MCID: MMM001
MIFTS: 50

Mammary Paget's Disease

Categories: Rare diseases, Reproductive diseases, Cancer diseases

Aliases & Classifications for Mammary Paget's Disease

MalaCards integrated aliases for Mammary Paget's Disease:

Name: Mammary Paget's Disease 12 53 15
Paget's Disease of the Breast 12 76
Paget's Disease of the Nipple 53 59
Paget Disease of the Breast 53 59
Paget's Disease, Mammary 53 73
Mammary Paget Disease 12 59
Paget's Disease 12 73
Paget Disease of the Nipple 59
Pagets Disease Mammary 55
Paget Cell Neoplasm 12
Osteitis Deformans 73

Characteristics:

Orphanet epidemiological data:

59
paget disease of the nipple
Age of onset: Adult;

Classifications:



External Ids:

Disease Ontology 12 DOID:3443
NCIt 50 C7073
Orphanet 59 ORPHA180275
MESH via Orphanet 45 D010144
UMLS via Orphanet 74 C0030185 C1704323
ICD10 via Orphanet 34 C50.0
ICD10 33 M88.9

Summaries for Mammary Paget's Disease

NIH Rare Diseases : 53 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.

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 back pain, muscle cramp and sciatica. 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 Circadian entrainment. The drugs Aredia and Tamoxifen 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 : 76 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 250)
# Related Disease Score Top Affiliating Genes
1 paget disease, extramammary 31.9 ERBB2 KRT20 KRT7 MUC1
2 breast osteosarcoma 10.9 ERBB2 KRT8
3 breast intraductal proliferative lesion 10.9 EGFR ERBB2
4 neurenteric cyst 10.9 KRT18 KRT8
5 endolymphatic sac tumor 10.8 KRT7 KRT8
6 benign breast adenomyoepithelioma 10.8 EGFR KRT7
7 hidrocystoma 10.8 KRT7 KRT8
8 apocrine sweat gland neoplasm 10.8 KRT7 KRT8
9 nasal cavity cancer 10.8 KRT7 KRT8
10 malignant peritoneal mesothelioma 10.7 EGFR MUC1
11 epithelioid trophoblastic tumor 10.7 KRT18 KRT7
12 scirrhous adenocarcinoma 10.7 ERBB2 PGR
13 breast apocrine carcinoma 10.7 ERBB2 PGR
14 solid adenocarcinoma with mucin production 10.7 KRT7 MUC1
15 bronchiolo-alveolar adenocarcinoma 10.7 EGFR KRT7
16 breast metaplastic carcinoma 10.7 ERBB2 PGR
17 brooke-spiegler syndrome 10.7 KRT7 KRT8
18 parachordoma 10.7 KRT7 MUC1
19 bile duct mucoepidermoid carcinoma 10.7 KRT7 MUC1
20 cystic basal cell carcinoma 10.7 KRT20 KRT7
21 middle ear adenoma 10.7 KRT7 MUC1
22 hepatoid adenocarcinoma 10.7 EGFR MUC1
23 odontoma 10.7 KRT18 KRT8
24 vaginal benign neoplasm 10.7 KRT20 KRT7
25 seminal vesicle adenocarcinoma 10.7 KRT20 KRT7
26 transverse colon cancer 10.7 KRT20 KRT7
27 cryptogenic cirrhosis 10.7 KRT18 KRT8
28 nodular hidradenoma 10.7 KRT7 MUC1
29 appendix adenocarcinoma 10.7 KRT20 KRT7
30 ovarian large-cell neuroendocrine carcinoma 10.7 KRT20 KRT7
31 vaginal adenoma 10.7 KRT20 KRT7
32 vaginal tubulovillous adenoma 10.7 KRT20 KRT7
33 eyelid neoplasm 10.7 KRT20 KRT7
34 glandular cystitis 10.7 KRT20 KRT7
35 malignant syringoma 10.7 KRT20 KRT7
36 krukenberg carcinoma 10.7 KRT20 KRT7
37 large intestine adenocarcinoma 10.7 KRT20 KRT7
38 linitis plastica 10.6 KRT20 KRT7
39 lung acinar adenocarcinoma 10.6 KRT20 KRT7
40 small cell carcinoma of the bladder 10.6 KRT20 KRT7
41 pleomorphic liposarcoma 10.6 KRT8 MUC1
42 monophasic synovial sarcoma 10.6 KRT7 MUC1
43 adenoid squamous cell carcinoma 10.6 KRT20 KRT7
44 eccrine sweat gland neoplasm 10.6 KRT20 KRT7
45 nephrogenic adenoma 10.6 KRT7 MUC1
46 metanephric adenoma 10.6 KRT18 KRT7
47 adenomyoma 10.6 KRT7 MUC1
48 comedo carcinoma 10.6 ERBB2 ESR1
49 intratubular embryonal carcinoma 10.6 KRT20 KRT7
50 seminal vesicle tumor 10.6 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:


back pain, muscle cramp, sciatica

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

26
# 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 KRT7 KRT8 KRT18
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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 limbs/digits/tail MP:0005371 9.02 EGFR ERBB2 ESR1 PGR VCP

Drugs & Therapeutics for Mammary Paget's Disease

FDA approved drugs:

# Drug Name Active Ingredient(s) 18 Company Approval Date
1
Aredia 18 49 PAMIDRONATE DISODIUM Chiron August 1996

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

(show all 50)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Tamoxifen Approved Phase 3 10540-29-1 2733526
2
Medroxyprogesterone acetate Approved, Investigational Phase 3 71-58-9
3
Citric Acid Approved, Nutraceutical, Vet_approved Phase 3 77-92-9 311
4
Medroxyprogesterone Phase 3 520-85-4 10631
5 Selective Estrogen Receptor Modulators Phase 3
6 Contraceptive Agents Phase 3
7 Contraceptive Agents, Male Phase 3
8 Contraceptives, Oral Phase 3
9 Hormone Antagonists Phase 3
10 Hormones Phase 3
11 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
12 Estrogen Antagonists Phase 3
13 Estrogen Receptor Modulators Phase 3
14 Estrogens Phase 3
15 Bone Density Conservation Agents Phase 3
16 Antineoplastic Agents, Hormonal Phase 3
17 Citrate Nutraceutical Phase 3
18
Carboplatin Approved Phase 2 41575-94-4 10339178 498142 38904
19
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
20
Cyclophosphamide Approved, Investigational Phase 2 50-18-0, 6055-19-2 2907
21
Melphalan Approved Phase 2 148-82-3 4053 460612
22
Trastuzumab Approved, Investigational Phase 2 180288-69-1 9903
23
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
24
Triamcinolone Approved, Vet_approved Phase 2 124-94-7 31307
25
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
26
Docetaxel Approved, Investigational Phase 2 114977-28-5 148124
27
Doxil Approved June 1999 Phase 2 31703
28 Alkylating Agents Phase 2
29 Immunosuppressive Agents Phase 2
30 Antirheumatic Agents Phase 2
31 Antineoplastic Agents, Alkylating Phase 2
32 triamcinolone acetonide Phase 2
33 Albumin-Bound Paclitaxel Phase 2
34 Topoisomerase Inhibitors Phase 2
35 Triamcinolone diacetate Phase 2
36 Triamcinolone hexacetonide Phase 2
37 Anti-Bacterial Agents Phase 2
38 Antibiotics, Antitubercular Phase 2
39 Antimitotic Agents Phase 2
40 Antineoplastic Agents, Phytogenic Phase 2
41 Omega 3 Fatty Acid Nutraceutical Phase 2
42
Metronidazole Approved Phase 1 443-48-1 4173
43
Pancrelipase Approved, Investigational Phase 1 53608-75-6
44 pancreatin Phase 1
45 Vaccines Phase 1
46 Anti-Infective Agents Phase 1
47 Antiparasitic Agents Phase 1
48 Liver Extracts Phase 1
49 Antiprotozoal Agents Phase 1
50 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 Not Applicable

Search NIH Clinical Center for Mammary Paget's Disease

Inferred drug relations via UMLS 73 / NDF-RT 51 :


Genetic Tests for Mammary Paget's Disease

Anatomical Context for Mammary Paget's Disease

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

41
Breast, Bone, Skin, Liver

Publications for Mammary Paget's Disease

Articles related to Mammary Paget's Disease:

(show top 50) (show all 63)
# Title Authors Year
1
Pigmented mammary Paget's disease without underlying carcinoma. ( 29797528 )
2018
2
Dermoscopy as a Supportive Instrument in the Early Recognition of Erosive Adenomatosis of the Nipple and Mammary Paget's Disease. ( 28566922 )
2017
3
Clinical and Sonographic Features of Mammary Paget's Disease. ( 28695812 )
2017
4
Total vaginectomy and radical vulvectomy for extension of extra-mammary Paget's disease. ( 29943937 )
2016
5
Cytokeratin 7-negative mammary Paget's disease: A diagnostic pitfall. ( 26944832 )
2016
6
Reflectance Confocal Microscopy for Diagnosis of Mammary Paget's Disease. ( 26976808 )
2016
7
Extra Mammary Paget's Disease of Vulva-a Case Report. ( 27872543 )
2016
8
Extra Mammary Paget's Disease: A Rare Case Report. ( 25657432 )
2015
9
Double Rarities, Double Challenges: Extra-Mammary Paget's Disease and Anal Adenocarcinoma. ( 26233548 )
2015
10
Urothelial bladder carcinoma metastasising to the scrotum mimicking primary extra-mammary Paget's disease. ( 24614713 )
2014
11
Superficial sebaceous carcinoma on the areola clinically mimicking mammary Paget's disease. ( 23078184 )
2013
12
Minimally invasive mammary Paget's disease without an underlying breast carcinoma. ( 23851623 )
2013
13
Pigmented mammary Paget's disease presenting with dermoscopic features of multiple dots. ( 23865386 )
2013
14
An Unusual Case of Mammary Paget's Disease Diagnosed Using Dynamic Contrast-Enhanced MRI. ( 23607031 )
2013
15
A rare presentation of mammary Paget's disease involving the entire breast in the absence of any underlying ductal malignancy. ( 23760322 )
2013
16
Extra mammary Paget's disease of the vulva. ( 23372329 )
2012
17
Pigmented mammary Paget's disease mimicking melanoma: report of three cases. ( 22064040 )
2012
18
Keratin Expression in Mammary Paget's Disease in situ with Intraductal Invasion. ( 23525171 )
2012
19
Characteristics of mammary Paget's disease in China: a national-wide multicenter retrospective study during 1999-2008. ( 22901142 )
2012
20
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
21
Extra-mammary Paget's disease of the perianal region: a review of the literature emphasizing the operative management technique. ( 20884199 )
2011
22
A hyperpigmented plaque on the right nipple: a quiz. Pigmented mammary Paget's disease. ( 21369688 )
2011
23
Effectiveness of photodynamic therapy for mammary and extra-mammary Paget's disease: a state of the science review. ( 21676258 )
2011
24
A case report of pigmented mammary Paget's disease mimicking nevus of the nipple. ( 21213079 )
2011
25
Mammary Paget's disease and extra-mammary Paget's disease: two morphologically similar but biologically different diseases. ( 19719834 )
2010
26
Pigmented mammary Paget's disease: not a melanoma. ( 19413640 )
2009
27
Pigmented spots as a sign of mammary Paget's disease. ( 18627392 )
2009
28
Pigmented mammary Paget's disease mimicking melanoma a further case in a man. ( 19470138 )
2009
29
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
30
Pigmented mammary Paget's disease presenting as an enlarged areola. ( 19601948 )
2009
31
[Extra-mammary Paget's disease of the scrotum]. ( 20051181 )
2009
32
Extra-mammary Paget's disease of the scrotum and penis: a case report. ( 20184077 )
2009
33
Allergic contact dermatitis mimicking mammary paget's disease. ( 19882019 )
2008
34
Clinicopathologic evaluation of mammary Paget's disease. ( 19967013 )
2008
35
Immunohistochemical tracking of an immune response in mammary Paget's disease. ( 18842336 )
2008
36
Pigmented mammary Paget's disease. ( 17567331 )
2007
37
Quadruple extra-mammary Paget's disease. ( 17225023 )
2007
38
Extensive mammary Paget's disease. ( 17397359 )
2007
39
Acinar pattern of mammary Paget's disease: a case report. ( 17760678 )
2007
40
Cytokeratin 7 staining was useful in a case of pigmented mammary Paget's disease resembling malignant melanoma. ( 17040462 )
2006
41
Mammary Paget's disease occurring after mastectomy. ( 16899115 )
2006
42
Pigmented mammary Paget's disease mimicking melanoma. ( 15057051 )
2004
43
Pigmented mammary Paget's disease in a man. ( 15032027 )
2003
44
The ectopic expression of gastric mucin in extramammary and mammary Paget's disease. ( 11979091 )
2002
45
Morphological evidence for field effect as a mechanism for tumour spread in mammary Paget's disease. ( 10583581 )
1999
46
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
47
Mammary Paget's disease with acantholytic features and without any detectable breast tumor. ( 10089077 )
1998
48
Underlying pathology in mammary Paget's disease. ( 9181226 )
1997
49
Human papilloma virus DNA: a factor in the pathogenesis of mammary Paget's disease? ( 8932876 )
1996
50
Mammary Paget's disease confined to the areola and associated with multifocal Toker cell hyperplasia. ( 8599455 )
1995

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

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

# Super pathways Score Top Affiliating Genes
1
Show member pathways
13.09 EGFR ERBB2 KRT18 KRT20 KRT7 KRT8
2
Show member pathways
12.59 EGFR ESR1 KRT18 KRT20 PGR
3
Show member pathways
12.4 EGFR ERBB2 ESR1 PGR
4
Show member pathways
11.91 KRT18 KRT20 KRT7 KRT8
5 11.59 KRT18 KRT20 KRT7 KRT8 MUC1 VCP
6
Show member pathways
11.58 KRT18 KRT7 KRT8
7
Show member pathways
11.4 EGFR ERBB2 ESR1
8
Show member pathways
11.29 EGFR ERBB2 ESR1 PGR

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 cytosol GO:0005829 9.97 ERBB2 ESR1 KRT18 KRT20 KRT7 KRT8
2 nucleus GO:0005634 9.9 CALCA EGFR ERBB2 ESR1 KRT18 KRT7
3 keratin filament GO:0045095 9.43 KRT18 KRT7 KRT8
4 cytoplasm GO:0005737 9.4 CALCA EGFR ERBB2 ESR1 KRT18 KRT20
5 intermediate filament GO:0005882 9.26 KRT18 KRT20 KRT7 KRT8

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

# Name GO ID Score Top Affiliating Genes
1 positive regulation of protein phosphorylation GO:0001934 9.58 EGFR ERBB2 SQSTM1
2 positive regulation of protein kinase B signaling GO:0051897 9.54 EGFR ERBB2 ESR1
3 positive regulation of blood vessel diameter GO:0097755 9.43 CALCA EGFR
4 phosphatidylinositol phosphorylation GO:0046854 9.43 EGFR ERBB2 ESR1
5 negative regulation of ERBB signaling pathway GO:1901185 9.32 EGFR ERBB2
6 keratinization GO:0031424 9.26 KRT18 KRT20 KRT7 KRT8
7 hepatocyte apoptotic process GO:0097284 9.16 KRT18 KRT8
8 cornification GO:0070268 8.92 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 enzyme binding GO:0019899 9.67 EGFR ESR1 PGR SQSTM1
2 protein phosphatase binding GO:0019903 9.43 EGFR ERBB2 VCP
3 phosphatidylinositol-4,5-bisphosphate 3-kinase activity GO:0046934 9.33 EGFR ERBB2 ESR1
4 obsolete signal transducer, downstream of receptor, with protein tyrosine kinase activity GO:0004716 9.32 EGFR ERBB2
5 identical protein binding GO:0042802 9.17 CALCA EGFR ERBB2 ESR1 PGR SQSTM1
6 nitric-oxide synthase regulator activity GO:0030235 8.96 EGFR ESR1
7 protein binding GO:0005515 10.18 CALCA EGFR ERBB2 ESR1 KRT18 KRT20

Sources for Mammary Paget's Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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