins D (PGD)IBA 06/01/2014 - "This study investigated and compared awareness and acceptance of PGD among patients with different hereditary cancer syndromes. " 01/01/2014 - "More than half of the participants reported PGD was an acceptable option for families with hereditary cancer syndromes and thought individuals with a strong family or personal history should be provided with information about PGD. " 02/01/2012 - "A systematic literature review was conducted through PubMed, Wiley Interscience, PsychInfo, and Cochrane Library databases to identify all articles assessing consumer knowledge and attitudes of PGD for hereditary cancer syndromes. " 01/01/2012 - "[Preimplantation genetic diagnosis (PGD) of hereditary cancer syndromes]." 09/01/2010 - "Preimplantation genetic diagnosis (PGD) is a technology that allows embryos without a deleterious mutation associated with a hereditary cancer syndrome to be identified and implanted. "
3.	OxygenIBA 01/01/2004 - "Analysis of von Hippel-Lindau hereditary cancer syndrome: implications of oxygen sensing." 09/01/2001 - "Molecular pathogenesis of the von Hippel-Lindau hereditary cancer syndrome: implications for oxygen sensing." 11/01/2003 - "Recent studies of a relatively rare hereditary cancer syndrome, von Hippel-Lindau (VHL) disease, have shed new light on the molecular pathogenesis of kidney cancer and, perhaps more important, on how mammalian cells sense and respond to changes in oxygen availability. "
4.	Succinate Dehydrogenase (Fumarate Reductase)IBA 01/01/2006 - "These tumors can arise in the context of hereditary cancer syndromes such as von Hippel- Lindau disease, multiple endocrine neoplasia type 2, and neurofibromatosis 1. Recent studies indicate that germ line mutations of genes encoding specific succinate dehydrogenase (SDH) subunits also predispose individuals to pheochromocytomas and paragangliomas. "
5.	FatsIBA 01/01/2004 - "The present study shows, that the increased risk for ovarian cancer is connected with the advanced age, the use of some hormonal medicaments, diet reach of animal fats, as well as the presence of family history or hereditary cancer syndromes (5-10% of the cases). "
6.	Retinaldehyde (Retinal)IBA 12/01/2010 - "von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary cancer syndrome that predisposes to the development of a variety of benign and malignant tumours, especially cerebellar haemangioblastomas, retinal angiomas and clear-cell renal cell carcinomas (RCC). " 03/10/2001 - "Germline mutations of the von Hippel-Lindau tumor suppressor gene (VHL) in humans causes a hereditary cancer syndrome characterized by the development of retinal and central nervous system hemangioblastomas. " 07/01/2013 - "Von Hippel-Lindau (VHL) disease is an autosomal dominant hereditary cancer syndrome that predisposes to the development of a variety of benign and malignant tumors, especially cerebellar hemangioblastomas, retinal angiomas and clear-cell renal cell carcinomas (RCC). " 01/01/2012 - "von Hippel-Lindau (VHL) disease is a hereditary cancer syndrome in which the patients develop retinal and central nervous system hemangioblastomas, pheochromocytomas and clear-cell renal tumors. " 06/01/2005 - "Von Hippel-Lindau (VHL) disease is a hereditary cancer syndrome that predisposes to the development of a panel of highly vascularized tumors including CNS and retinal hemangioblastomas, endolymphatic sac tumors, clear-cell renal cell carcinomas (RCC), pheochromocytomas and pancreatic neuroendocrine tumors. "
7.	hereditary Leiomyomatosis and renal cell cancerIBA 02/12/2007 - "Hereditary leiomyomatosis and renal cell cancer is a recently described hereditary cancer syndrome in which affected individuals are predisposed to the development of leiomyomas of the skin and uterus. " 01/01/2013 - "Hereditary leiomyomatosis and renal cell cancer (HLRCC) is a hereditary cancer syndrome in which affected individuals are predisposed to the development of multiple leiomyomas of the skin and uterus and aggressive forms of kidney cancer. " 06/01/2010 - "Hereditary leiomyomatosis and renal cell cancer is a hereditary cancer syndrome in which affected individuals are at risk for cutaneous and uterine leiomyomas, and renal cancer. " 06/01/2007 - "Hereditary leiomyomatosis and renal cell cancer is a recently described hereditary cancer syndrome in which affected individuals are at risk for cutaneous and uterine leiomyomas, and kidney cancer. "
8.	Fumarate Hydratase (Fumarase)IBA 08/01/2015 - "Mutations in fumarate hydratase (FH) on chromosome 1q43 cause a rare cancer syndrome, hereditary leiomyomatosis and renal cell cancer (HLRCC), but are rare in nonsyndromic and common uterine leiomyoma (UL) or fibroids. " 12/01/2004 - "Individuals with the inherited cancer syndrome hereditary leiomyomatosis and renal cell cancer (HLRCC) that have germline defects in the fumarate hydratase (FH) gene develop papillary RCC and uterine and skin leiomyomas. "
9.	EnzymesIBA 05/30/2013 - "The identification of mutated metabolic enzymes in hereditary cancer syndromes has established a direct link between metabolic dysregulation and cancer. " 09/01/2004 - "The group consists of following subgroups: 1) hereditary cancer syndromes with mutations in the NM-attached oncoproteins or tumor suppressor genes; 2) sporadic tumors with somatic mutations in the NM-attached oncoproteins, tumor suppressor genes or replication enzymes; 3) leukemias with fused NMPs. "
10.	Pancreatic islet cell tumorsIBA 11/01/1997 - "von Hippel-Lindau disease is a hereditary cancer syndrome characterized by the development of vascular tumors of the central nervous system and retina, clear cell renal carcinomas, pheochromocytomas, pancreatic islet cell tumors, endolymphatic sac tumors, and benign cysts affecting a variety of organs. " 05/01/2012 - "Pancreatic islet cell tumors are neuroendocrine tumors, which can produce hormones and can arise as part of multiple endocrine neoplasia type 1 or von-Hippel-Lindau-disease, two genetically well-defined hereditary cancer syndromes. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 09/09/2009 - "Multiple primary neoplasms occur either by hazard or in the context of hereditary cancer syndromes, after chronic toxic exposition, in immunodeficiency or as secondary malignancies after radio- and/or chemotherapy. " 01/01/1997 - "The sequential appearance of two different brain tumors in the same patient without intervening radiation or chemotherapy is a rare event, most often seen in hereditary cancer syndromes. "
2.	Chemoprevention 10/01/2000 - "Therefore, genetic testing will become integral in tailoring surveillance, chemoprevention, and surgical management plans for patients at risk for hereditary cancer syndromes. "
3.	Thyroidectomy 04/01/1992 - "Prediction of affected MEN2A gene carriers by DNA linkage analysis for early total thyroidectomy: a progress in clinical screening program for children with hereditary cancer syndrome."
4.	Ovariectomy (Oophorectomy) 02/01/2004 - "Although the role of prophylactic oophorectomy is currently under debate and not well defined, it is of increasing and considerable relevance, especially in premenopausal women, particularly those with identifiable hereditary cancer syndromes. "