MCID: MLG049
MIFTS: 32

Malignant Syringoma

Categories: Rare diseases, Skin diseases, Cancer diseases

Aliases & Classifications for Malignant Syringoma

MalaCards integrated aliases for Malignant Syringoma:

Name: Malignant Syringoma 12 15
Microcystic Adnexal Carcinoma 12 53
Microcystic Adnexal Carcinoma of Skin 12
Syringomatous Carcinoma 12
Eccrine Epithelioma 73

Classifications:



External Ids:

Disease Ontology 12 DOID:5569
UMLS 73 C0346027

Summaries for Malignant Syringoma

NIH Rare Diseases : 53 Microcystic adnexal carcinoma is a rare tumor of the skin that most often develops in the head and neck region, particularly in the middle of the face, though it may occur in the skin of other parts of the body as well. The average age of diagnosis is 56. This tumor is often first noticed as a bump or yellowish spot in the skin.  Though microcystic adnexal carcinomas frequently grow into and disturb nearby tissues and is therefore considered an invasive cancer, this type of tumor rarely spreads to more distant parts of the body (metastasizes). The main treatment for microcystic adnexal carcinoma is Mohs micrographic surgery, which is thought to improve the chances that all of the tumor cells are removed during surgery.

MalaCards based summary : Malignant Syringoma, also known as microcystic adnexal carcinoma, is related to nodular basal cell carcinoma and rete testis adenocarcinoma. An important gene associated with Malignant Syringoma is DAND5 (DAN Domain BMP Antagonist Family Member 5), and among its related pathways/superpathways are Developmental Biology and Cytoskeletal Signaling. The drugs nivolumab and Antibodies have been mentioned in the context of this disorder. Affiliated tissues include skin and bone.

Related Diseases for Malignant Syringoma

Diseases in the Syringoma family:

Syringomas, Multiple Malignant Syringoma

Diseases related to Malignant Syringoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 105)
# Related Disease Score Top Affiliating Genes
1 nodular basal cell carcinoma 10.4 KRT15 KRT17
2 rete testis adenocarcinoma 10.1 KRT20 PTGER4
3 rete testis neoplasm 10.1 KRT20 PTGER4
4 hidrocystoma 10.0 KRT15 KRT7
5 apocrine sweat gland neoplasm 10.0 KRT15 KRT7
6 anus basaloid carcinoma 10.0 KRT7 PTGER4
7 clear cell basal cell carcinoma 10.0 KRT17 KRT7
8 endosalpingiosis 9.9 KRT7 PTGER4
9 actinic keratosis 9.9 KRT17 KRT20
10 syringoma 9.9
11 nodular hidradenoma 9.9 KRT7 PTGER4
12 bowen's disease 9.9 KRT17 KRT7
13 basaloid squamous cell carcinoma 9.8 KRT17 KRT7
14 pericardium cancer 9.8 KRT7 PTGER4
15 vaginal benign neoplasm 9.8 KRT20 KRT7
16 seminal vesicle adenocarcinoma 9.8 KRT20 KRT7
17 transverse colon cancer 9.8 KRT20 KRT7
18 appendix adenocarcinoma 9.8 KRT20 KRT7
19 ovarian large-cell neuroendocrine carcinoma 9.8 KRT20 KRT7
20 vaginal adenoma 9.8 KRT20 KRT7
21 vaginal tubulovillous adenoma 9.8 KRT20 KRT7
22 eyelid neoplasm 9.8 KRT20 KRT7
23 glandular cystitis 9.8 KRT20 KRT7
24 krukenberg carcinoma 9.8 KRT20 KRT7
25 large intestine adenocarcinoma 9.8 KRT20 KRT7
26 linitis plastica 9.8 KRT20 KRT7
27 lung acinar adenocarcinoma 9.8 KRT20 KRT7
28 small cell carcinoma of the bladder 9.8 KRT20 KRT7
29 adenoid squamous cell carcinoma 9.8 KRT20 KRT7
30 mucinous bronchioloalveolar adenocarcinoma 9.8 KRT20 KRT7
31 vulval paget's disease 9.8 KRT20 KRT7
32 bile duct cystadenocarcinoma 9.8 KRT20 KRT7
33 intratubular embryonal carcinoma 9.8 KRT20 KRT7
34 ovarian mucinous adenocarcinoma 9.8 KRT20 KRT7
35 gastric diffuse adenocarcinoma 9.8 KRT20 KRT7
36 meibomian cyst 9.8 KRT20 KRT7
37 bladder lymphoma 9.8 KRT20 KRT7
38 bladder adenocarcinoma 9.8 KRT20 KRT7
39 jejunal adenocarcinoma 9.8 KRT20 KRT7
40 chronic cystitis 9.8 KRT20 KRT7
41 vulva adenocarcinoma 9.8 KRT20 KRT7
42 medulloepithelioma 9.8 KRT20 KRT7
43 glycogen-rich clear cell breast carcinoma 9.8 KRT20 KRT7
44 rectum adenocarcinoma 9.8 KRT20 KRT7
45 renal pelvis carcinoma 9.8 KRT20 KRT7
46 paget disease, extramammary 9.8 KRT20 KRT7
47 pericardial mesothelioma 9.8 KRT7 PTGER4
48 appendix cancer 9.8 KRT20 KRT7
49 mucinous cystadenocarcinoma 9.8 KRT20 KRT7
50 biliary papillomatosis 9.8 KRT20 KRT7

Graphical network of the top 20 diseases related to Malignant Syringoma:



Diseases related to Malignant Syringoma

Symptoms & Phenotypes for Malignant Syringoma

Drugs & Therapeutics for Malignant Syringoma

Drugs for Malignant Syringoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
nivolumab Approved Phase 2 946414-94-4
2 Antibodies Phase 2
3 Immunoglobulins Phase 2
4 Antibodies, Monoclonal Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Talimogene Laherparepvec and Nivolumab in Treating Patients With Refractory Lymphomas or Advanced or Refractory Non-melanoma Skin Cancers Recruiting NCT02978625 Phase 2

Search NIH Clinical Center for Malignant Syringoma

Genetic Tests for Malignant Syringoma

Anatomical Context for Malignant Syringoma

MalaCards organs/tissues related to Malignant Syringoma:

41
Skin, Bone

Publications for Malignant Syringoma

Articles related to Malignant Syringoma:

(show all 19)
# Title Authors Year
1
Metastatic Microcystic Adnexal Carcinoma with DNA Sequencing Results and Response to Systemic Antineoplastic Chemotherapy. ( 28870941 )
2017
2
Misdiagnosed microcystic adnexal carcinoma on the lateral forehead and challenges in reconstruction of a large and bone-deep defect. ( 26713651 )
2016
3
An important mimicker: plaque-type syringoma mistakenly diagnosed as microcystic adnexal carcinoma. ( 25111356 )
2014
4
Expression of p75 neurotrophin receptor in desmoplastic trichoepithelioma, infiltrative basal cell carcinoma, and microcystic adnexal carcinoma. ( 23598854 )
2013
5
The immunohistochemical differential diagnosis of microcystic adnexal carcinoma, desmoplastic trichoepithelioma and morpheaform basal cell carcinoma using BerEP4 and stem cell markers. ( 23398472 )
2013
6
Sweat duct proliferation associated with aggregates of elastic tissue and atrophodermia vermiculata: a simulator of microcystic adnexal carcinoma. Report of two cases. ( 20175822 )
2010
7
Supraorbital neuroma masquerading as local recurrence from a previously excised microcystic adnexal carcinoma. ( 19648071 )
2010
8
Microcystic adnexal carcinoma: an immunohistochemical reappraisal. ( 18065959 )
2008
9
Plaque-type syringoma: two cases misdiagnosed as microcystic adnexal carcinoma. ( 18005172 )
2008
10
A microcystic adnexal carcinoma in the auditory canal 15 years after radiotherapy of a 12-year-old boy with nasopharynx carcinoma. ( 15925985 )
2005
11
Microcystic adnexal carcinoma: an immunohistochemical study including markers of proliferation and apoptosis. ( 11257620 )
2001
12
Sclerosing sweat-duct carcinoma (malignant syringoma) of the upper eyelid: a patient report with immunohistochemical and ultrastructural analysis. ( 9842565 )
1998
13
Eyelid microcystic adnexal carcinoma. ( 7710401 )
1995
14
Solitary syringoma. Report of five cases and clinicopathologic comparison with microcystic adnexal carcinoma of the skin. ( 8599451 )
1995
15
Two perplexing skin tumors: microcystic adnexal carcinoma and keratoacanthoma. ( 7944178 )
1994
16
Combined microcystic adnexal carcinoma and squamous cell carcinoma arising in the ovarian cystic teratoma--a brief case report. ( 7702793 )
1994
17
Microcystic adnexal carcinoma: a case report with immunohistochemical and electron microscopical examinations. ( 1593021 )
1992
18
Microcystic adnexal carcinoma. An immunohistochemical comparison with other cutaneous appendage tumors. ( 1689137 )
1990
19
Malignant syringoma of the eyelid. ( 6493709 )
1984

Variations for Malignant Syringoma

Cosmic variations for Malignant Syringoma:

9
(show top 50) (show all 361)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM44097 TP53 skin,face,carcinoma,NS c.530C>T p.P177L 17:7675082-7675082 8
2 COSM10728 TP53 skin,face,carcinoma,NS c.839G>A p.R280K 17:7673781-7673781 8
3 COSM10988 TP53 skin,face,carcinoma,NS c.772G>A p.E258K 17:7674191-7674191 8
4 COSM10705 TP53 skin,face,carcinoma,NS c.586C>T p.R196* 17:7674945-7674945 8
5 COSM44295 TP53 skin,face,carcinoma,NS c.993+1G>A p.? 17:7673534-7673534 8
6 COSM10939 TP53 skin,face,carcinoma,NS c.832C>T p.P278S 17:7673788-7673788 8
7 COSM43544 TP53 skin,face,carcinoma,NS c.260C>A p.P87Q 17:7676109-7676109 8
8 COSM44326 TP53 skin,face,carcinoma,NS c.706T>C p.Y236H 17:7674257-7674257 8
9 COSM10662 TP53 skin,face,carcinoma,NS c.743G>A p.R248Q 17:7674220-7674220 8
10 COSM11449 TP53 skin,face,carcinoma,NS c.388C>T p.L130F 17:7675224-7675224 8
11 COSM44068 TP53 skin,face,carcinoma,NS c.532C>A p.H178N 17:7675080-7675080 8
12 COSM43582 TP53 skin,face,carcinoma,NS c.454C>T p.P152S 17:7675158-7675158 8
13 COSM10654 TP53 skin,face,carcinoma,NS c.637C>T p.R213* 17:7674894-7674894 8
14 COSM10656 TP53 skin,face,carcinoma,NS c.742C>T p.R248W 17:7674221-7674221 8
15 COSM44552 TP53 skin,face,carcinoma,NS c.509C>T p.T170M 17:7675103-7675103 8
16 COSM45830 TP53 skin,face,carcinoma,NS c.766A>C p.T256P 17:7674197-7674197 8
17 COSM10812 TP53 skin,face,carcinoma,NS c.722C>T p.S241F 17:7674241-7674241 8
18 COSM10726 TP53 skin,face,carcinoma,NS c.856G>A p.E286K 17:7673764-7673764 8
19 COSM10992 TP53 skin,face,carcinoma,NS c.844C>G p.R282G 17:7673776-7673776 8
20 COSM45304 TP53 skin,face,carcinoma,NS c.375+1G>A p.? 17:7675993-7675993 8
21 COSM43588 TP53 skin,face,carcinoma,NS c.740A>C p.N247T 17:7674223-7674223 8
22 COSM11081 TP53 skin,face,carcinoma,NS c.733G>T p.G245C 17:7674230-7674230 8
23 COSM11073 TP53 skin,face,carcinoma,NS c.1024C>T p.R342* 17:7670685-7670685 8
24 COSM45733 TP53 skin,face,carcinoma,NS c.432G>A p.Q144Q 17:7675180-7675180 8
25 COSM10704 TP53 skin,face,carcinoma,NS c.844C>T p.R282W 17:7673776-7673776 8
26 COSM43700 TP53 skin,face,carcinoma,NS c.712T>A p.C238S 17:7674251-7674251 8
27 COSM10733 TP53 skin,face,carcinoma,NS c.574C>T p.Q192* 17:7674957-7674957 8
28 COSM44227 TP53 skin,face,carcinoma,NS c.854A>T p.E285V 17:7673766-7673766 8
29 COSM10768 TP53 skin,face,carcinoma,NS c.535C>T p.H179Y 17:7675077-7675077 8
30 COSM43651 TP53 skin,face,carcinoma,NS c.763A>T p.I255F 17:7674200-7674200 8
31 COSM44435 TP53 skin,face,carcinoma,NS c.96+1G>A p.? 17:7676381-7676381 8
32 COSM43920 TP53 skin,face,carcinoma,NS c.680C>T p.S227F 17:7674283-7674283 8
33 COSM44436 TP53 skin,face,carcinoma,NS c.375+2T>C p.? 17:7675992-7675992 8
34 COSM43596 TP53 skin,face,carcinoma,NS c.841G>A p.D281N 17:7673779-7673779 8
35 COSM10749 TP53 skin,face,carcinoma,NS c.830G>T p.C277F 17:7673790-7673790 8
36 COSM11084 TP53 skin,face,carcinoma,NS c.517G>A p.V173M 17:7675095-7675095 8
37 COSM43742 TP53 skin,face,carcinoma,NS c.419C>T p.T140I 17:7675193-7675193 8
38 COSM10887 TP53 skin,face,carcinoma,NS c.833C>G p.P278R 17:7673787-7673787 8
39 COSM44241 TP53 skin,face,carcinoma,NS c.592G>T p.E198* 17:7674939-7674939 8
40 COSM43665 TP53 skin,face,carcinoma,NS c.746G>C p.R249T 17:7674217-7674217 8
41 COSM45677 TP53 skin,face,carcinoma,NS c.714T>A p.C238* 17:7674249-7674249 8
42 COSM607136 TGFBR1 skin,neck,carcinoma,NS c.1247C>T p.S416F 9:99146601-99146601 8
43 COSM13234 SUFU skin,face,carcinoma,NS c.560C>T p.P187L 10:102592687-102592687 8
44 COSM13233 SUFU skin,face,carcinoma,NS c.591C>T p.F197F 10:102592718-102592718 8
45 COSM3942016 SMO skin,face,carcinoma,NS c.1376C>T p.A459V 7:129209307-129209307 8
46 COSM13146 SMO skin,face,carcinoma,NS c.1604G>T p.W535L 7:129210500-129210500 8
47 COSM6962965 SMO skin,neck,carcinoma,NS c.1103C>T p.P368L 7:129206332-129206332 8
48 COSM13144 SMO skin,face,carcinoma,NS c.1598G>A p.S533N 7:129210494-129210494 8
49 COSM13145 SMO skin,face,carcinoma,NS c.595C>T p.R199W 7:129205260-129205260 8
50 COSM216037 SMO skin,face,carcinoma,NS c.1234C>T p.L412F 7:129206557-129206557 8

Expression for Malignant Syringoma

Search GEO for disease gene expression data for Malignant Syringoma.

Pathways for Malignant Syringoma

Pathways related to Malignant Syringoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.57 DAND5 KRT15 KRT17 KRT20 KRT7
2 11.82 KRT17 KRT20 KRT7
3
Show member pathways
11.38 KRT15 KRT17 KRT20 KRT7
4
Show member pathways
11.29 KRT17 KRT7

GO Terms for Malignant Syringoma

Cellular components related to Malignant Syringoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 8.92 KRT15 KRT17 KRT20 KRT7

Biological processes related to Malignant Syringoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 epidermis development GO:0008544 9.26 KRT15 KRT17
2 keratinization GO:0031424 9.26 KRT15 KRT17 KRT20 KRT7
3 intermediate filament organization GO:0045109 9.16 KRT17 KRT20
4 cornification GO:0070268 8.92 KRT15 KRT17 KRT20 KRT7

Molecular functions related to Malignant Syringoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 structural constituent of cytoskeleton GO:0005200 8.8 KRT15 KRT17 KRT20

Sources for Malignant Syringoma

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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