MCID: SPR013
MIFTS: 36

Spiradenoma malady

Categories: Rare diseases, Cancer diseases, Skin diseases, Ear diseases

Aliases & Classifications for Spiradenoma

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Aliases & Descriptions for Spiradenoma:

Name: Spiradenoma 11 13
Eccrine Spiradenoma 11 48 68
Benign Eccrine Spiradenoma 11 48
Eccrine Spiradenoma, Benign 48
 
Eccrine Spiradenoma of Skin 11
Sarcoma, Epithelioid 68
Es 48

Classifications:



External Ids:

Disease Ontology11 DOID:5444
SNOMED-CT62 403938001, 4977000
NCIt45 C4170

Summaries for Spiradenoma

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Wikipedia:71 Spiradenoma, also spiroma or eccrine spiradenoma, is a cutaneous condition that is typically... more...

MalaCards based summary: Spiradenoma, also known as eccrine spiradenoma, is related to syringoma and malignant spiradenoma. An important gene associated with Spiradenoma is CYLD (CYLD Lysine 63 Deubiquitinase), and among its related pathways is Striated Muscle Contraction. Affiliated tissues include skin, t cells and b cells.

Related Diseases for Spiradenoma

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Diseases in the Spiradenoma family:

Malignant Spiradenoma

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

(show top 50)    (show all 233)
idRelated DiseaseScoreTop Affiliating Genes
1syringoma30.0MUC1, VIM
2malignant spiradenoma12.1
3benign eccrine breast spiradenoma11.8
4breast malignant eccrine spiradenoma11.8
5adnexal spiradenoma/cylindroma of a sweat gland11.8
6brooke-spiegler syndrome11.5
7ewing sarcoma11.1
8cylindromatosis, familial11.0
9benign intermediate mesothelioma10.4CYLD, KRT14
10glomangioma10.4CYLD, TP53
11connective tissue benign neoplasm10.4CYLD, KRT14
12logopenic progressive aphasia10.4MUC1, S100B
13intermediate dend syndrome10.3MUC1, S100B
14papillary adenoma10.3CYLD, KRT14
15lyme disease - neurological complications10.3MUC1, S100B
16relapsing epidemic typhus10.3MUC1, S100B
17pituitary deficiency due to rathke's pouch cysts10.3MUC1, S100B
18subacute leukemia10.3MUC1, S100B
19pseudoglandular variant testicular seminoma10.3MUC1, S100B
20penis verrucous carcinoma10.3MUC1, S100B
21breast papillary carcinoma10.3MUC1, S100B
22epicardium cancer10.3MUC1, S100B
23hypotonia10.3S100B, VIM
24exstrophy-epispadias complex10.3S100B, VIM
25absence of fingerprints congenital milia10.3S100B, VIM
26lipoadenoma10.3MUC1, S100B
27hypoglossal nerve neoplasm10.3S100B, TP53
28cribriform carcinoma10.2KRT14, TP53
29fraxf syndrome10.2S100B, VIM
30sporadic idiopathic steroid-resistant nephrotic syndrome with minimal changes10.2MUC1, S100B
31malignant cystic nephroma10.2S100B, VIM
32water-clear cell adenoma10.2KRT14, MUC1
33liver disease10.2KRT14, MUC1
34malignant adenofibroma10.2S100B, VIM
35epithelioid cell melanoma10.2S100B, VIM
36amelogenesis imperfecta local hypoplastic10.2KRT14, VIM
372q33.1 microdeletion syndrome10.2MUC1, VIM
38ovarian serous adenofibroma10.2MUC1, TP53
39pauciarticular onset juvenile idiopathic arthritis10.2S100B, VIM
40mature b-cell neoplasm10.2KRT14, TP53
41advanced sleep phase syndrome10.2MUC1, TP53
42osteopetrosis and infantile neuroaxonal dystrophy10.2S100B, VIM
43anal squamous cell carcinoma10.2KRT14, TP53
44oligoastrocytoma10.2KRT14, VIM
45ductal carcinoma in situ10.2KRT14, TP53
46distal monosomy 7q3610.2MUC1, VIM
47transitional meningioma10.2MUC1, VIM
48extrahepatic bile duct adenocarcinoma10.2MUC1, TP53
49striated muscle rhabdoid tumor10.1MUC1, TP53
50monophasic synovial sarcoma10.1MUC1, VIM

Graphical network of the top 20 diseases related to Spiradenoma:



Diseases related to spiradenoma

Symptoms & Phenotypes for Spiradenoma

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Drugs & Therapeutics for Spiradenoma

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FDA approved drugs:

id Drug Name Active Ingredient(s)16 Company Approval Date
1
Erbitux16 44 CETUXIMAB Imclone, Bristol-Myers Squibb February 2004
FDA Label: Erbitux
Disease/s that Drug Treats:Colorectal Cancer
Indications and Usage:16 Erbitux® is an epidermal growth factor receptor (EGFR) antagonist indicatedfor treatment of:Head and Neck Cancer Locally or regionally advanced squamous cell carcinoma of the headand neck in combination with radiation therapy. (1.1, 14.1) Recurrent locoregional disease or metastatic squamous cell carcinomaof the head and neck in combination with platinum-based therapy with5-FU. (1.1, 14.1) Recurrent or metastatic squamous cell carcinoma of the head and neckprogressing after platinum-based therapy. (1.1, 14.1)Colorectal CancerK-Ras wild-type, EGFR-expressing, metastatic colorectal cancer asdetermined by FDA-approved tests in combination with FOLFIRI for first-line treatment, in combination with irinotecan in patients who are refractory toirinotecan-based chemotherapy, as a single agent in patients who have failed oxaliplatin- andirinotecan-based chemotherapy or who are intolerant to irinotecan.(1.2, 5.7, 12.1, 14.2)Limitation of Use: Erbitux is not indicated for treatment of Ras-mutantcolorectal cancer. (5.7, 14.2)
DrugBank Targets:14 1. Epidermal growth factor receptor;2. Low affinity immunoglobulin gamma Fc region receptor III-B;3. Complement C1r subcomponent;4. Complement C1q subcomponent subunit A;5. Complement C1q subcomponent subunit B;6. Complement C1q subcomponent subunit C;7. Low affinity immunoglobulin gamma Fc region receptor III-A;8. Complement C1s subcomponent;9. High affinity immunoglobulin gamma Fc receptor I;10. Low affinity immunoglobulin gamma Fc region receptor II-a;11. Low affinity immunoglobulin gamma Fc region receptor II-b;12. Low affinity immunoglobulin gamma Fc region receptor II-c
Mechanism of Action:16 
Target: binding of epidermal growth factor (EGF) and other ligands to epidermal growth factor receptor (EGFR, HER1, c-ErbB-1)
Action: inhibitor
FDA: The epidermal growth factor receptor (EGFR, HER1, c-ErbB-1) is a transmembraneglycoprotein that is a member of a subfamily of type I receptor tyrosine kinases including EGFR,HER2, HER3, and HER4. The EGFR is constitutively expressed in many normal epithelialtissues, including the skin and hair follicle. Expression of EGFR is also detected in many humancancers including those of the head and neck, colon, and rectum. Cetuximab binds specifically to the EGFR on both normal and tumor cells, and competitivelyinhibits the binding of epidermal growth factor (EGF) and other ligands, such as transforminggrowth factor-alpha. In vitro assays and in vivo animal studies have shown that binding ofcetuximab to the EGFR blocks phosphorylation and activation of receptor-associated kinases,resulting in inhibition of cell growth, induction of apoptosis, and decreased matrixmetalloproteinase and vascular endothelial growth factor production. Signal transduction throughthe EGFR results in activation of wild-type Ras proteins, but in cells with activating Ras somaticmutations, the resulting mutant Ras proteins are continuously active regardless of EGFRregulation.In vitro, cetuximab can mediate antibody-dependent cellular cytotoxicity (ADCC) against certainhuman tumor types. In vitro assays and in vivo animal studies have shown that cetuximabinhibits the growth and survival of tumor cells that express the EGFR. No anti-tumor effects ofcetuximab were observed in human tumor xenografts lacking EGFR expression. The addition ofcetuximab to radiation therapy or irinotecan in human tumor xenograft models in mice resultedin an increase in anti-tumor effects compared to radiation therapy or chemotherapy alone.
2
Evista16 44 RALOXIFENE HYDROCHLORIDE Eli Lilly September 2007
FDA Label: Evista
Disease/s that Drug Treats:osteoporosis and reduction of breast cancer risk in postmenopausal women
Indications and Usage:16 EVISTA is an estrogen agonist/antagonist indicated for Treatment and prevention of osteoporosis in postmenopausal women.(1.1)
DrugBank Targets:14 1. Estrogen receptor;2. Estrogen receptor beta
Mechanism of Action:16 
Target: estrogenic pathways
Action: can be an activator or antooagonist
FDA: Decreases in estrogen levels after oophorectomy or menopause lead to increases in bone resorption andaccelerated bone loss. Bone is initially lost rapidly because the compensatory increase in bone formation isinadequate to offset resorptive losses. In addition to loss of estrogen, this imbalance between resorption andformation may be due to age-related impairment of osteoblasts or their precursors. In some women, these changeswill eventually lead to decreased bone mass, osteoporosis, and increased risk for fractures, particularly of the spine,hip, and wrist. Vertebral fractures are the most common type of osteoporotic fracture in postmenopausal women.The biological actions of raloxifene are largely mediated through binding to estrogen receptors. This bindingresults in activation of certain estrogenic pathways and blockade of others. Thus, raloxifene is an estrogenagonist/antagonist, commonly referred to as a selective estrogen receptor modulator (SERM).Raloxifene decreases resorption of bone and reduces biochemical markers of bone turnover to thepremenopausal range. These effects on bone are manifested as reductions in the serum and urine levels of boneturnover markers, decreases in bone resorption based on radiocalcium kinetics studies, increases in bone mineraldensity (BMD), and decreases in incidence of fractures.
3
Istodax16 44 ROMIDEPSIN Gloucester Pharmaceuticals November 2009
FDA Label: Istodax
Disease/s that Drug Treats:cutaneous T-cell lymphoma
Indications and Usage:16 ISTODAX is a histone deacetylase (HDAC) inhibitor indicated for: Treatment of cutaneous T-cell lymphoma (CTCL) in patients who havereceived at least one prior systemic therapy (1). Treatment of peripheral T-cell lymphoma (PTCL) in patients who havereceived at least one prior therapy (1).These indications are based on response rate. Clinical benefit such asimprovement in overall survival has not been demonstrated (1).
DrugBank Targets:14 -
Mechanism of Action:16 
Target: histone deacetylase (HDAC)
Action: inhibitor
FDA: Romidepsin is a histone deacetylase (HDAC) inhibitor. HDACs catalyze the removal of acetyl groups from acetylated lysine residues in histones, resulting in themodulation of gene expression. HDACs also deacetylate non-histone proteins, such as transcription factors. In vitro, romidepsin causes the accumulation of acetylatedhistones, and induces cell cycle arrest and apoptosis of some cancer cell lines with IC50 values in the nanomolar range. The mechanism of the antineoplastic effect ofromidepsin observed in nonclinical and clinical studies has not been fully characterized.
4
Treanda16 44 BENDAMUSTINE HYDROCHLORIDE Cephalon October 2008
FDA Label: Treanda
Disease/s that Drug Treats:Chronic lymphocytic leukemia and B-cell non-Hodgkin’s lymphoma
Indications and Usage:16 TREANDA is an alkylating drug indicated for treatment of patients with: Chronic lymphocytic leukemia (CLL). Efficacy relative to first linetherapies other than chlorambucil has not been established. (1.1) Indolent B-cell non-Hodgkin lymphoma (NHL) that has progressed duringor within six months of treatment with rituximab or a rituximab-containingregimen. (1.2)
DrugBank Targets: -
Mechanism of Action:16 
Target: -
Action: -
FDA: Bendamustine is a bifunctional mechlorethamine derivative containing a purine-like benzimidazole ring.Mechlorethamine and its derivatives form electrophilic alkyl groups. These groups form covalent bonds with electronrichnucleophilic moieties, resulting in interstrand DNA crosslinks. The bifunctional covalent linkage can lead to celldeath via several pathways. Bendamustine is active against both quiescent and dividing cells. The exact mechanism ofaction of bendamustine remains unknown.

Interventional clinical trials:

Search ClinicalTrials, NIH Clinical Center for Spiradenoma

Genetic Tests for Spiradenoma

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Anatomical Context for Spiradenoma

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MalaCards organs/tissues related to Spiradenoma:

36
Skin, T cells, B cells, Endothelial, Testes, Colon, Bone

Publications for Spiradenoma

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Articles related to Spiradenoma:

(show top 50)    (show all 164)
idTitleAuthorsYear
1
FDG PET/CT in Malignant Eccrine Spiradenoma. (28005641)
2017
2
A rare case of eccrine spiradenoma-treatment and management. (27069311)
2016
3
Giant vascular eccrine spiradenoma of the leg: MR imaging findings. (27397887)
2016
4
Spiradenoma causing longitudinal splitting of the nail. (27663150)
2016
5
Distinctive immunostaining of claudin-4 in spiradenomas. (26616722)
2016
6
Malignant Spiradenoma: a Giant Cutaneous Adnexal Tumor. (26563774)
2015
7
Spiradenocarcinoma in Preexisting Spiradenoma With a Large In Situ Adenocarcinoma Component. (25830720)
2015
8
Eccrine Spiradenoma Arising from the Breast Skin. (26236527)
2015
9
Giant Vascular Eccrine Spiradenoma. (26365030)
2015
10
Inexplicable Co- Existence of Eccrine Spiradenoma and Lichen Planus in an old Case of Basal Cell Carcinoma. (25737995)
2015
11
Sonographic and sonoelastographic findings of a rarely seen soft tissue tumor: eccrine spiradenoma. (26576987)
2015
12
Zosteriform spiradenoma with spiradenocarcinoma: A rare entity. (26904446)
2015
13
Malignant Eccrine Spiradenoma of the Face. (26167988)
2015
14
Malignant eccrine spiradenoma of the external ear. (26211868)
2015
15
A serpentine inside eccrine spiradenoma: a new trichoscopic sign. (25878450)
2015
16
Trichoepithelioma and spiradenoma collision tumor. (25942028)
2015
17
Giant eccrine spiradenoma mimicking a malignant tumor. (25566913)
2015
18
Malignant eccrine breast spiradenoma. A case report and literature review. (26318134)
2015
19
Multiple Segmental Eccrine Spiradenoma with a Zosteriform Pattern: A Case Report and Literature Review. (26273161)
2015
20
Spiradenoma and cylindroma originate from the hair follicle bulge and not from the eccrine sweat gland: an immunohistochemical study with CD200 and other stem cell markers. (25354097)
2014
21
A malignant eccrine spiradenoma of the scalp. (24842352)
2014
22
Differential diagnosis of eccrine spiradenoma: A case report. (25187803)
2014
23
Eccrine spiradenoma in knee. (25284865)
2014
24
Cystic eccrine spiradenoma of the finger mimicking a ganglion. (25289292)
2014
25
Fine needle aspiration cytology of eccrine spiradenoma of back: report of a rare case. (25328342)
2014
26
Case of recurrence of spiradenoma in palpebral conjunctiva. (24969313)
2014
27
Tubular Spitz Naevus Mimicking Eccrine Spiradenoma. (23756597)
2014
28
Neonatal spiradenoma. (22747799)
2013
29
Infiltrative Recurrent Eccrine Spiradenoma of the Anterior Neck Treated Using Mohs Micrographic Surgery. (24118397)
2013
30
Giant vascular eccrine spiradenoma mimicking calcifying epithelioma. (23961898)
2013
31
Brooke-Spiegler syndrome associated with cylindroma, trichoepithelioma and eccrine spiradenoma. (24261740)
2013
32
Immunohistochemical characterization of non-epithelial cells in spiradenoma. (24580131)
2013
33
Painful eccrine spiradenoma containing nerve fibers: a case report. (22777362)
2012
34
Dermatoscopic pattern of a spiradenoma. (23785623)
2012
35
Malignant spiradenoma/cylindroma of the vulva. (23147883)
2012
36
Giant vascular eccrine spiradenoma. (22148049)
2011
37
A rare case of giant malignant eccrine spiradenoma. (21342314)
2011
38
Carcinosarcoma ex eccrine spiradenoma of the vulva: report of the first case. (21464722)
2011
39
Alteration of the I^-catenin pathway in spiradenoma. (21518380)
2011
40
Differential considerations of skin tumours with florid vascularisation: report of a solitary giant vascular eccrine spiradenoma. (22689604)
2011
41
Malignant eccrine spiradenoma: a meta-analysis of reported cases. (20851376)
2011
42
Eccrine spiradenoma arising in the breast misdiagnosed as an epidermal inclusion cyst. (21430945)
2011
43
Transition from cylindroma to spiradenoma in CYLD-defective tumours is associated with reduced DKK2 expression. (21598248)
2011
44
Expression of p53 and TP53 mutational analysis in malignant neoplasms arising in preexisting spiradenoma, cylindroma, and spiradenocylindroma, sporadic or associated with Brooke-Spiegler syndrome. (20075707)
2010
45
Not all stubbed toes are innocuous--A case report of rare malignant eccrine spiradenoma (spiradenocarcinoma) of the toe. (20483125)
2010
46
Malignant eccrine spiradenoma: a new case report. (19614990)
2010
47
Malignant eccrine spiradenoma: case report and review of the literature, including 15 Japanese cases. (19222500)
2010
48
Spiradenoma with marked adenomyoepitheliomatous features. (20859082)
2010
49
A case of eccrine spiradenoma in a patient with neurofibromatosis. (20548911)
2010
50
Carcinoma ex spiradenoma/cylindroma confirmed by immunohistochemical and molecular loss-of-heterozygosity profiling. (19684510)
2009

Variations for Spiradenoma

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Expression for genes affiliated with Spiradenoma

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Search GEO for disease gene expression data for Spiradenoma.

Pathways for genes affiliated with Spiradenoma

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Pathways related to Spiradenoma according to GeneCards Suite gene sharing:

idSuper pathwaysScoreTop Affiliating Genes
19.0ACTC1, VIM

GO Terms for genes affiliated with Spiradenoma

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Biological processes related to Spiradenoma according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1DNA damage response, signal transduction by p53 class mediator resulting in cell cycle arrestGO:000697710.1MUC1, TP53
2DNA damage response, signal transduction by p53 class mediator resulting in transcription of p21 class mediatorGO:000697810.1MUC1, TP53
3muscle filament slidingGO:00300499.7ACTC1, VIM
4positive regulation of gene expressionGO:00106288.6ACTC1, TP53, VIM

Molecular functions related to Spiradenoma according to GeneCards Suite gene sharing:

idNameGO IDScoreTop Affiliating Genes
1keratin filament bindingGO:19902549.9KRT14, VIM
2p53 bindingGO:00020399.5MUC1, TP53

Sources for Spiradenoma

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2CDC
6CNVD
10DGIdb
15ExPASy
16FDA
17FMA
27GTR
28HGMD
29HMDB
30ICD10
31ICD10 via Orphanet
32ICD9CM
33IUPHAR
34KEGG
37MedGen
39MeSH
40MESH via Orphanet
41MGI
44NCI
45NCIt
46NDF-RT
49NINDS
50Novoseek
52OMIM
53OMIM via Orphanet
57PubMed
58QIAGEN
63SNOMED-CT via Orphanet
67Tumor Gene Family of Databases
68UMLS
69UMLS via Orphanet