SCCO
MCID: OVR058
MIFTS: 39

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 20 15 17 70
Small Cell Carcinoma of the Ovary 20 58
Small Cell Ovarian Carcinoma 58
Scco 58

Classifications:

Orphanet: 58  
Rare gynaecological and obstetric diseases


External Ids:

Disease Ontology 12 DOID:6179
NCIt 50 C27390
ICD10 via Orphanet 33 C56
Orphanet 58 ORPHA370396
UMLS 70 C2212006

Summaries for Ovarian Small Cell Carcinoma

GARD : 20 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 of the ovary of hypercalcemic type and small cell carcinoma, 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 are Neural Stem Cell Differentiation Pathways and Lineage-specific Markers and Transcription Ligand-dependent activation of the ESR1/SP pathway. The drugs Cisplatin and Etoposide have been mentioned in the context of this disorder. Affiliated tissues include ovary, breast and uterus, and related phenotypes are Increased shRNA abundance (Z-score > 2) and Increased shRNA abundance (Z-score > 2)

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 365)
# Related Disease Score Top Affiliating Genes
1 small-cell carcinoma of the ovary of hypercalcemic type 32.6 SMARCA4 MSANTD2
2 small cell carcinoma 31.0 SYP SMARCA4 MUC1 ENO2
3 spindle cell sarcoma 30.2 S100B MUC1 DES
4 cystic teratoma 30.2 SYP ENO2 CALB2
5 pleomorphic liposarcoma 30.0 S100B MUC1 INHA DES
6 juvenile type testicular granulosa cell tumor 29.9 WT1 SMARCB1 SMARCA4 CALB2
7 sex cord-gonadal stromal tumor 29.9 WT1 MUC1 INHA CALB2
8 large cell carcinoma 29.9 SYP PTHLH MUC1 ENO2
9 endometrial stromal sarcoma 29.9 S100B INHA DES
10 benign teratoma 29.9 SYP MUC1 ENO2 CALB2
11 teratoma 29.9 SYP SMARCB1 ENO2 CALB2
12 coffin-siris syndrome 1 29.9 SMARCB1 SMARCA4 DES ARID1A
13 neuroendocrine carcinoma 29.8 SYP S100B MUC1 ENO2
14 dysgerminoma 29.8 WT1 PTHLH INHA ENO2
15 atypical teratoid rhabdoid tumor 29.5 SYP SMARCB1 SMARCA4 S100B MUC1 DES
16 rhabdoid cancer 29.2 WT1 SYP SMARCB1 SMARCA4 MUC1 DES
17 desmoplastic small round cell tumor 29.1 WT1 SYP S100B MUC1 ENO2 DES
18 rhabdomyosarcoma 28.9 WT1 SYP SMARCB1 SMARCA4 S100B ENO2
19 pulmonary type ovarian small cell carcinoma 11.6
20 fallopian tube carcinosarcoma 10.4 WT1 CALB2
21 papillary thymic adenocarcinoma 10.4 SYP CALB2
22 epididymal neoplasm 10.3 WT1 CALB2
23 adrenogenital syndrome 10.3 SYP INHA
24 ovarian fibrothecoma 10.3 DES CALB2
25 testicular fibroma 10.3 WT1 CALB2
26 wolffian duct adenocarcinoma 10.3 WT1 CALB2
27 pericardium cancer 10.3 WT1 CALB2
28 ovarian germ cell teratoma 10.3 SYP CALB2
29 malignant biphasic mesothelioma 10.3 WT1 CALB2
30 medullomyoblastoma 10.3 SYP DES
31 esophageal neuroendocrine tumor 10.3 SYP MUC1
32 vulvar eccrine porocarcinoma 10.3 SYP MUC1
33 papillary ependymoma 10.3 SYP S100B
34 ovarian gonadoblastoma 10.3 WT1 CALB2
35 lung leiomyosarcoma 10.3 SYP CALB2
36 glomangioma 10.3 SYP DES
37 primary hepatic neuroendocrine carcinoma 10.3 SYP S100B
38 hypoganglionosis 10.3 SYP S100B
39 ovarian sex-cord stromal tumor 10.3 WT1 CALB2
40 epididymis adenocarcinoma 10.3 WT1 CALB2
41 epididymis cancer 10.3 WT1 CALB2
42 smarca4-deficient sarcoma of thorax 10.3 SMARCB1 SMARCA4
43 rosette-forming glioneuronal tumor 10.3 SYP S100B
44 biliary tract benign neoplasm 10.3 SYP MUC1
45 angiocentric glioma 10.3 SYP S100B
46 lung sarcomatoid carcinoma 10.3 MUC1 CALB2
47 keratocystic odontogenic tumor 10.3 PTHLH CALB2
48 pancreatic mucinous cystadenoma 10.3 MUC1 CALB2
49 parachordoma 10.3 MUC1 DES
50 pericardial mesothelioma 10.3 MUC1 CALB2

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

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

26 (show all 22)
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Increased shRNA abundance (Z-score > 2) GR00366-A-100 9.7 WT1
2 Increased shRNA abundance (Z-score > 2) GR00366-A-102 9.7 SMARCA4
3 Increased shRNA abundance (Z-score > 2) GR00366-A-11 9.7 MSANTD2
4 Increased shRNA abundance (Z-score > 2) GR00366-A-122 9.7 MSANTD2 WT1
5 Increased shRNA abundance (Z-score > 2) GR00366-A-145 9.7 WT1
6 Increased shRNA abundance (Z-score > 2) GR00366-A-152 9.7 WT1
7 Increased shRNA abundance (Z-score > 2) GR00366-A-169 9.7 MSANTD2
8 Increased shRNA abundance (Z-score > 2) GR00366-A-172 9.7 SMARCA4
9 Increased shRNA abundance (Z-score > 2) GR00366-A-176 9.7 SMARCB1
10 Increased shRNA abundance (Z-score > 2) GR00366-A-180 9.7 MSANTD2
11 Increased shRNA abundance (Z-score > 2) GR00366-A-194 9.7 SMARCB1
12 Increased shRNA abundance (Z-score > 2) GR00366-A-213 9.7 S100B
13 Increased shRNA abundance (Z-score > 2) GR00366-A-214 9.7 WT1
14 Increased shRNA abundance (Z-score > 2) GR00366-A-28 9.7 SMARCA4
15 Increased shRNA abundance (Z-score > 2) GR00366-A-30 9.7 WT1
16 Increased shRNA abundance (Z-score > 2) GR00366-A-43 9.7 WT1
17 Increased shRNA abundance (Z-score > 2) GR00366-A-47 9.7 WT1
18 Increased shRNA abundance (Z-score > 2) GR00366-A-48 9.7 S100B
19 Increased shRNA abundance (Z-score > 2) GR00366-A-66 9.7 S100B
20 Increased shRNA abundance (Z-score > 2) GR00366-A-75 9.7 SMARCB1
21 Increased shRNA abundance (Z-score > 2) GR00366-A-81 9.7 MSANTD2
22 Increased shRNA abundance (Z-score > 2) GR00366-A-85 9.7 MSANTD2 WT1

MGI Mouse Phenotypes related to Ovarian Small Cell Carcinoma:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 neoplasm MP:0002006 9.43 ARID1A INHA PTHLH SMARCA4 SMARCB1 WT1
2 normal MP:0002873 9.23 ARID1A CALB2 INHA PTHLH S100B SMARCA4

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
Cisplatin Approved Phase 2 15663-27-1 84093 441203 2767
2
Etoposide Approved Phase 2 33419-42-0 36462
3
Pembrolizumab Approved Phase 2 1374853-91-4
4 Antineoplastic Agents, Immunological Phase 2
5 Etoposide phosphate Phase 2

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Multicentric Non-randomized Phase II of Pembrolizumab in Combination With Etoposide-cisplatin-based Chemotherapy in First-line Advanced Small Cell Ovarian Carcinoma of Hypercalcemic Type Not yet recruiting NCT04602377 Phase 2 Pembrolizumab 25 MG/ML [Keytruda]

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:

40
Ovary, Breast, Uterus, Bone Marrow, Lymph Node, Cervix

Publications for Ovarian Small Cell Carcinoma

Articles related to Ovarian Small Cell Carcinoma:

(show top 50) (show all 235)
# Title Authors PMID Year
1
SMARCA4 (BRG1) and SMARCB1 (INI1) expression in TTF-1 negative neuroendocrine carcinomas including merkel cell carcinoma. 61
33581550 2021
2
Update on SWI/SNF-related gynecologic mesenchymal neoplasms: SMARCA4-deficient uterine sarcoma and SMARCB1-deficient vulvar neoplasms. 61
33252159 2021
3
Atypical teratoid/rhabdoid tumors (ATRTs) with SMARCA4 mutation are molecularly distinct from SMARCB1-deficient cases. 61
33331994 2021
4
Re-assigning the histologic identities of COV434 and TOV-112D ovarian cancer cell lines. 61
33328126 2021
5
Ovarian small cell carcinoma, hypercalcemic type: rare and aggressive tumor. 61
33397715 2021
6
Reprogramming of Nucleotide Metabolism Mediates Synergy between Epigenetic Therapy and MAP Kinase Inhibition. 61
33087508 2021
7
Re-expression of SMARCA4/BRG1 in small cell carcinoma of ovary, hypercalcemic type (SCCOHT) promotes an epithelial-like gene signature through an AP-1-dependent mechanism. 61
33355532 2020
8
Dose-intensive regimen treatment for small-cell carcinoma of the ovary of hypercalcemic type (SCCOHT). 61
32723678 2020
9
Small cell carcinoma of the ovary hypercalcemic type (SCCOHT): Comprehensive management of a newly diagnosed young adult. 61
32654763 2020
10
SWI/SNF-deficient malignancies of the female genital tract. 61
32978032 2020
11
Small-Cell Carcinoma of the Ovary, Hypercalcemic Type-Genetics, New Treatment Targets, and Current Management Guidelines. 61
32156746 2020
12
Small Cell Carcinoma of the Ovary: Clinicopathologic and Immunohistochemical Analysis of 7 New Cases of a Rare Malignancy. 61
32772748 2020
13
A Case of Lethal Abdominal Compartment Syndrome due to Rapidly Expanding Ovarian Small Cell Carcinoma Pulmonary Type. 61
32839683 2020
14
Arginine Depletion Therapy with ADI-PEG20 Limits Tumor Growth in Argininosuccinate Synthase-Deficient Ovarian Cancer, Including Small-Cell Carcinoma of the Ovary, Hypercalcemic Type. 61
32409304 2020
15
Primary ovarian small cell carcinoma of hypercalcemic type in a pregnant woman: A case report. 61
32791659 2020
16
ESGO-SIOPE guidelines for the management of adolescents and young adults with non-epithelial ovarian cancers. 61
32615119 2020
17
Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT) beyond SMARCA4 Mutations: A Comprehensive Genomic Analysis. 61
32575483 2020
18
Successful pregnancy following chemotherapy in a survivor of small cell carcinoma of the ovary, hypercalcemic type (SCCOHT): A case report and review of literature. 61
32405521 2020
19
Differential Diagnosis of Small Cell Carcinoma of the Ovary or Ovarian Metastases of Small Cell Carcinoma of the Lung: A Case Report and Review of the Literature. 61
32884525 2020
20
Optimal age for genetic cancer predisposition testing in hereditary SMARCA4 Ovarian Cancer Families: How young is too young? 61
32382648 2020
21
SMARCA family of genes. 61
32312722 2020
22
Germline mutations of SMARCA4 in small cell carcinoma of the ovary, hypercalcemic type and in SMARCA4-deficient undifferentiated uterine sarcoma: Clinical features of a single family and comparison of large cohorts. 61
31954538 2020
23
Loss of BRG1 (SMARCA4) Immunoexpression in a Pediatric Non-Central Nervous System Tumor Cohort. 61
31403913 2020
24
Clinical utility of SMARCA4 testing by immunohistochemistry in rare ovarian tumours. 61
31844183 2020
25
Rare case of remission of a patient with small cell carcinoma of the ovary, hypercalcaemic type (SCCOHT) stage IV: Case report. 61
31978720 2020
26
The novel reversible LSD1 inhibitor SP-2577 promotes anti-tumor immunity in SWItch/Sucrose-NonFermentable (SWI/SNF) complex mutated ovarian cancer. 61
32649682 2020
27
Mixed Serous and Clear Cell Adenocarcinoma of the Ovary Presenting with Symptomatic Hypercalcemia: A Case Report and Clinical Considerations. 61
32069206 2020
28
Small Cell Carcinoma of Ovary, Hypercalcemic Type (Malignant Rhabdoid Tumor of Ovary) with Loss of SMARCA4 (BRG1) Expression: Report of Two Cases. 61
32149361 2020
29
Combined CDK4/6 and PD-1 Inhibition in Refractory SMARCA4-Deficient Small-Cell Carcinoma of the Ovary, Hypercalcemic Type. 61
32704608 2020
30
[Pathological and molecular features of malignancies underlined by BAF complexes inactivation]. 61
31255411 2019
31
SMARCA4 inactivation defines a subset of undifferentiated uterine sarcomas with rhabdoid and small cell features and germline mutation association. 61
31190001 2019
32
Small-cell carcinoma-associated ovarian mucinous carcinoma: A case report and literature review. 61
31585810 2019
33
Cytologic diagnosis of small cell carcinoma of the ovary: A case report with review of the literature. 61
30821422 2019
34
Pembrolizumab-Induced Thyroiditis. 61
31111437 2019
35
The dilemma of early preventive oophorectomy in familial small cell carcinoma of the ovary of hypercalcemic type. 61
30886884 2019
36
Ovarian Small Cell Carcinoma of Hypercalcemic Type in an Adolescent Girl. 61
30956495 2019
37
Ovarian small cell carcinoma in one of a pair of monozygous twins. 61
30284660 2019
38
SMARCA4-deficient Thoracic Sarcomas: Clinicopathologic Study of 30 Cases With an Emphasis on Their Nosology and Differential Diagnoses. 61
30451731 2019
39
The ATPase module of mammalian SWI/SNF family complexes mediates subcomplex identity and catalytic activity-independent genomic targeting. 61
30858614 2019
40
CDK4/6 inhibitors target SMARCA4-determined cyclin D1 deficiency in hypercalcemic small cell carcinoma of the ovary. 61
30718512 2019
41
Small cell carcinoma of the ovary, hypercalcemic type (SCCOHT): a challenge for clinicopathological diagnosis. 61
31934039 2019
42
Genetic Causes of Rare Pediatric Ovarian Tumors. 61
31409083 2019
43
An In-Depth Look at Small Cell Carcinoma of the Ovary, Hypercalcemic Type (SCCOHT): Clinical Implications from Recent Molecular Findings. 61
30662543 2019
44
Histone Deacetylase Inhibitors Synergize with Catalytic Inhibitors of EZH2 to Exhibit Antitumor Activity in Small Cell Carcinoma of the Ovary, Hypercalcemic Type. 61
30232145 2018
45
SWI/SNF Complexes in Ovarian Cancer: Mechanistic Insights and Therapeutic Implications. 61
30037854 2018
46
Multimodal molecular analysis of an atypical small cell carcinoma of the ovary, hypercalcemic type. 61
30275002 2018
47
SMARCA4-deficient undifferentiated uterine sarcoma (malignant rhabdoid tumor of the uterus): a clinicopathologic entity distinct from undifferentiated carcinoma. 61
29700418 2018
48
Small Cell Carcinoma of the Ovary, Hypercalcemic Type: Clinical and Imaging Review. 61
28943050 2018
49
Pediatric Patients with Small-Cell Carcinoma of the Ovary Have Similar Survival to Adults: A Review of the National Cancer Database. 61
30269708 2018
50
Two types of small cell carcinoma of the ovary: Two typical case reports. 61
30094313 2018

Variations for Ovarian Small Cell Carcinoma

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 11.03 SYP SMARCA4 S100B
2
Show member pathways
10.64 SMARCB1 SMARCA4 ARID1A

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 SWI/SNF complex GO:0016514 9.33 SMARCB1 SMARCA4 ARID1A
2 brahma complex GO:0035060 9.26 SMARCB1 ARID1A
3 nBAF complex GO:0071565 9.13 SMARCB1 SMARCA4 ARID1A
4 npBAF complex GO:0071564 8.8 SMARCB1 SMARCA4 ARID1A

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

# Name GO ID Score Top Affiliating Genes
1 chromatin remodeling GO:0006338 9.58 SMARCB1 SMARCA4 ARID1A
2 positive regulation by host of viral transcription GO:0043923 9.43 SMARCB1 SMARCA4
3 positive regulation of transcription of nucleolar large rRNA by RNA polymerase I GO:1901838 9.37 SMARCB1 SMARCA4
4 RNA polymerase I preinitiation complex assembly GO:0001188 9.32 SMARCB1 SMARCA4
5 positive regulation of histone H4 acetylation GO:0090240 9.26 SMARCB1 MUC1
6 positive regulation of glucose mediated signaling pathway GO:1902661 9.16 SMARCB1 SMARCA4
7 ATP-dependent chromatin remodeling GO:0043044 9.13 SMARCB1 SMARCA4 ARID1A
8 nucleosome disassembly GO:0006337 8.8 SMARCB1 SMARCA4 ARID1A

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

# Name GO ID Score Top Affiliating Genes
1 Tat protein binding GO:0030957 9.16 SMARCB1 SMARCA4
2 RNA polymerase I CORE element sequence-specific DNA binding GO:0001164 8.96 SMARCB1 SMARCA4
3 p53 binding GO:0002039 8.8 SMARCB1 SMARCA4 MUC1

Sources for Ovarian Small Cell Carcinoma

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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