MCID: MMM001
MIFTS: 51

Mammary Paget's Disease

Categories: Rare diseases, Cancer diseases, Reproductive diseases

Aliases & Classifications for Mammary Paget's Disease

MalaCards integrated aliases for Mammary Paget's Disease:

Name: Mammary Paget's Disease 12 49 14
Paget's Disease of the Breast 12 72
Paget's Disease of the Nipple 49 55
Paget Disease of the Breast 49 55
Paget's Disease, Mammary 49 69
Mammary Paget Disease 12 55
Paget's Disease 12 69
Paget Disease of the Nipple 55
Pagets Disease Mammary 51
Paget Cell Neoplasm 12
Osteitis Deformans 69

Characteristics:

Orphanet epidemiological data:

55
paget disease of the nipple
Age of onset: Adult;

Classifications:



External Ids:

Disease Ontology 12 DOID:3443
NCIt 46 C7073
Orphanet 55 ORPHA180275
MESH via Orphanet 42 D010144
UMLS via Orphanet 70 C0030185 C1704323
ICD10 via Orphanet 33 C50.0
ICD10 32 M88.9

Summaries for Mammary Paget's Disease

NIH Rare Diseases : 49 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 180275Disease definitionPaget disease of the nipple describes a rare presentation of breast cancer, seen most frequently in women aged 50-60, manifesting with nipple drainage and itching, erythema, crusty and excoriated nipple, thickened plaques, and hyperpigmentation (less frequently). It is due to tumorcells invading the nipple-areola complex and represents 1-3% of all new breast cancer diagnoses.Visit the Orphanet disease page for more resources. Last updated: 3/20/2014

MalaCards based summary : Mammary Paget's Disease, also known as paget's disease of the breast, is related to paget disease, extramammary and breast osteosarcoma, and has symptoms including sciatica, muscle cramp and back pain. An important gene associated with Mammary Paget's Disease is ERBB2 (Erb-B2 Receptor Tyrosine Kinase 2), and among its related pathways/superpathways are Developmental Biology and Gastric cancer. The drugs Aredia and Medroxyprogesterone acetate have been mentioned in the context of this disorder. Affiliated tissues include breast, bone and skin, and related phenotypes are Decreased shRNA abundance (Z-score < -2) and Decreased shRNA abundance (Z-score < -2)

Wikipedia : 72 Paget\'s disease of the breast is a type of cancer that outwardly may have the appearance of eczema,... more...

Related Diseases for Mammary Paget's Disease

Diseases related to Mammary Paget's Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 259)
# Related Disease Score Top Affiliating Genes
1 paget disease, extramammary 31.6 ERBB2 KRT20 KRT7 MUC1
2 breast osteosarcoma 10.6 ERBB2 KRT8
3 breast intraductal proliferative lesion 10.6 EGFR ERBB2
4 tumor suppressor gene on chromosome 11 10.5 EGFR ERBB2
5 neurenteric cyst 10.5 KRT18 KRT8
6 endolymphatic sac tumor 10.5 KRT7 KRT8
7 hidrocystoma 10.4 KRT7 KRT8
8 prostate squamous cell carcinoma 10.4 KRT7 KRT8
9 nasal cavity cancer 10.4 KRT7 KRT8
10 cryptogenic cirrhosis 10.4 KRT18 KRT8
11 malignant peritoneal mesothelioma 10.4 EGFR MUC1
12 bronchiolo-alveolar adenocarcinoma 10.4 EGFR KRT7
13 epithelioid trophoblastic tumor 10.4 KRT18 KRT7
14 large cell acanthoma 10.4 KRT14 KRT7
15 hepatoid adenocarcinoma 10.3 EGFR MUC1
16 scirrhous adenocarcinoma 10.3 ERBB2 PGR
17 breast apocrine carcinoma 10.3 ERBB2 PGR
18 middle ear adenoma 10.3 KRT7 MUC1
19 breast adenomyoepithelioma 10.3 KRT14 KRT7
20 parachordoma 10.3 KRT7 MUC1
21 breast myoepithelial neoplasm 10.3 KRT14 KRT7
22 bile duct mucoepidermoid carcinoma 10.3 KRT7 MUC1
23 nodular hidradenoma 10.3 KRT7 MUC1
24 basal cell carcinoma, infundibulocystic 10.3 KRT14 KRT20
25 cystic basal cell carcinoma 10.3 KRT20 KRT7
26 eccrine porocarcinoma 10.3 KRT7 MUC1
27 pleomorphic liposarcoma 10.3 KRT8 MUC1
28 lung acinar adenocarcinoma 10.3 KRT20 KRT7
29 vaginal benign neoplasm 10.3 KRT20 KRT7
30 seminal vesicle adenocarcinoma 10.3 KRT20 KRT7
31 epispadias 10.3 CALCA ERBB2
32 transverse colon cancer 10.3 KRT20 KRT7
33 ovarian large-cell neuroendocrine carcinoma 10.3 KRT20 KRT7
34 vaginal adenoma 10.3 KRT20 KRT7
35 vaginal tubulovillous adenoma 10.3 KRT20 KRT7
36 metanephric adenoma 10.3 KRT18 KRT7
37 eyelid neoplasm 10.3 KRT20 KRT7
38 malignant syringoma 10.3 KRT20 KRT7
39 monophasic synovial sarcoma 10.3 KRT7 MUC1
40 krukenberg carcinoma 10.3 KRT20 KRT7
41 odontoma 10.3 KRT14 KRT18 KRT8
42 large intestine adenocarcinoma 10.3 KRT20 KRT7
43 linitis plastica 10.3 KRT20 KRT7
44 small cell carcinoma of the bladder 10.3 KRT20 KRT7
45 nephrogenic adenoma 10.3 KRT7 MUC1
46 adenoid squamous cell carcinoma 10.3 KRT20 KRT7
47 eccrine sweat gland neoplasm 10.3 KRT20 KRT7
48 proliferating trichilemmal cyst 10.3 KRT18 KRT7 KRT8
49 adenomyoma 10.2 KRT7 MUC1
50 seminal vesicle tumor 10.2 KRT18 KRT7

Graphical network of the top 20 diseases related to Mammary Paget's Disease:



Diseases related to Mammary Paget's Disease

Symptoms & Phenotypes for Mammary Paget's Disease

UMLS symptoms related to Mammary Paget's Disease:


sciatica, muscle cramp, back pain

GenomeRNAi Phenotypes related to Mammary Paget's Disease according to GeneCards Suite gene sharing:

25
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Decreased shRNA abundance (Z-score < -2) GR00366-A-154 9.4 KRT18
2 Decreased shRNA abundance (Z-score < -2) GR00366-A-17 9.4 KRT18
3 Decreased shRNA abundance (Z-score < -2) GR00366-A-180 9.4 KRT8
4 Decreased shRNA abundance (Z-score < -2) GR00366-A-33 9.4 KRT18
5 Decreased shRNA abundance (Z-score < -2) GR00366-A-40 9.4 KRT8
6 Decreased shRNA abundance (Z-score < -2) GR00366-A-68 9.4 KRT7
7 Decreased shRNA abundance (Z-score < -2) GR00366-A-71 9.4 KRT18 KRT7 KRT8
8 Decreased shRNA abundance (Z-score < -2) GR00366-A-72 9.4 KRT7
9 Decreased shRNA abundance (Z-score < -2) GR00366-A-84 9.4 KRT18
10 Decreased shRNA abundance (Z-score < -2) GR00366-A-91 9.4 KRT7

MGI Mouse Phenotypes related to Mammary Paget's Disease:

43
# Description MGI Source Accession Score Top Affiliating Genes
1 digestive/alimentary MP:0005381 9.63 KRT8 CFTR EGFR ERBB2 ESR1 KRT14
2 neoplasm MP:0002006 9.35 PGR EGFR ERBB2 ESR1 KRT14
3 reproductive system MP:0005389 9.17 KRT8 PGR CFTR EGFR ERBB2 ESR1

Drugs & Therapeutics for Mammary Paget's Disease

FDA approved drugs:

# Drug Name Active Ingredient(s) 17 Company Approval Date
1
Aredia 17 45 PAMIDRONATE DISODIUM Chiron August 1996

Drugs for Mammary Paget's Disease (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 49)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Medroxyprogesterone acetate Approved, Investigational Phase 3 71-58-9
2
Tamoxifen Approved Phase 3 10540-29-1 2733526
3
Citric Acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
4 Antineoplastic Agents, Hormonal Phase 3
5 Bone Density Conservation Agents Phase 3
6 Contraceptive Agents Phase 3
7 Contraceptive Agents, Male Phase 3
8 Contraceptives, Oral Phase 3
9 Estrogen Antagonists Phase 3
10 Estrogen Receptor Modulators Phase 3
11 Estrogens Phase 3
12 Hormone Antagonists Phase 3
13 Hormones Phase 3
14 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
15
Medroxyprogesterone Phase 3 520-85-4 10631
16 Selective Estrogen Receptor Modulators Phase 3
17 Citrate Nutraceutical Phase 3
18
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
19
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
20
Melphalan Approved Phase 2 148-82-3 460612 4053
21 Thiotepa Approved, Investigational Phase 2 52-24-4 5453
22
Trastuzumab Approved, Investigational Phase 2 180288-69-1 9903
23
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
24
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
25
Triamcinolone Approved, Vet_approved Phase 2 124-94-7 31307
26
Docetaxel Approved May 1996, Investigational Phase 2 114977-28-5 148124 9877265
27
Doxil Approved June 1999 Phase 2 31703
28 Alkylating Agents Phase 2
29 Antirheumatic Agents Phase 2
30 Immunosuppressive Agents Phase 2
31 Albumin-Bound Paclitaxel Phase 2
32 Anti-Bacterial Agents Phase 2
33 Antibiotics, Antitubercular Phase 2
34 Antimitotic Agents Phase 2
35 Antineoplastic Agents, Phytogenic Phase 2
36 Topoisomerase Inhibitors Phase 2
37 triamcinolone acetonide Phase 2
38 Triamcinolone diacetate Phase 2
39 Triamcinolone hexacetonide Phase 2
40 Omega 3 Fatty Acid Nutraceutical Phase 2
41
Metronidazole Approved Phase 1 443-48-1 4173
42
Pancrelipase Approved, Investigational Phase 1 53608-75-6
43 Anti-Infective Agents Phase 1
44 Antiparasitic Agents Phase 1
45 Antiprotozoal Agents Phase 1
46 Vaccines Phase 1
47 Liver Extracts Phase 1
48 pancreatin Phase 1
49 Cola Nutraceutical Phase 1

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 S9630, Medroxyprogesterone in Treating Women With Breast Cancer Completed NCT00002920 Phase 3 medroxyprogesterone;tamoxifen citrate
2 Docosahexaenoic Acid in Preventing Recurrence in Breast Cancer Survivors Unknown status NCT01849250 Phase 2 Docosahexaenoic Acid
3 Combination Chemotherapy With or Without Trastuzumab Followed By an Autologous Stem Cell Transplant and Radiation Therapy in Treating Patients With Stage III or Stage IV Breast Cancer Completed NCT00182793 Phase 2 carboplatin;cyclophosphamide;melphalan;thiotepa
4 Combination Chemotherapy With or Without Trastuzumab in Treating Patients With Stage II or Stage III Breast Cancer Active, not recruiting NCT00295893 Phase 2 carboplatin;cyclophosphamide;docetaxel;doxorubicin hydrochloride;paclitaxel
5 Vaccine Therapy With or Without Sargramostim in Treating Patients With Advanced or Metastatic Cancer Completed NCT00028496 Phase 1
6 Vaccine Therapy in Treating Patients With Advanced or Metastatic Cancer Completed NCT00027534 Phase 1
7 Biological Therapy in Treating Patients With Metastatic Cancer Completed NCT00004604 Phase 1
8 Partial Breast Radiation Therapy in Treating Women Undergoing Breast Conservation Therapy for Early-Stage Breast Cancer Unknown status NCT00599989

Search NIH Clinical Center for Mammary Paget's Disease

Inferred drug relations via UMLS 69 / NDF-RT 47 :


Genetic Tests for Mammary Paget's Disease

Anatomical Context for Mammary Paget's Disease

MalaCards organs/tissues related to Mammary Paget's Disease:

38
Breast, Bone, Skin, Liver

Publications for Mammary Paget's Disease

Articles related to Mammary Paget's Disease:

(show top 50) (show all 59)
# Title Authors Year
1
Clinical and Sonographic Features of Mammary Paget's Disease. ( 28695812 )
2017
2
Dermoscopy as a Supportive Instrument in the Early Recognition of Erosive Adenomatosis of the Nipple and Mammary Paget's Disease. ( 28566922 )
2017
3
Reflectance Confocal Microscopy for Diagnosis of Mammary Paget's Disease. ( 26976808 )
2016
4
Extra Mammary Paget's Disease of Vulva-a Case Report. ( 27872543 )
2016
5
Cytokeratin 7-negative mammary Paget's disease: A diagnostic pitfall. ( 26944832 )
2016
6
Double Rarities, Double Challenges: Extra-Mammary Paget's Disease and Anal Adenocarcinoma. ( 26233548 )
2015
7
Extra Mammary Paget's Disease: A Rare Case Report. ( 25657432 )
2015
8
Urothelial bladder carcinoma metastasising to the scrotum mimicking primary extra-mammary Paget's disease. ( 24614713 )
2014
9
Superficial sebaceous carcinoma on the areola clinically mimicking mammary Paget's disease. ( 23078184 )
2013
10
Pigmented mammary Paget's disease presenting with dermoscopic features of multiple dots. ( 23865386 )
2013
11
Minimally invasive mammary Paget's disease without an underlying breast carcinoma. ( 23851623 )
2013
12
A rare presentation of mammary Paget's disease involving the entire breast in the absence of any underlying ductal malignancy. ( 23760322 )
2013
13
An Unusual Case of Mammary Paget's Disease Diagnosed Using Dynamic Contrast-Enhanced MRI. ( 23607031 )
2013
14
Extra mammary Paget's disease of the vulva. ( 23372329 )
2012
15
Keratin Expression in Mammary Paget's Disease in situ with Intraductal Invasion. ( 23525171 )
2012
16
Characteristics of mammary Paget's disease in China: a national-wide multicenter retrospective study during 1999-2008. ( 22901142 )
2012
17
Pigmented mammary Paget's disease mimicking melanoma: report of three cases. ( 22064040 )
2012
18
Extra-mammary Paget's disease of the perianal region: a review of the literature emphasizing the operative management technique. ( 20884199 )
2011
19
A hyperpigmented plaque on the right nipple: a quiz. Pigmented mammary Paget's disease. ( 21369688 )
2011
20
A case report of pigmented mammary Paget's disease mimicking nevus of the nipple. ( 21213079 )
2011
21
Cystic fibrosis transmembrane conductance regulator is helpful in the distinction of extra-mammary Paget's disease from squamous cell carcinoma in situ (Bowen's disease). ( 21352263 )
2011
22
Effectiveness of photodynamic therapy for mammary and extra-mammary Paget's disease: a state of the science review. ( 21676258 )
2011
23
Mammary Paget's disease and extra-mammary Paget's disease: two morphologically similar but biologically different diseases. ( 19719834 )
2010
24
Pigmented mammary Paget's disease mimicking melanoma a further case in a man. ( 19470138 )
2009
25
Pigmented spots as a sign of mammary Paget's disease. ( 18627392 )
2009
26
Pigmented mammary Paget's disease: not a melanoma. ( 19413640 )
2009
27
Different panels of markers should be used to predict mammary Paget's disease associated with in situ or invasive ductal carcinoma of the breast. ( 19201736 )
2009
28
Extra-mammary Paget's disease of the scrotum and penis: a case report. ( 20184077 )
2009
29
Pigmented mammary Paget's disease presenting as an enlarged areola. ( 19601948 )
2009
30
Clinicopathologic evaluation of mammary Paget's disease. ( 19967013 )
2008
31
Immunohistochemical tracking of an immune response in mammary Paget's disease. ( 18842336 )
2008
32
Allergic contact dermatitis mimicking mammary paget's disease. ( 19882019 )
2008
33
Pigmented mammary Paget's disease. ( 17567331 )
2007
34
Acinar pattern of mammary Paget's disease: a case report. ( 17760678 )
2007
35
Extensive mammary Paget's disease. ( 17397359 )
2007
36
Quadruple extra-mammary Paget's disease. ( 17225023 )
2007
37
Cytokeratin 7 staining was useful in a case of pigmented mammary Paget's disease resembling malignant melanoma. ( 17040462 )
2006
38
Mammary Paget's disease occurring after mastectomy. ( 16899115 )
2006
39
Pigmented mammary Paget's disease mimicking melanoma. ( 15057051 )
2004
40
Pigmented mammary Paget's disease in a man. ( 15032027 )
2003
41
The ectopic expression of gastric mucin in extramammary and mammary Paget's disease. ( 11979091 )
2002
42
Mammary Paget's disease: evidence of diverse origin of the disease with a subgroup of Paget's disease developing from the superficial portion of lactiferous duct and a discontinuous pattern of tumor spread. ( 10594841 )
1999
43
Morphological evidence for field effect as a mechanism for tumour spread in mammary Paget's disease. ( 10583581 )
1999
44
Mammary Paget's disease with acantholytic features and without any detectable breast tumor. ( 10089077 )
1998
45
Underlying pathology in mammary Paget's disease. ( 9181226 )
1997
46
Mammary Paget's disease confined to the areola and associated with multifocal Toker cell hyperplasia. ( 8599455 )
1995
47
A case of mammary Paget's disease without an underlying carcinoma: microscopic analysis of the DNA content in Paget cells. ( 8014269 )
1994
48
Mammary Paget's disease and associated carcinoma. An immunohistochemical study. ( 7680194 )
1993
49
Stump the experts. Extra-mammary Paget's disease in the perianal region. ( 2157742 )
1990
50
Study of neu-protein expression in mammary Paget's disease with and without underlying breast carcinoma and in extramammary Paget's disease. ( 1701961 )
1990

Variations for Mammary Paget's Disease

Expression for Mammary Paget's Disease

Search GEO for disease gene expression data for Mammary Paget's Disease.

Pathways for Mammary Paget's Disease

GO Terms for Mammary Paget's Disease

Cellular components related to Mammary Paget's Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 cytoplasm GO:0005737 9.73 CALCA CFTR EGFR ERBB2 ESR1 KRT14
2 apical plasma membrane GO:0016324 9.62 CFTR EGFR ERBB2 MUC1
3 keratin filament GO:0045095 9.46 KRT14 KRT18 KRT7 KRT8
4 cell periphery GO:0071944 9.43 KRT14 KRT18 KRT8
5 intermediate filament GO:0005882 9.02 KRT14 KRT18 KRT20 KRT7 KRT8
6 nucleus GO:0005634 10.07 CALCA EGFR ERBB2 ESR1 KRT14 KRT18

Biological processes related to Mammary Paget's Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 keratinization GO:0031424 9.35 KRT14 KRT18 KRT20 KRT7 KRT8
2 positive regulation of blood vessel diameter GO:0097755 9.32 CALCA EGFR
3 negative regulation of ERBB signaling pathway GO:1901185 9.26 EGFR ERBB2
4 hepatocyte apoptotic process GO:0097284 9.16 KRT18 KRT8
5 cornification GO:0070268 9.02 KRT14 KRT18 KRT20 KRT7 KRT8

Molecular functions related to Mammary Paget's Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 protein binding GO:0005515 9.93 CALCA CFTR EGFR ERBB2 ESR1 KRT14
2 enzyme binding GO:0019899 9.56 CFTR EGFR ESR1 PGR
3 signal transducer, downstream of receptor, with protein tyrosine kinase activity GO:0004716 8.96 EGFR ERBB2
4 nitric-oxide synthase regulator activity GO:0030235 8.62 EGFR ESR1

Sources for Mammary Paget's Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
27 GO
28 GTR
29 HGMD
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 MedGen
41 MeSH
42 MESH via Orphanet
43 MGI
45 NCI
46 NCIt
47 NDF-RT
50 NINDS
51 Novoseek
53 OMIM
54 OMIM via Orphanet
58 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
Content
Loading form....