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MCID: MLG049
MIFTS: 37

Malignant Syringoma

Categories: Cancer diseases, Rare diseases, Skin diseases
Genes Variations Tissues Related diseases Publications Pathways
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Aliases & Classifications for Malignant Syringoma

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MalaCards integrated aliases for Malignant Syringoma:

Name: Malignant Syringoma 12 15
Microcystic Adnexal Carcinoma 12 52
Microcystic Adnexal Carcinoma of Skin 12
Syringomatous Carcinoma 12
Eccrine Epithelioma 71

Classifications:

MalaCards categories:
Global: Rare diseases Cancer diseases
Anatomical: Skin diseases
See all MalaCards categories (disease lists)


External Ids:

Disease Ontology 12 DOID:5569
UMLS 71 C0346027
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Summaries for Malignant Syringoma

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NIH Rare Diseases : 52 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 infiltrative basal cell carcinoma and external ear carcinoma. An important gene associated with Malignant Syringoma is CLEC2A (C-Type Lectin Domain Family 2 Member A), and among its related pathways/superpathways is Keratinization. Affiliated tissues include skin, bone and breast.

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Related Diseases for Malignant Syringoma

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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 273)
# Related Disease Score Top Affiliating Genes
1 infiltrative basal cell carcinoma 30.5 KRT20 KRT15 CLEC2A
2 external ear carcinoma 30.4 KRT7 CEACAM5
3 basal cell carcinoma 30.0 PHLDA1 KRT7 KRT20 KRT15 CEACAM5
4 meibomian cyst 30.0 KRT7 KRT20 CLEC2A
5 syringoma 30.0 KRT7 DAND5 CLEC2A CEACAM5
6 teratoma 29.9 KRT7 KRT20 CEACAM5
7 polycystic liver disease 1 with or without kidney cysts 29.9 KRT7 KRT20 CEACAM5
8 cystic teratoma 29.9 KRT7 KRT20 CEACAM5
9 morpheaform basal cell carcinoma 29.7 PHLDA1 KRT20 KRT15 DAND5 CLEC2A
10 eyelid carcinoma 29.6 PIP KRT7 KRT20
11 sebaceous adenocarcinoma 29.6 PTGER4 PIP
12 skin benign neoplasm 29.5 PIP KRT7 KRT20 CLEC2A
13 clear cell basal cell carcinoma 29.2 PHLDA1 KRT7 KRT20 CLEC2A CEACAM5
14 apocrine sweat gland neoplasm 29.0 PIP KRT7 KRT15 CLEC2A CEACAM5
15 sweat gland cancer 28.3 PIP KRT7 KRT20 KRT15 CLEC2A CEACAM5
16 rare tumor 10.5
17 inverted follicular keratosis 10.4 PHLDA1 CLEC2A
18 pilar sheath acanthoma 10.4 PHLDA1 CLEC2A
19 epidermal appendage tumor 10.3 PHLDA1 KRT15
20 sebaceous gland neoplasm 10.3 KRT15 CLEC2A
21 renal pelvis transitional cell carcinoma 10.3 KRT7 CLEC2A
22 squamous cell carcinoma 10.3
23 acanthoma 10.3 KRT7 CLEC2A
24 hair follicle neoplasm 10.3 KRT15 CLEC2A
25 eyelid benign neoplasm 10.3 KRT20 CLEC2A
26 ovarian cystadenoma 10.3 KRT7 CEACAM5
27 leukoplakia of penis 10.2 KRT7 CEACAM5
28 papillary adenoma 10.2 KRT7 CEACAM5
29 ureter adenocarcinoma 10.2 KRT7 CEACAM5
30 pancreatic foamy gland adenocarcinoma 10.2 KRT7 CEACAM5
31 mucinous adenofibroma 10.2 KRT7 CEACAM5
32 neurofibromatosis, type ii 10.2
33 congenital heart defects, hamartomas of tongue, and polysyndactyly 10.2
34 adenocarcinoma 10.2
35 mucoepidermoid carcinoma 10.2
36 paresthesia 10.2
37 mucinous intrahepatic cholangiocarcinoma 10.2 KRT7 CEACAM5
38 villous adenoma 10.2 KRT20 CEACAM5
39 renal pelvis adenocarcinoma 10.2 KRT7 CEACAM5
40 biliary papillomatosis 10.2 KRT7 CEACAM5
41 adenoid basal cell carcinoma 10.2 KRT7 CEACAM5
42 appendiceal neoplasm 10.2 KRT7 CEACAM5
43 squamous cell bile duct carcinoma 10.2 KRT7 CEACAM5
44 bile duct mucoepidermoid carcinoma 10.2 KRT7 CEACAM5
45 liver sarcoma 10.2 KRT7 CEACAM5
46 biliary tract benign neoplasm 10.2 KRT7 CEACAM5
47 warthin tumor 10.2 KRT7 CEACAM5
48 sarcomatous intrahepatic cholangiocarcinoma 10.2 KRT7 CEACAM5
49 nasal cavity olfactory neuroblastoma 10.2 KRT7 CEACAM5
50 thymus adenocarcinoma 10.2 KRT20 CEACAM5

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



Diseases related to Malignant Syringoma
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Symptoms & Phenotypes for Malignant Syringoma

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Drugs & Therapeutics for Malignant Syringoma

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Search Clinical Trials , NIH Clinical Center for Malignant Syringoma

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Genetic Tests for Malignant Syringoma

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Anatomical Context for Malignant Syringoma

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MalaCards organs/tissues related to Malignant Syringoma:

40
Skin, Bone, Breast, Bone Marrow, Lymph Node, Lung, Salivary Gland
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Publications for Malignant Syringoma

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Articles related to Malignant Syringoma:

(show top 50) (show all 263)
# Title Authors PMID Year
1
Clinical course, risk factors, and treatment of microcystic adnexal carcinoma: a short series report. 61 52
12974700 2003
2
Collision tumor of microcystic adnexal carcinoma and squamous cell carcinoma discovered on Mohs sections. 61
32490104 2020
3
Next-generation sequencing implicates oncogenic roles for p53 and JAK/STAT signaling in microcystic adnexal carcinomas. 61
31857679 2020
4
Multifocal Recurrent Microcystic Adnexal Carcinoma. 61
32398617 2020
5
Morphologically High-Grade Microcystic Adnexal Carcinoma: A report of two cases. 61
32359178 2020
6
Microcystic adnexal carcinoma of the skin in atypical site (pubic region): clinical and dermatoscopic features. 61
30249079 2020
7
Microcystic Adnexal Carcinoma of the Face Treated With Definitive Chemoradiation: A Case Report and Review of the Literature. 61
32280832 2020
8
Dermoscopic pitfall: Microcystic adnexal carcinoma mimicking basal cell carcinoma. 61
31997790 2020
9
State of the art of Mohs surgery for rare cutaneous tumors in the Spanish Registry of Mohs Surgery (REGESMOHS). 61
31777957 2020
10
Solid Microcystic Adnexal Carcinoma on the Thigh: An Unusual Location. 61
31436574 2020
11
Microcystic adnexal carcinoma: report of rare cases. 61
31912868 2020
12
Ln-γ 2 chain of laminin-332 is a useful marker in differentiating between benign and malignant sclerosing adnexal neoplasms. 61
31429981 2020
13
Approaches to Perineural, Lymphovascular, and Single-Cell Disease. 61
31764297 2019
14
Microcystic Adnexal Carcinoma: A Rare, Commonly Misdiagnosed Malignancy. 61
31125069 2019
15
Microcystic Adnexal Carcinoma Originating in a Nevus Sebaceous: A Case Report of a 16-year-old Boy. 61
31386165 2019
16
Bilateral Facial Apocrine Fibrosing Hamartoma Mimicking Microcystic Adnexal Carcinoma. 61
31045870 2019
17
Utility of Ber-EP4 and MOC-31 in Basaloid Skin Tumor Detection. 61
29629953 2019
18
Plaque-Type Syringoma Masquerading as Microcystic Adnexal Carcinoma: Review of the Literature and Description of a Novel Technique That Emphasizes Lesion Architecture to Help Make the Diagnosis. 61
30921007 2019
19
Epidemiology and Survival of Microcystic Adnexal Carcinoma by Sex in the United States. 61
31567608 2019
20
Factors associated with the utilization of Mohs micrographic surgery in the treatment of microcystic adnexal carcinoma. 61
30721714 2019
21
Evidence-Based Clinical Practice Guidelines for Management of Microcystic Adnexal Carcinoma. 61
31268489 2019
22
Evidence-Based Clinical Practice Guidelines for Microcystic Adnexal Carcinoma: Informed by a Systematic Review. 61
31268498 2019
23
Microcystic adnexal carcinoma. 61
31347515 2019
24
Sclerosing Microcystic Adenocarcinoma: Report of a Rare Case and Review of Literature. 61
29987694 2019
25
Successful treatment of microcystic adnexal carcinoma on the philtrum with oral S-1 monotherapy. 61
30663155 2019
26
Microcystic adnexal carcinoma with large cysts: unusual clinical presentation and histological findings. 61
31251218 2019
27
Immunoreactivity for Sox10 in Basaloid Neoplasms of The Skin. 61
30531326 2019
28
Malignant Sweat Gland Tumors. 61
30497677 2019
29
Axillary and elbow lymph node metastasis arising after complete excision of microcystic adnexal carcinoma of a hand: A rare presentation. 61
31655287 2019
30
Solid carcinoma is a variant of microcystic adnexal carcinoma: A 14-case series. 61
30187501 2018
31
A case and literature review of orbital microcystic adnexal carcinoma. 61
29461905 2018
32
Microcystic adnexal carcinoma-diagnostic criteria and therapeutic methods: case report and review of the literature. 61
29602625 2018
33
Demographics and outcomes of microcystic adnexal carcinoma. 61
29559397 2018
34
Cutaneous Squamous Cell Carcinoma With Sclerosing Features: An Uncommon and Potentially Aggressive Variant. 61
29742557 2018
35
Microcystic adnexal carcinoma of the external auditory canal. 61
30235377 2018
36
Microcystic adnexal carcinoma of the orbit mimicking pagetoid sebaceous gland carcinoma. 61
29053046 2018
37
Microcystic Adnexal Carcinoma of the Nipple. 61
30059333 2018
38
[Microcystic adnexal carcinoma: A case report]. 61
29622469 2018
39
Histopathologic pitfalls of Mohs micrographic surgery and a review of tumor histology. 61
27832425 2018
40
Microcystic adnexal carcinoma with sebaceous differentiation: Three cases. 61
29352496 2018
41
An Unusual Morphological Presentation of Cutaneous Squamous Cell Carcinoma Mimicking Microcystic Adnexal Carcinoma: A Diagnostic Pitfall. 61
29998100 2018
42
Microcystic adnexal carcinoma mimicking basal cell carcinoma. 61
29034297 2017
43
Metastatic Microcystic Adnexal Carcinoma with DNA Sequencing Results and Response to Systemic Antineoplastic Chemotherapy. 61
28870941 2017
44
Microcystic Adnexal Carcinoma: A Review of the Literature. 61
28654578 2017
45
Mohs micrographic surgery of rare cutaneous tumours. 61
27976423 2017
46
Microcystic Adnexal Carcinoma and a Summary of Other Rare Malignant Adnexal Tumours. 61
28681209 2017
47
Longstanding microcystic adnexal carcinoma in a child. 61
28542906 2017
48
Update on Malignant Sweat Gland Tumors. 61
28477887 2017
49
Image Gallery: Microcystic adnexal carcinoma (syringomatous carcinoma and sclerosing sweat duct carcinoma) as an extensive sclerotic erythematous plaque with telangiectasia over the face. 61
28581237 2017
50
Epidemiology and Prognosis of Primary Periocular Sweat Gland Carcinomas. 61
26974419 2017
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Variations for Malignant Syringoma

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Cosmic variations for Malignant Syringoma:

9 (show top 50) (show all 1402)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)		 Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM87910628 TP53 skin,face,carcinoma,NS c.772G>A p.E258K 17:7674191-7674191 6
2 COSM87898709 TP53 skin,face,carcinoma,NS c.742C>T p.R248W 17:7674221-7674221 6
3 COSM87929396 TP53 skin,face,carcinoma,NS c.530C>T p.P177L 17:7675082-7675082 6
4 COSM87910495 TP53 skin,face,carcinoma,NS c.832C>T p.P278S 17:7673788-7673788 6
5 COSM87898991 TP53 skin,face,carcinoma,NS c.535C>T p.H179Y 17:7675077-7675077 6
6 COSM87899497 TP53 skin,face,carcinoma,NS c.586C>T p.R196* 17:7674945-7674945 6
7 COSM87906130 TP53 skin,face,carcinoma,NS c.854A>T p.E285V 17:7673766-7673766 6
8 COSM87944274 TP53 skin,face,carcinoma,NS c.419C>T p.T140I 17:7675193-7675193 6
9 COSM87903307 TP53 skin,face,carcinoma,NS c.841G>A p.D281N 17:7673779-7673779 6
10 COSM87898714 TP53 skin,face,carcinoma,NS c.844C>T p.R282W 17:7673776-7673776 6
11 COSM87900980 TP53 skin,face,carcinoma,NS c.839G>A p.R280K 17:7673781-7673781 6
12 COSM87961406 TP53 skin,face,carcinoma,NS c.532C>A p.H178N 17:7675080-7675080 6
13 COSM87905569 TP53 skin,face,carcinoma,NS c.740A>C p.N247T 17:7674223-7674223 6
14 COSM87908365 TP53 skin,face,carcinoma,NS c.454C>T p.P152S 17:7675158-7675158 6
15 COSM87918616 TP53 skin,face,carcinoma,NS c.993+1G>A p.? 17:7673534-7673534 6
16 COSM87911563 TP53 skin,face,carcinoma,NS c.388C>T p.L130F 17:7675224-7675224 6
17 COSM87899145 TP53 skin,face,carcinoma,NS c.763A>T p.I255F 17:7674200-7674200 6
18 COSM87899377 TP53 skin,face,carcinoma,NS c.375+1G>A p.? 17:7675993-7675993 6
19 COSM87919309 TP53 skin,face,carcinoma,NS c.509C>T p.T170M 17:7675103-7675103 6
20 COSM87900604 TP53 skin,face,carcinoma,NS c.637C>T p.R213* 17:7674894-7674894 6
21 COSM87898424 TP53 skin,face,carcinoma,NS c.722C>T p.S241F 17:7674241-7674241 6
22 COSM87897850 TP53 skin,face,carcinoma,NS c.833C>G p.P278R 17:7673787-7673787 6
23 COSM87897627 TP53 skin,face,carcinoma,NS c.574C>T p.Q192* 17:7674957-7674957 6
24 COSM87917387 TP53 skin,face,carcinoma,NS c.746G>C p.R249T 17:7674217-7674217 6
25 COSM87970671 TP53 skin,face,carcinoma,NS c.766A>C p.T256P 17:7674197-7674197 6
26 COSM87899049 TP53 skin,face,carcinoma,NS c.96+1G>A p.? 17:7676381-7676381 6
27 COSM87900565 TP53 skin,face,carcinoma,NS c.1024C>T p.R342* 17:7670685-7670685 6
28 COSM87915380 TP53 skin,face,carcinoma,NS c.830G>T p.C277F 17:7673790-7673790 6
29 COSM87906137 TP53 skin,face,carcinoma,NS c.260C>A p.P87Q 17:7676109-7676109 6
30 COSM87906983 TP53 skin,face,carcinoma,NS c.592G>T p.E198* 17:7674939-7674939 6
31 COSM87899887 TP53 skin,face,carcinoma,NS c.856G>A p.E286K 17:7673764-7673764 6
32 COSM87899906 TP53 skin,face,carcinoma,NS c.375+2T>C p.? 17:7675992-7675992 6
33 COSM87991360 TP53 skin,face,carcinoma,NS c.714T>A p.C238* 17:7674249-7674249 6
34 COSM87918646 TP53 skin,face,carcinoma,NS c.712T>A p.C238S 17:7674251-7674251 6
35 COSM87898544 TP53 skin,face,carcinoma,NS c.680C>T p.S227F 17:7674283-7674283 6
36 COSM87906806 TP53 skin,face,carcinoma,NS c.517G>A p.V173M 17:7675095-7675095 6
37 COSM87961289 TP53 skin,face,carcinoma,NS c.706T>C p.Y236H 17:7674257-7674257 6
38 COSM87898351 TP53 skin,neck,carcinoma,NS c.743G>A p.R248Q 17:7674220-7674220 6
39 COSM88110742 TP53 skin,face,carcinoma,NS c.432G>A p.Q144= 17:7675180-7675180 6
40 COSM87919221 TP53 skin,face,carcinoma,NS c.844C>G p.R282G 17:7673776-7673776 6
41 COSM87898463 TP53 skin,face,carcinoma,NS c.733G>T p.G245C 17:7674230-7674230 6
42 COSM135128838 TGFBR1 skin,neck,carcinoma,NS c.1259C>T p.S420F 9:99146601-99146601 6
43 COSM93619372 SUFU skin,face,carcinoma,NS c.591C>T p.F197= 10:102592718-102592718 6
44 COSM93618837 SUFU skin,face,carcinoma,NS c.560C>T p.P187L 10:102592687-102592687 6
45 COSM84675948 SMO skin,face,carcinoma,NS c.595C>T p.R199W 7:129205260-129205260 6
46 COSM84674285 SMO skin,face,carcinoma,NS c.1604G>T p.W535L 7:129210500-129210500 6
47 COSM84675075 SMO skin,face,carcinoma,NS c.1376C>T p.A459V 7:129209307-129209307 6
48 COSM84675839 SMO skin,face,carcinoma,NS c.1598G>A p.S533N 7:129210494-129210494 6
49 COSM84680970 SMO skin,face,carcinoma,NS c.1955C>T p.A652V 7:129212042-129212042 6
50 COSM84678061 SMO skin,neck,carcinoma,NS c.1103C>T p.P368L 7:129206332-129206332 6
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Expression for Malignant Syringoma

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Search GEO for disease gene expression data for Malignant Syringoma.
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Pathways for Malignant Syringoma

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

# Super pathways Score Top Affiliating Genes
1
Keratinization 65
Show member pathways
 11.25 KRT7 KRT20 KRT15
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GO Terms for Malignant Syringoma

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

# Name GO ID Score Top Affiliating Genes
1 intermediate filament GO:0005882 8.8 KRT7 KRT20 KRT15

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

# Name GO ID Score Top Affiliating Genes
1 keratinization GO:0031424 9.33 KRT7 KRT20 KRT15
2 cornification GO:0070268 9.13 KRT7 KRT20 KRT15
3 negative regulation of T cell apoptotic process GO:0070233 8.62 PIP DOCK8
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Sources for Malignant Syringoma

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3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
The MalaCards human disease database index: 1-9 A B C D E F G H I J K L M N O P Q R S T U V W X Y Z
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