MCID: MMM001
MIFTS: 55

Mammary Paget's Disease

Categories: Cancer diseases, Rare diseases, Reproductive diseases, Skin 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 12 15 73
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 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, is related to paget disease, extramammary and in situ carcinoma, and has symptoms including back pain, sciatica and muscle cramp. An important gene associated with Mammary Paget's Disease is ERBB2 (Erb-B2 Receptor Tyrosine Kinase 2), and among its related pathways/superpathways are Gastric cancer and Cytoskeletal Signaling. The drugs Medroxyprogesterone acetate and Tamoxifen have been mentioned in the context of this disorder. Affiliated tissues include bone, breast and skin, and related phenotypes are cardiovascular system and integument

Disease Ontology : 12 A breast adenocarcinoma that has material basis in epidermal Paget cells, which are malignant glandular epithelial cells with abundant and clear cytoplasm, usually containing mucin, and pleomorphic and hyperchromatic nucleus.

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 240)
# Related Disease Score Top Affiliating Genes
1 paget disease, extramammary 32.1 ERBB2 KRT20 KRT7 MUC1
2 in situ carcinoma 29.7 EGFR ERBB2 ESR1 PGR
3 acneiform dermatitis 10.3 EGFR ERBB2
4 breast squamous cell carcinoma 10.3 EGFR ERBB2
5 brooke-spiegler syndrome 10.3 KRT7 KRT8
6 prostate squamous cell carcinoma 10.3 KRT7 KRT8
7 nasal cavity cancer 10.3 KRT7 KRT8
8 atypical choroid plexus papilloma 10.3 KRT7 KRT8
9 hidrocystoma 10.3 KRT7 KRT8
10 cutaneous adenocystic carcinoma 10.3 KRT7 KRT8
11 nasal cavity squamous cell carcinoma 10.3 KRT7 KRT8
12 apocrine sweat gland neoplasm 10.3 KRT7 KRT8
13 hepatoid adenocarcinoma 10.3 EGFR MUC1
14 pleomorphic liposarcoma 10.3 KRT8 MUC1
15 chronic orbital inflammation 10.3 CTLA4 MUC1
16 malignant peritoneal mesothelioma 10.3 EGFR MUC1
17 breast osteosarcoma 10.2 ERBB2 KRT8
18 nodular hidradenoma 10.2 KRT7 MUC1
19 adenomyoma 10.2 KRT7 MUC1
20 parachordoma 10.2 KRT7 MUC1
21 mucinous ovarian cystadenoma 10.2 KRT7 MUC1
22 breast intraductal proliferative lesion 10.2 EGFR ERBB2
23 hidradenoma 10.2 KRT7 MUC1
24 duodenal disease 10.2 KRT7 MUC1
25 spindle cell sarcoma 10.2 KRT7 MUC1
26 secretory meningioma 10.2 KRT7 MUC1
27 bile duct mucoepidermoid carcinoma 10.2 KRT7 MUC1
28 uterine body mixed cancer 10.2 ERBB2 PGR
29 middle ear adenoma 10.2 KRT7 MUC1
30 lipoadenoma 10.2 KRT7 MUC1
31 monophasic synovial sarcoma 10.2 KRT7 MUC1
32 well-differentiated liposarcoma 10.2 KRT7 MUC1
33 large intestine adenocarcinoma 10.2 KRT20 KRT7
34 jejunal adenocarcinoma 10.2 KRT20 KRT7
35 endocervical adenocarcinoma 10.2 KRT20 KRT7
36 bladder lymphoma 10.2 KRT20 KRT7
37 chronic cystitis 10.2 KRT20 KRT7
38 eccrine sweat gland neoplasm 10.2 KRT20 KRT7
39 rectum adenocarcinoma 10.2 KRT20 KRT7
40 ovarian mucinous adenocarcinoma 10.2 KRT20 KRT7
41 bladder adenocarcinoma 10.2 KRT20 KRT7
42 linitis plastica 10.2 KRT20 KRT7
43 infiltrative basal cell carcinoma 10.2 KRT20 KRT7
44 medulloepithelioma 10.2 KRT20 KRT7
45 renal pelvis carcinoma 10.2 KRT20 KRT7
46 thymus adenocarcinoma 10.2 KRT20 KRT7
47 appendix disease 10.2 KRT20 KRT7
48 ovarian mucinous neoplasm 10.2 KRT20 KRT7
49 gastric diffuse adenocarcinoma 10.2 KRT20 KRT7
50 lung acinar adenocarcinoma 10.2 KRT20 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, sciatica, muscle cramp

MGI Mouse Phenotypes related to Mammary Paget's Disease:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 cardiovascular system MP:0005385 9.8 CTLA4 EGFR ERBB2 ESR1 KRT8 PGR
2 integument MP:0010771 9.7 CTLA4 EGFR ERBB2 ESR1 PGR SQSTM1
3 limbs/digits/tail MP:0005371 9.35 EGFR ERBB2 ESR1 PGR VCP
4 skeleton MP:0005390 9.17 CTLA4 EGFR ERBB2 ESR1 PGR SQSTM1

Drugs & Therapeutics for Mammary Paget's Disease

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

(show top 50) (show all 54)
# 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 Estrogen Antagonists Phase 3
5 Estrogen Receptor Modulators Phase 3
6 Contraceptive Agents, Male Phase 3
7 Contraceptive Agents Phase 3
8 Antineoplastic Agents, Hormonal Phase 3
9 Selective Estrogen Receptor Modulators Phase 3
10 Estrogens Phase 3
11 Contraceptives, Oral Phase 3
12 Hormone Antagonists Phase 3
13 Hormones Phase 3
14 Citrate Phase 3
15 Bone Density Conservation Agents Phase 3
16
Medroxyprogesterone Phase 3 520-85-4 10631
17 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 3
18 Estrogen Receptor Antagonists Phase 3
19
Cyclophosphamide Approved, Investigational Phase 2 6055-19-2, 50-18-0 2907
20
Trastuzumab Approved, Investigational Phase 2 180288-69-1 9903
21
Thiotepa Approved, Investigational Phase 2 52-24-4 5453
22
Melphalan Approved Phase 2 148-82-3 4053 460612
23
Carboplatin Approved Phase 2 41575-94-4 10339178 38904 498142
24
Triamcinolone Approved, Vet_approved Phase 2 124-94-7 31307
25
Paclitaxel Approved, Vet_approved Phase 2 33069-62-4 36314
26
Doxorubicin Approved, Investigational Phase 2 23214-92-8 31703
27
Docetaxel Approved, Investigational Phase 2 114977-28-5 148124
28
Doxil Approved June 1999 Phase 2 31703
29 Omega 3 Fatty Acid Phase 2
30 Immunosuppressive Agents Phase 2
31 Alkylating Agents Phase 2
32 Immunologic Factors Phase 2,Phase 1
33 Antineoplastic Agents, Alkylating Phase 2
34 Antirheumatic Agents Phase 2
35 Triamcinolone diacetate Phase 2
36 Antibiotics, Antitubercular Phase 2
37 Antineoplastic Agents, Phytogenic Phase 2
38 Triamcinolone hexacetonide Phase 2
39 Albumin-Bound Paclitaxel Phase 2
40 Anti-Bacterial Agents Phase 2
41 Antimitotic Agents Phase 2
42 Topoisomerase Inhibitors Phase 2
43 triamcinolone acetonide Phase 2
44
Metronidazole Approved Phase 1 443-48-1 4173
45
Pancrelipase Approved, Investigational Phase 1 53608-75-6
46 Vaccines Phase 1
47 Antiparasitic Agents Phase 1
48 Antiprotozoal Agents Phase 1
49 Anti-Infective Agents Phase 1
50 Liver Extracts 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
9 PAGETEX® Photodynamic Therapy Device for the Treatment of Extra Mammary Paget's Disease of the Vulva (EMPV). Not yet recruiting NCT03713203 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
Bone, Breast, Skin, Lymph Node, Prostate, Lung, Liver

Publications for Mammary Paget's Disease

Articles related to Mammary Paget's Disease:

(show top 50) (show all 688)
# Title Authors Year
1
Extramammary Paget's Disease. ( 30497678 )
2019
2
Pigmented mammary Paget's disease without underlying carcinoma. ( 29797528 )
2018
3
A rare case of Chiari malformation secondary to Paget's disease of skull. ( 30233039 )
2018
4
Gigantic mammary Paget's disease of a very elderly woman. ( 30446858 )
2018
5
Mammary Paget's Disease of the Nipple: Relatively Common but Still Unknown to Many. ( 30486607 )
2018
6
Prognostic significance of mammary Paget's disease in Chinese women: a 10-year, population-based, matched cohort study. ( 30538501 )
2018
7
Paget's Disease of Bone: An Entity Still Exists in India. ( 30090729 )
2018
8
A microscopic evaluation of Paget's disease of bone from a Byzantine monastic crypt in Jordan. ( 30154045 )
2018
9
Increased frequency of impaired fasting glucose and isolated systolic hypertension in Paget's disease of bone. ( 30284104 )
2018
10
Total knee arthroplasty in patients with Paget's disease of bone: A systematic review. ( 30364786 )
2018
11
Long-term control of Paget's disease of bone with low-dose, once-weekly, oral bisphosphonate preparations, in a "real world" setting. ( 30406885 )
2018
12
Paget's disease of bone. ( 30449547 )
2018
13
Extramammary invasive Paget's disease and apocrine angiomatous hamartoma: an unusual association. ( 30514671 )
2018
14
Bladder Carcinoma Presenting as Paget's Disease of Vulva: An Uncommon Entity. ( 30504985 )
2018
15
Primary Cutaneous Mucinous Carcinoma with Extramammary Paget's Disease: Eccrine or Apocrine? ( 29366305 )
2018
16
Efficacy of low-dose 5-fluorouracil/cisplatin therapy for invasive extramammary Paget's disease. ( 29446148 )
2018
17
Pigmented Paget's disease of the nipple mistaken for melanoma in situ: a diagnostic pitfall for the unwary. ( 29490872 )
2018
18
Acquired resistance to pamidronate treated effectively with zoledronate in juvenile Paget's disease. ( 29502293 )
2018
19
Metastatic Extramammary Paget's Disease: Pathogenesis and Novel Therapeutic Approach. ( 29503810 )
2018
20
Extramammary Paget's disease of the scrotum with complete response to imiquimod and photodynamic therapy. ( 29507011 )
2018
21
Intraoperative Immunostaining for Cytokeratin-7 During Mohs Micrographic Surgery Demonstrates Low Local Recurrence Rates in Extramammary Paget's Disease. ( 29517496 )
2018
22
Successful treatment of metastatic extramammary Paget's disease with pemetrexed monotherapy systematically and 5-fluorouracil topically. ( 29536970 )
2018
23
Esophageal Paget's Disease Secondary to Hypopharyngeal Carcinoma: a Case Report. ( 29542074 )
2018
24
p62/sequestosome 1 deficiency accelerates osteoclastogenesis in vitro and leads to Paget's disease-like bone phenotypes in mice. ( 29555685 )
2018
25
Corrigendum: Metastatic Extramammary Paget's Disease: Pathogenesis and Novel Therapeutic Approach. ( 29569629 )
2018
26
Successful treatment of extramammary Paget's disease with S-1 and docetaxel combination chemotherapy. ( 29572930 )
2018
27
Complete response of extramammary Paget's disease with imiquimod and PDT: Report of two cases. ( 29575126 )
2018
28
The diagnosis and management of extramammary Paget's disease. ( 29575944 )
2018
29
Pagetoid Bowen Disease Initially Misdiagnosed as Ectopic Extramammary Paget's Disease. ( 29606821 )
2018
30
Transcriptome analyses reveal FOXA1 dysregulation in mammary and extramammary Paget's disease. ( 29630912 )
2018
31
Serum Carcinoembryonic Antigen Level as a Marker for Advanced Stage and Chemotherapeutic Response in Extramammary Paget's Disease. ( 29691588 )
2018
32
Paget's disease of the male breast: case report and a point of view from actual literature. ( 29694313 )
2018
33
Perianal Extramammary Paget's Disease: More Than Meets the Eye. ( 29696480 )
2018
34
Practical consensus recommendatons for Paget's disease in breast cancer. ( 29721469 )
2018
35
Surgical Treatment for Extramammary Paget's Disease. ( 29725859 )
2018
36
The lucent (CT) and enhancing (MR) rim, a sign of Paget's disease of the skull: case report. ( 29730701 )
2018
37
Paget's disease of jaw bones as primary manifestation: A case report of a proper diagnosis made by general dentist. ( 29733536 )
2018
38
Assessment of the methods used to detect HER2-positive advanced extramammary Paget's disease. ( 29744813 )
2018
39
P16 Expression in Extramammary Paget's Disease of the Vulva and Scrotum Is Not Human Papillomavirus Related. ( 29745285 )
2018
40
A review of non-invasive imaging in extramammary Paget's disease. ( 29763511 )
2018
41
Axillary extramammary Paget's disease on lymphadenectomy scar for melanoma: an instance of immunocompromised district. ( 29766708 )
2018
42
Hepatosplenomegaly, pneumopathy, bone changes and fronto-temporal dementia: Niemann-Pick type B and SQSTM1-associated Paget's disease in the same individual. ( 29948344 )
2018
43
Depigmented extramammary Paget's disease without histological dermal invasion identified by multiple inguinal and pelvic lymph node metastases. ( 29952291 )
2018
44
Efficacy and tolerance of photodynamic therapy for vulvar Paget's disease: a multicentric retrospective study. ( 29976540 )
2018
45
Inguinal and scrotal extramammary Paget's disease: 18F-FDG PET/CT imaging. ( 29991846 )
2018
46
Paget's disease derived in situ from reserve cell hyperplasia, squamous metaplasia, and squamous cell carcinoma of the esophagogastric junction: a case report. ( 30046968 )
2018
47
Prognostic Factors of Extramammary Paget's Disease. ( 30112691 )
2018
48
Primary extramammary Paget's disease of the skin: treatment and survival. ( 30133752 )
2018
49
Zoledronic acid prevents pagetic-like lesions and accelerated bone loss in the p62P394L mouse model of Paget's disease. ( 30154079 )
2018
50
Monitoring serum cytokeratin 19 fragment 21-1 to determine the efficacy of docetaxel chemotherapy in advanced extramammary Paget's disease. ( 30194782 )
2018

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 nucleus GO:0005634 9.65 CALCA EGFR ERBB2 ESR1 KRT7 KRT8
2 cytoplasm GO:0005737 9.36 CALCA EGFR ERBB2 ESR1 KRT20 KRT7

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 9.54 EGFR ERBB2 SQSTM1
2 positive regulation of protein kinase B signaling GO:0051897 9.5 EGFR ERBB2 ESR1
3 regulation of ERK1 and ERK2 cascade GO:0070372 9.4 EGFR ERBB2
4 phosphatidylinositol phosphorylation GO:0046854 9.33 EGFR ERBB2 ESR1
5 positive regulation of blood vessel diameter GO:0097755 9.26 CALCA EGFR
6 cornification GO:0070268 9.13 KRT20 KRT7 KRT8
7 negative regulation of ERBB signaling pathway GO:1901185 8.62 EGFR ERBB2

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 CTLA4 EGFR ERBB2 ESR1 KRT20
2 enzyme binding GO:0019899 9.67 EGFR ESR1 PGR SQSTM1
3 protein phosphatase binding GO:0019903 9.43 EGFR ERBB2 VCP
4 nitric-oxide synthase regulator activity GO:0030235 9.32 EGFR ESR1
5 identical protein binding GO:0042802 9.17 CALCA EGFR ERBB2 ESR1 PGR SQSTM1
6 phosphatidylinositol-4,5-bisphosphate 3-kinase activity GO:0046934 9.13 EGFR ERBB2 ESR1

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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