SCCO
MCID: OVR058
MIFTS: 38

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 53 15 73
Small Cell Carcinoma of the Ovary 53 59
Small Cell Ovarian Carcinoma 59
Scco 59

Classifications:



External Ids:

Disease Ontology 12 DOID:6179
NCIt 50 C27390
Orphanet 59 ORPHA370396
ICD10 via Orphanet 34 C56
UMLS 73 C2212006

Summaries for Ovarian Small Cell Carcinoma

NIH Rare Diseases : 53 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, lymph node and pituitary, and related phenotypes are liver/biliary system and neoplasm

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 82)
# Related Disease Score Top Affiliating Genes
1 small cell carcinoma 31.1 SMARCA4 SYP
2 atypical teratoid rhabdoid tumor 30.0 SMARCA4 SYP
3 pleomorphic liposarcoma 29.8 INHA 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 12.5
7 rhabdoid tumor predisposition syndrome 2 11.1
8 childhood ovarian cancer 11.1
9 ovarian cancer 1 10.3
10 ovarian cancer 10.2
11 angiomyoma 10.1 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 chordoid meningioma 10.1 MUC1 SYP
18 clear cell ependymoma 10.1 MUC1 SYP
19 brachydactyly, type e1 10.1 MUC1 PTHLH
20 ependymoma 10.1 MUC1 SYP
21 melanotic neuroectodermal tumor 10.1 MUC1 SYP
22 pancreatoblastoma 10.1 MUC1 SYP
23 perivascular epithelioid cell tumor 10.1 MUC1 SYP
24 mucinous ovarian cystadenoma 10.1 MUC1 SYP
25 anaplastic ependymoma 10.1 MUC1 SYP
26 extraskeletal ewing sarcoma 10.1 MUC1 SYP
27 papilloma of choroid plexus 10.1 MUC1 SYP
28 sclerosing hemangioma 10.0 MUC1 SYP
29 cerebral primitive neuroectodermal tumor 10.0 MUC1 SYP
30 gastric tubular adenocarcinoma 10.0 MUC1 SYP
31 peritoneal serous adenocarcinoma 10.0 SYP WT1
32 tubular adenocarcinoma 10.0 MUC1 SYP
33 endosalpingiosis 10.0 MUC1 WT1
34 mixed ductal-endocrine carcinoma 10.0 MUC1 SYP
35 cauda equina neoplasm 10.0 SYP WT1
36 venous hemangioma 10.0 SYP WT1
37 male reproductive organ benign neoplasm 10.0 SYP WT1
38 optic nerve glioma 10.0 MUC1 SYP
39 spindle cell carcinoma 10.0 MUC1 SYP
40 dysgerminoma of ovary 10.0 PTHLH WT1
41 adenosquamous carcinoma 10.0 MUC1 PTHLH
42 sertoli-leydig cell tumor 10.0 MUC1 WT1
43 ovarian mucinous neoplasm 10.0 SYP WT1
44 large cell carcinoma 10.0 PTHLH SYP
45 peritoneal mesothelioma 10.0 MUC1 WT1
46 neurofibroma 10.0 MUC1 SYP
47 cavernous hemangioma 10.0 MUC1 SYP
48 chondrosarcoma, extraskeletal myxoid 10.0 SYP WT1
49 oncocytoma 10.0 MUC1 SYP
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

MGI Mouse Phenotypes related to Ovarian Small Cell Carcinoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 liver/biliary system MP:0005370 9.46 INHA PTHLH SMARCA4 WT1
2 neoplasm MP:0002006 9.26 INHA PTHLH SMARCA4 WT1
3 normal MP:0002873 9.02 INHA PTHLH SMARCA4 SYP WT1

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

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:

41
Ovary, Lymph Node, Pituitary, Thymus, Pineal, Bone, Bone Marrow

Publications for Ovarian Small Cell Carcinoma

Articles related to Ovarian Small Cell Carcinoma:

(show top 50) (show all 85)
# Title Authors Year
1
Ovarian Small Cell Carcinoma: A Rare Case Report and Review of Literature. ( 29731803 )
2018
2
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
3
Ovarian small cell carcinoma in one of a pair of monozygous twins. ( 30284660 )
2018
4
Small Cell Carcinoma of the Ovary: A Rare Tumor With a Poor Prognosis. ( 29621125 )
2018
5
Two types of small cell carcinoma of the ovary: Two typical case reports. ( 30094313 )
2018
6
Histone Deacetylase Inhibitors Synergize with Catalytic Inhibitors of EZH2 to Exhibit Antitumor Activity in Small Cell Carcinoma of the Ovary, Hypercalcemic Type. ( 30232145 )
2018
7
Pediatric Patients with Small-Cell Carcinoma of the Ovary Have Similar Survival to Adults: A Review of the National Cancer Database. ( 30269708 )
2018
8
Multimodal molecular analysis of an atypical small cell carcinoma of the ovary, hypercalcemic type. ( 30275002 )
2018
9
Pediatric Patients with Small-Cell Carcinoma of the Ovary Have Similar Survival to Adults: A Review of the National Cancer Database. ( 30454499 )
2018
10
SMARCA4 inactivating mutations cause concomitant Coffin-Siris syndrome, microphthalmia and small-cell carcinoma of the ovary hypercalcaemic type. ( 28608987 )
2017
11
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
12
Small cell carcinoma of the ovary hypercalcemic type (SCCOHT): A rare case after in vitro fertilization (IVF). ( 28367208 )
2017
13
The histone methyltransferase EZH2 is a therapeutic target in small cell carcinoma of the ovary, hypercalcaemic type. ( 28444909 )
2017
14
F-18 Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography Imaging for Diagnosing Ovarian Small Cell Carcinoma of the Hypercalcemic Type. ( 26941866 )
2016
15
A case of ovarian small cell carcinoma of the pulmonary type that was observed as it developed. ( 29787016 )
2016
16
Ovarian Small Cell Carcinoma Hypercalcemic Type: A Case Report >. ( 28124855 )
2016
17
Ovarian small-cell carcinoma hypercalcemic type successfully treated: a case report and literature review. ( 27042109 )
2016
18
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
19
Dual loss of the SWI/SNF complex ATPases SMARCA4/BRG1 and SMARCA2/BRM is highly sensitive and specific for small cell carcinoma of the ovary, hypercalcaemic type. ( 26356327 )
2016
20
Concomitant loss of SMARCA2 and SMARCA4 expression in small cell carcinoma of the ovary, hypercalcemic type. ( 26564006 )
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
Successful treatment of preadolescents with small cell carcinoma of the ovary hypercalcemic type. ( 23459374 )
2013
27
Primary ovarian small cell carcinoma of pulmonary type with enlarged paraaortic lymph node masses: a case report and review of the literature. ( 22873108 )
2012
28
Ovarian small cell carcinoma complicated by carcinomatous meningitis. ( 22826783 )
2012
29
Ovarian small cell carcinoma of hypercalcaemic-type: history of the disease in a patient who refused treatment. ( 21534770 )
2011
30
Ovarian small cell carcinoma, hypercalcemic type exhibiting a response to high-dose chemotherapy. ( 21117517 )
2010
31
Hypercalcemic-type ovarian small cell carcinoma with unique CD34 expression. ( 19788625 )
2009
32
Clinical management of ovarian small-cell carcinoma of the hypercalcemic type: a proposal for conservative surgery in an advanced stage of disease. ( 19407558 )
2009
33
Familial small cell carcinoma of the ovary. ( 19621450 )
2009
34
Ovarian small cell carcinoma and cytokeratin perinuclear dotlike staining. ( 18227717 )
2008
35
Large cell variant ovarian small cell carcinoma: case report. ( 18958794 )
2008
36
A case of pulmonary type of ovarian small cell carcinoma. ( 17441897 )
2007
37
Complete remission of ovarian small cell carcinoma treated with irinotecan and cisplatin: a case report. ( 17695433 )
2007
38
Ovarian small cell carcinoma of the hypercalcemic type in children and adolescents: a prognostically unfavorable but curable disease. ( 16998935 )
2006
39
Recurrent small cell carcinoma of the ovary treated with docetaxel: A case report. ( 17009997 )
2006
40
Advanced small cell carcinoma of the ovary in a pediatric patient with long-term survival and review of the literature. ( 15750452 )
2005
41
Small cell carcinoma of the ovary with hypercalcemia and ectopic parathyroid hormone production. ( 15794681 )
2005
42
Small cell carcinoma of the ovary presenting as bone marrow metastasis: a rare presenting feature. ( 16761770 )
2005
43
Primary ovarian small cell carcinoma of the pulmonary type: a case report and review of the literature. ( 15032292 )
2004
44
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
45
Small cell carcinoma of the ovary with hypercalcemia causes severe pancreatitis and altered mental status. ( 15581988 )
2004
46
Small cell carcinoma of the ovary: a report of three cases and review of the literature. ( 14766457 )
2004
47
Stage IIIC small cell carcinoma of the ovary: survival with conservative surgery and chemotherapy. ( 15121630 )
2004
48
Pathological case of the month. Acute symptomatic hypercalcemia associated with ovarian small cell carcinoma. ( 11772197 )
2002
49
Ovarian small cell carcinoma: a rare neoplasm in a 15-year-old female. ( 10888454 )
2000
50
Large-cell variant of small cell carcinoma of the ovary with hypercalcaemia. ( 11129517 )
2000

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

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 extracellular space GO:0005615 8.92 INHA MUC1 PTHLH SMARCA4

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

# Name GO ID Score Top Affiliating Genes
1 negative regulation of cell growth GO:0030308 9.16 SMARCA4 WT1
2 male gonad development GO:0008584 8.96 INHA WT1
3 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 hormone activity GO:0005179 8.96 INHA PTHLH
2 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 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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