Familial Pancreatic Cancer

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Retrieved

2022-04-26

Source

Gard

Trials

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Genes

BRCA2, PALB2, CDKN2A, PALLD, BRCA1, RABL3, SMAD4, KRAS, TP53, ATM, H3P10, PALD1, MSH6, PKHD1, FANCG, SUB1, H3P8, GTF2H5, CD47, IDO2, MAGT1, CDH10, SF3B6, LAMTOR2, CDK2, CDKN1A, CHEK2, RPP14, CDK2AP2, FANCC

Drugs

(1-methyl-2-nitro-1H-imidazole-5-yl)methyl N,N'-bis(2-bromoethyl) diamidophosphate, 2-hydroxy-N,N,N-trimethylethan-1-aminium (Z)-4-(5-((3-benzyl-4-oxo-2-thioxothiazolidin-5-ylidene)methyl)furan-2-yl)benzoate, 26 base single stranded phosphodiester DNA oligonucleotide, 3-bromopyruvate, 4-amino-1-[(1S,4R,5S)-2-fluoro-4,5-dihydroxy-3-(hydroxymethyl) cyclopent-2-en-1-yl] pyrimidin-2-one, 4-imino-1, 3-diazobicyclo-[3.1.0]-hexan-2-one, 5'-O-(trans-9''-octadecenoyl)-1-beta-D-2'deoxy-2',2'-difluorocytidine, 5,10-methylene-tetrahydrofolic acid, 5-10-Methylene-tetrahydrofolate, Adenoviral vector of serotype 5 modified to contain a chimeric sequence consisting of a minimal urokinase-type plasminogen activator receptor promoter preceded by three Notch-responsive elements, and coated with oligopeptide end-modified poly (beta-amino) esters, Algenpantucel-L, Antroquinonol, Autologous human adipose perivascular stromal cells genetically modified to secrete soluble tumour necrosis factor-related apoptosis-inducing ligand, Axitinib ( INLYTA ), Bevacizumab ( Equidacent, AVASTIN, Aybintio, MVASI ), Bovine bile extract, Brivudine, Cetuximab ( ERBITUX ), Chimeric antibody to mesothelin, Chimeric monoclonal antibody against claudin-18 splice variant 2, Cisplatin (liposomal), Cysteamine bitartrate ( CYSTAGON ), Cytochrome P450 isoform 2B1 gene transfected human embryonic kidney 293 cells encapsulated in polymeric cellulose sulphate, Demcizumab, Deuterium oxide, Devimistat, Eryaspase ( GRASPA ), G17(9) gastrin-diphtheria toxoid conjugate, GVAX, Genetically modified human adenovirus encoding human PH20 hyaluronidase, Glufosfamide, Heat killed whole cell mycobacterium obuense, Heparan sulfate mimetic, Herpes simplex type 1 virus containing cellular B-myb gene as tumour-specific promoter, Human telomerase reverse transcriptase peptide (611-626), Human trtravalent, bi-specific monoclonal antibody that binds and co-inhibits two growth factor receptors; insulin-like growth factor receptor 1 (IGF-1R) and v-erb-b2 avian erythroblastic leukemia viral oncogene homolog 3 (ErbB3), Humanised IgG4 monoclonal antibody to the human toll-like receptor type 2, Humanized IgG monoclonal antibody hRS7 conjugated to topoisomerase I inhibitor, Imexon, Immunoglobulin G1, anti-(human tumour-associated calcium signal transducer 2)(human-Mus musculus monoclonal hRS7 heavy chain), disulfide with human-Mus musculus monoclonal hRS7 ê-chain, dimer, hexakis(thioether) with (4S)-4-[[[[4-[[(2S)-2-(4-aminobutyl)-2-[[2-[2-[[26-[4-[[[[4-[(3-mercapto-2,5-dioxo-1-pyrrolidinyl)methyl]cyclohexyl][...][3',4':6,7]indolizino[1,2-b]quinoline-3,14(4H,12H)-dione, Iodine (131I) anti-CEA sheep-human chimeric monoclonal antibody, Live attenuated Listeria monocytogenes delta actA/delta inlB strain expressing human mesothelin, Masitinib mesilate, Mixture of seven synthetic fragments consisting of p21 RAS peptides, Modified adenovirus serotype 5/35 containing a CMV promoter-driven transgene cassette with the human transgenes for a membrane-bound CD40 ligand and full length 4-1BBL, N-[(2S)-2,3-dihydroxypropyl]-3-[(2-fluoro-4-iodophenyl)amino]isonicotinamide hydrochloride, N1,N14,-diethyl-3,12,-dihydroxyhomospermine, Nanoliposomal irinotecan ( ONIVYDE ), Nimotuzumab ( THERALOC ), PEGylated recombinant human hyaluronidase PH20, Paclitaxel (liposomal), Paclitaxel protein-bound particles ( ABRAXANE ), Pegylated recombinant human interleukin-10, Polyinosine-polycytidylic acid coupled with the polycationic polyethyleneimine, Re188 P2045 somatostatin analog, Recombinant human monoclonal antibody of the IgG1 kappa class against prostate stem cell antigen, Relacorilant, Rubitecan, S-[2,3-bispalmitoyloxy-(2R)-propyl]-cysteinyl-GNNDESNISFKEK, Salirasib, Targeted Gastrin 17 Complexed Peptide, Trabedersen, Trastuzumab ( HERCEPTIN ), Yttrium (90Y)-DOTA-radiolabelled humanized monoclonal antibody against mucin 1, [5-amino-1-(4-fluoro-phenyl)-1H-pyrazol-4-yl]-[3-(2,3-dihydroxy-propoxy)-phenyl]-methanone, human reovirus type 3 Dearing strain, motixafortide, sodium 2-hydroxylinoleate

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Familial pancreatic cancer (FPC) is the occurrence of pancreatic cancer in two or more first-degree relatives (parent and child, or two siblings). It is sometimes referred to as FPC only when there is not a known hereditary cancer syndrome in an affected family. In familial cases, pancreatic cancer often occurs before age 50 (earlier than other forms of pancreatic cancer). In 60% of cases it occurs within the head of the pancreas. Symptoms of pancreatic cancer are generally non-specific and may include pain in the upper abdomen that radiates to the back; loss of appetite; significant weight loss; and jaundice due to bile duct obstruction. Pancreatic cancer often goes undetected until the advanced stages of the disease, and rapid tumor growth and metastasis are common.

A specific genetic cause of FPC (in the absence of a known syndrome) has not been identified. Familial cases of pancreatic cancer due to hereditary conditions may be caused by mutations in any of several genes. Individuals who carry a mutation in one of these genes are often at an increased risk of other types of cancers as well. Currently, only 10-20% of families with FPC will have a mutation identified by genetic testing. Hereditary cancer syndromes and other inherited conditions associated with pancreatic cancer include:

In many cases, inheritance of FPC is autosomal dominant; in some cases, is may be multifactorial. It is important to note that pancreatic cancer itself is not inherited, but a genetic predisposition to developing cancer is inherited.

Treatment may include surgical resection, chemotherapy, and/or radiotherapy. Resection is the only potential cure, but most patients have advanced, non-resectable tumors by the time of diagnosis.