SCCO
MCID: OVR058
MIFTS: 35

Ovarian Small Cell Carcinoma (SCCO)

Categories: Cancer diseases, Endocrine diseases, Rare diseases, Reproductive diseases

Aliases & Classifications for Ovarian Small Cell Carcinoma

MalaCards integrated aliases for Ovarian Small Cell Carcinoma:

Name: Ovarian Small Cell Carcinoma 12 54 15 17 74
Small Cell Carcinoma of the Ovary 54 60
Small Cell Ovarian Carcinoma 60
Scco 60

Classifications:



External Ids:

Disease Ontology 12 DOID:6179
NCIt 51 C27390
ICD10 via Orphanet 35 C56
Orphanet 60 ORPHA370396
UMLS 74 C2212006

Summaries for Ovarian Small Cell Carcinoma

NIH Rare Diseases : 54 Ovarian small cell carcinoma is a rare form of ovarian cancer that typically occurs in young women. It is typically an aggressive cancer that can spread (metastasize) to other parts of the body. There are two types of ovarian small cell carcinoma: hypercalcemic and pulmonary. Approximately 2/3 of individuals with ovarian small cell carcinoma have the hypercalcemic type. Symptoms are fairly nonspecific and are similar to other types of ovarian cancer. Symptoms may include: bloating, pelvic or abdominal pain, and appetite changes. Treatment may involve surgery to remove the tumor/tumors if the cancer is detected at an early stage, chemotherapy, and radiation therapy.

MalaCards based summary : Ovarian Small Cell Carcinoma, also known as small cell carcinoma of the ovary, is related to small cell carcinoma and atypical teratoid rhabdoid tumor, and has symptoms including abdominal pain An important gene associated with Ovarian Small Cell Carcinoma is SMARCA4 (SWI/SNF Related, Matrix Associated, Actin Dependent Regulator Of Chromatin, Subfamily A, Member 4), and among its related pathways/superpathways is Neural Stem Cell Differentiation Pathways and Lineage-specific Markers. The drug lysine has been mentioned in the context of this disorder. Affiliated tissues include ovary, bone and lymph node.

Disease Ontology : 12 An ovarian carcinoma that is an undifferentiated neoplasm composed of primitive-appearing cells.

Related Diseases for Ovarian Small Cell Carcinoma

Diseases in the Ovarian Clear Cell Carcinoma family:

Ovarian Small Cell Carcinoma

Diseases related to Ovarian Small Cell Carcinoma via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 110)
# Related Disease Score Top Affiliating Genes
1 small cell carcinoma 31.3 SMARCA4 SYP
2 atypical teratoid rhabdoid tumor 30.1 SMARCA4 SYP
3 pleomorphic liposarcoma 29.8 DES MUC1
4 rhabdoid cancer 29.6 SMARCA4 SYP WT1
5 hypercalcemic type ovarian small cell carcinoma 12.7
6 pulmonary type ovarian small cell carcinoma 12.6
7 rhabdoid tumor predisposition syndrome 2 11.2
8 childhood ovarian cancer 11.2
9 ovarian cancer 1 10.3
10 ovarian cancer 10.3
11 angiomyoma 10.2 PTHLH SYP
12 testicular granulosa cell tumor 10.1 SMARCA4 WT1
13 juvenile type testicular granulosa cell tumor 10.1 SMARCA4 WT1
14 sinonasal undifferentiated carcinoma 10.1 MUC1 SYP
15 papillary tumor of the pineal region 10.1 MUC1 SYP
16 middle ear adenoma 10.1 MUC1 SYP
17 cystadenoma 10.1 MUC1 SYP
18 chordoid meningioma 10.1 MUC1 SYP
19 clear cell ependymoma 10.1 MUC1 SYP
20 brachydactyly, type e1 10.1 MUC1 PTHLH
21 ependymoma 10.1 MUC1 SYP
22 pancreatoblastoma 10.1 MUC1 SYP
23 mucinous ovarian cystadenoma 10.1 MUC1 SYP
24 anaplastic ependymoma 10.1 MUC1 SYP
25 papilloma of choroid plexus 10.1 MUC1 SYP
26 sclerosing hemangioma 10.1 MUC1 SYP
27 cerebral primitive neuroectodermal tumor 10.1 MUC1 SYP
28 gastric tubular adenocarcinoma 10.1 MUC1 SYP
29 tubular adenocarcinoma 10.1 MUC1 SYP
30 peritoneal serous adenocarcinoma 10.1 SYP WT1
31 mixed ductal-endocrine carcinoma 10.1 MUC1 SYP
32 endosalpingiosis 10.1 MUC1 WT1
33 cauda equina neoplasm 10.1 SYP WT1
34 venous hemangioma 10.1 SYP WT1
35 male reproductive organ benign neoplasm 10.1 SYP WT1
36 adenosquamous carcinoma 10.1 MUC1 PTHLH
37 sarcomatoid mesothelioma 10.1 MUC1 WT1
38 optic nerve glioma 10.1 MUC1 SYP
39 dysgerminoma 10.0 PTHLH WT1
40 dysgerminoma of ovary 10.0 PTHLH WT1
41 large cell carcinoma 10.0 PTHLH SYP
42 sertoli-leydig cell tumor 10.0 MUC1 WT1
43 ovarian mucinous neoplasm 10.0 SYP WT1
44 peritoneal mesothelioma 10.0 MUC1 WT1
45 neurofibroma 10.0 MUC1 SYP
46 hemangioblastoma 10.0 MUC1 SYP
47 oncocytoma 10.0 MUC1 SYP
48 chondrosarcoma, extraskeletal myxoid 10.0 SYP WT1
49 sex cord-gonadal stromal tumor 10.0 SYP WT1
50 malignant ovarian surface epithelial-stromal neoplasm 10.0 MUC1 WT1

Graphical network of the top 20 diseases related to Ovarian Small Cell Carcinoma:



Diseases related to Ovarian Small Cell Carcinoma

Symptoms & Phenotypes for Ovarian Small Cell Carcinoma

UMLS symptoms related to Ovarian Small Cell Carcinoma:


abdominal pain

Drugs & Therapeutics for Ovarian Small Cell Carcinoma

Drugs for Ovarian Small Cell Carcinoma (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


# Name Status Phase Clinical Trials Cas Number PubChem Id
1 lysine Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial Sarcoma Recruiting NCT02601950 Phase 2 Tazemetostat
2 International Ovarian & Testicular Stromal Tumor Registry Recruiting NCT01970696
3 Tazemetostat Expanded Access Program for Adults With Solid Tumors Temporarily not available NCT03874455 Tazemetostat

Search NIH Clinical Center for Ovarian Small Cell Carcinoma

Genetic Tests for Ovarian Small Cell Carcinoma

Anatomical Context for Ovarian Small Cell Carcinoma

MalaCards organs/tissues related to Ovarian Small Cell Carcinoma:

42
Ovary, Bone, Lymph Node, Breast, Bone Marrow

Publications for Ovarian Small Cell Carcinoma

Articles related to Ovarian Small Cell Carcinoma:

(show top 50) (show all 82)
# Title Authors Year
1
An In-Depth Look at Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT): Clinical Implications from Recent Molecular Findings. ( 30662543 )
2019
2
CDK4/6 inhibitors target SMARCA4-determined cyclin D1 deficiency in hypercalcemic small cell carcinoma of the ovary. ( 30718512 )
2019
3
Cytologic diagnosis of small cell carcinoma of the ovary: A case report with review of the literature. ( 30821422 )
2019
4
The dilemma of early preventive oophorectomy in familial small cell carcinoma of the ovary of hypercalcemic type. ( 30886884 )
2019
5
Ovarian Small Cell Carcinoma: A Rare Case Report and Review of Literature. ( 29731803 )
2018
6
Primary ovarian small cell carcinoma of pulmonary type with coexisting endometrial carcinoma in a breast cancer patient receiving tamoxifen: A case report and literature review. ( 29879027 )
2018
7
Ovarian small cell carcinoma in one of a pair of monozygous twins. ( 30284660 )
2018
8
Small Cell Carcinoma of the Ovary: A Rare Tumor With a Poor Prognosis. ( 29621125 )
2018
9
Two types of small cell carcinoma of the ovary: Two typical case reports. ( 30094313 )
2018
10
Histone Deacetylase Inhibitors Synergize with Catalytic Inhibitors of EZH2 to Exhibit Antitumor Activity in Small Cell Carcinoma of the Ovary, Hypercalcemic Type. ( 30232145 )
2018
11
Pediatric Patients with Small-Cell Carcinoma of the Ovary Have Similar Survival to Adults: A Review of the National Cancer Database. ( 30269708 )
2018
12
Multimodal molecular analysis of an atypical small cell carcinoma of the ovary, hypercalcemic type. ( 30275002 )
2018
13
Clinical, morphological and immunohistochemical evidence that small-cell carcinoma of the ovary of hypercalcaemic type (SCCOHT) may be a primitive germ-cell neoplasm. ( 28130795 )
2017
14
Small cell carcinoma of the ovary hypercalcemic type (SCCOHT): A rare case after in vitro fertilization (IVF). ( 28367208 )
2017
15
The histone methyltransferase EZH2 is a therapeutic target in small cell carcinoma of the ovary, hypercalcaemic type. ( 28444909 )
2017
16
F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Imaging for Diagnosing Ovarian Small Cell Carcinoma of the Hypercalcemic Type. ( 26941866 )
2016
17
A case of ovarian small cell carcinoma of the pulmonary type that was observed as it developed. ( 29787016 )
2016
18
Ovarian Small Cell Carcinoma Hypercalcemic Type: A Case Report >. ( 28124855 )
2016
19
Ovarian small-cell carcinoma hypercalcemic type successfully treated: a case report and literature review. ( 27042109 )
2016
20
Refractory Small Cell Carcinoma of the Ovary - Hypercalcemic Type (SCCOHT) Treated with Romidepsin and Topotecan: A Case Report and Review of the Literature. ( 29772136 )
2016
21
SMARCA4 (BRG1) loss of expression is a useful marker for the diagnosis of ovarian small cell carcinoma of the hypercalcemic type (ovarian rhabdoid tumor): a comprehensive analysis of 116 rare gynecologic tumors, 9 soft tissue tumors, and 9 melanomas. ( 26135561 )
2015
22
Ovarian Small Cell Carcinoma of Pulmonary Type Arising in Mature Cystic Teratomas With Metastases to the Contralateral Ovary. ( 25990936 )
2015
23
Germline SMARCA4 mutations in patients with ovarian small cell carcinoma of hypercalcemic type. ( 25886974 )
2015
24
Loss of the tumor suppressor SMARCA4 in small cell carcinoma of the ovary, hypercalcemic type (SCCOHT). ( 26942101 )
2014
25
Ovarian small cell carcinoma of hypercalcemic type - evidence of germline origin and smarca4 gene inactivation. a pilot study. ( 24375037 )
2013
26
Primary ovarian small cell carcinoma of pulmonary type with enlarged paraaortic lymph node masses: a case report and review of the literature. ( 22873108 )
2012
27
Ovarian small cell carcinoma complicated by carcinomatous meningitis. ( 22826783 )
2012
28
Ovarian small cell carcinoma of hypercalcaemic-type: history of the disease in a patient who refused treatment. ( 21534770 )
2011
29
Ovarian small cell carcinoma, hypercalcemic type exhibiting a response to high-dose chemotherapy. ( 21117517 )
2010
30
Hypercalcemic-type ovarian small cell carcinoma with unique CD34 expression. ( 19788625 )
2009
31
Clinical management of ovarian small-cell carcinoma of the hypercalcemic type: a proposal for conservative surgery in an advanced stage of disease. ( 19407558 )
2009
32
Ovarian small cell carcinoma and cytokeratin perinuclear dotlike staining. ( 18227717 )
2008
33
Large cell variant ovarian small cell carcinoma: case report. ( 18958794 )
2008
34
A case of pulmonary type of ovarian small cell carcinoma. ( 17441897 )
2007
35
Complete remission of ovarian small cell carcinoma treated with irinotecan and cisplatin: a case report. ( 17695433 )
2007
36
Ovarian small cell carcinoma of the hypercalcemic type in children and adolescents: a prognostically unfavorable but curable disease. ( 16998935 )
2006
37
Small cell carcinoma of the ovary presenting as bone marrow metastasis: a rare presenting feature. ( 16761770 )
2005
38
Advanced small cell carcinoma of the ovary in a pediatric patient with long-term survival and review of the literature. ( 15750452 )
2005
39
Small cell carcinoma of the ovary with hypercalcemia and ectopic parathyroid hormone production. ( 15794681 )
2005
40
Primary ovarian small cell carcinoma of the pulmonary type: a case report and review of the literature. ( 15032292 )
2004
41
Establishment and characterization of a human ovarian small cell carcinoma, hypercalcemic type, cell line (OS-1) secreting PTH, PthrP and ACTH--special reference to the susceptibility of anti-cancer drugs. ( 16035505 )
2004
42
Small cell carcinoma of the ovary: a report of three cases and review of the literature. ( 14766457 )
2004
43
Stage IIIC small cell carcinoma of the ovary: survival with conservative surgery and chemotherapy. ( 15121630 )
2004
44
Small cell carcinoma of the ovary with hypercalcemia causes severe pancreatitis and altered mental status. ( 15581988 )
2004
45
Pathological case of the month. Acute symptomatic hypercalcemia associated with ovarian small cell carcinoma. ( 11772197 )
2002
46
Ovarian small cell carcinoma: a rare neoplasm in a 15-year-old female. ( 10888454 )
2000
47
Large-cell variant of small cell carcinoma of the ovary with hypercalcaemia. ( 11129517 )
2000
48
Ovarian small cell carcinoma of the hypercalcemic type in a 14 month old: the youngest reported case. ( 10102028 )
1999
49
Small-cell carcinoma of the ovary of the hypercalcemic type in an 8-year-old girl. ( 10415312 )
1999
50
Photodynamic therapy for the hypercalcemic type of the small cell carcinoma of the ovary in a mouse xenograft model. ( 10600305 )
1999

Variations for Ovarian Small Cell Carcinoma

Cosmic variations for Ovarian Small Cell Carcinoma:

9 (show all 37)
# Cosmic Mut ID Gene Symbol COSMIC Disease Classification
(Primary site, Site subtype, Primary histology, Histology subtype)
Mutation CDS Mutation AA GRCh38 Location Conf
1 COSM3724504 WT1 ovary,NS,carcinoma,small cell carcinoma c.1036A>C p.K346Q 11:32396266-32396266 0
2 COSM10704 TP53 ovary,NS,carcinoma,small cell carcinoma c.844C>T p.R282W 17:7673776-7673776 0
3 COSM44535 TP53 ovary,NS,carcinoma,small cell carcinoma c.761T>A p.I254N 17:7674202-7674202 0
4 COSM3724490 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.2506G>T p.G836* 19:11019591-11019591 0
5 COSM6493920 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.1245+1G>T p.? 19:10989444-10989444 0
6 COSM3724530 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.2439-2A>T p.? 19:11018955-11018955 0
7 COSM335837 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.991C>T p.Q331* 19:10987797-10987797 0
8 COSM3724498 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.4170+1G>A p.? 19:11035133-11035133 0
9 COSM368689 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.3546+1G>T p.? 19:11030894-11030894 0
10 COSM1737873 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.3277C>T p.R1093* 19:11027845-11027845 0
11 COSM3735018 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.561C>G p.Y187* 19:10986394-10986394 0
12 COSM3724528 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.3168+1G>A p.? 19:11025509-11025509 0
13 COSM3724529 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.2438+1G>A p.? 19:11013113-11013113 0
14 COSM3735032 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.1419+1G>T p.? 19:10991324-10991324 0
15 COSM3724497 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.3546+1G>A p.? 19:11030894-11030894 0
16 COSM1737872 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.3760G>T p.E1254* 19:11033503-11033503 0
17 COSM3735019 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.2362C>T p.Q788* 19:11013036-11013036 0
18 COSM1524017 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.2539C>T p.Q847* 19:11019624-11019624 0
19 COSM3724525 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.3565C>T p.R1189* 19:11033308-11033308 0
20 COSM3735034 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.1761+2T>A p.? 19:10996382-10996382 0
21 COSM3724491 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.3496C>T p.Q1166* 19:11030843-11030843 0
22 COSM3735016 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.1378C>T p.Q460* 19:10991282-10991282 0
23 COSM3724500 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.2859+1G>A p.? 19:11021968-11021968 0
24 COSM3735020 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.3676C>T p.Q1226* 19:11033419-11033419 0
25 COSM3724499 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.1761+1G>A p.? 19:10996381-10996381 0
26 COSM3735035 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.3168+1G>C p.? 19:11025509-11025509 0
27 COSM3735028 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.2915T>C p.L972P 19:11023573-11023573 0
28 COSM3735030 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.2275-1G>T p.? 19:11012948-11012948 0
29 COSM6493921 SMARCA4 ovary,NS,carcinoma,small cell carcinoma c.2973+1G>A p.? 19:11023632-11023632 0
30 COSM6942904 PTPRT ovary,NS,carcinoma,small cell carcinoma c.3823C>G p.L1275V 20:42098510-42098510 0
31 COSM6493919 POLE ovary,NS,carcinoma,small cell carcinoma c.861T>A p.D287E 12:132676594-132676594 0
32 COSM6942903 KMT2A ovary,NS,carcinoma,small cell carcinoma c.9005A>T p.D3002V 11:118504906-118504906 0
33 COSM6942902 ovary,NS,carcinoma,small cell carcinoma c.9014A>T p.D3005V 11:118504906-118504906 0
34 COSM99925 ovary,NS,carcinoma,small cell carcinoma c.844C>T p.R282W 17:7673776-7673776 0
35 COSM3378339 ovary,NS,carcinoma,small cell carcinoma c.844C>T p.R282W 17:7673776-7673776 0
36 COSM6942905 ovary,NS,carcinoma,small cell carcinoma c.3787C>G p.L1263V 20:42098510-42098510 0
37 COSM1636702 ovary,NS,carcinoma,small cell carcinoma c.844C>T p.R282W 17:7673776-7673776 0

Expression for Ovarian Small Cell Carcinoma

Search GEO for disease gene expression data for Ovarian Small Cell Carcinoma.

Pathways for Ovarian Small Cell Carcinoma

Pathways related to Ovarian Small Cell Carcinoma according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1 10.52 SMARCA4 SYP

GO Terms for Ovarian Small Cell Carcinoma

Cellular components related to Ovarian Small Cell Carcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 neuromuscular junction GO:0031594 8.62 DES SYP

Biological processes related to Ovarian Small Cell Carcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 positive regulation of pri-miRNA transcription by RNA polymerase II GO:1902895 8.62 SMARCA4 WT1

Molecular functions related to Ovarian Small Cell Carcinoma according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 p53 binding GO:0002039 8.62 MUC1 SMARCA4

Sources for Ovarian Small Cell Carcinoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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