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Ovarian Mixed Germ Cell-Sex Cord-Stromal Tumor

MedGen UID:
277457
•Concept ID:
C1335166
•
Neoplastic Process
Synonyms: Ovarian Germ Cell-Sex Cord-Stromal Tumor; Ovarian Mixed Germ Cell-Sex Cord Neoplasm; Ovarian Mixed Germ Cell-Sex Cord Tumor; Ovarian Mixed Germ Cell-Sex Cord-Stromal Neoplasm

Definition

A neoplasm that arises from the ovary and is characterized by the presence of germ cell and sex cord-stromal tissues that are intimately admixed. If there is no malignant germ cell component present, the clinical course is benign. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVOvarian Mixed Germ Cell-Sex Cord-Stromal Tumor

Professional guidelines

PubMed

Gonadoblastoma versus ovarian mixed germ cell-sex cord stromal tumor in women or girls with no evidence of a disorder of sex development: A problem in differential diagnosis.
Roth LM, Czernobilsky B, Mann SA, Cheng L
Pathol Res Pract 2020 Nov;216(11):153198. Epub 2020 Sep 5 doi: 10.1016/j.prp.2020.153198. PMID: 33002849

Recent clinical studies

Etiology

Clinical Challenges in the Management of Malignant Ovarian Germ Cell Tumours.
Saani I, Raj N, Sood R, Ansari S, Mandviwala HA, Sanchez E, Boussios S
Int J Environ Res Public Health 2023 Jun 9;20(12) doi: 10.3390/ijerph20126089. PMID: 37372675Free PMC Article
Gonadoblastoma versus ovarian mixed germ cell-sex cord stromal tumor in women or girls with no evidence of a disorder of sex development: A problem in differential diagnosis.
Roth LM, Czernobilsky B, Mann SA, Cheng L
Pathol Res Pract 2020 Nov;216(11):153198. Epub 2020 Sep 5 doi: 10.1016/j.prp.2020.153198. PMID: 33002849
Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391
Pathobiology of ovarian carcinomas.
Devouassoux-Shisheboran M, Genestie C
Chin J Cancer 2015 Jan;34(1):50-5. doi: 10.5732/cjc.014.10273. PMID: 25556618Free PMC Article
Sex cord-stromal tumors of the ovary and testis: their similarities and differences with consideration of selected problems.
Young RH
Mod Pathol 2005 Feb;18 Suppl 2:S81-98. doi: 10.1038/modpathol.3800311. PMID: 15502809

Diagnosis

Clinical Challenges in the Management of Malignant Ovarian Germ Cell Tumours.
Saani I, Raj N, Sood R, Ansari S, Mandviwala HA, Sanchez E, Boussios S
Int J Environ Res Public Health 2023 Jun 9;20(12) doi: 10.3390/ijerph20126089. PMID: 37372675Free PMC Article
Gonadoblastoma versus ovarian mixed germ cell-sex cord stromal tumor in women or girls with no evidence of a disorder of sex development: A problem in differential diagnosis.
Roth LM, Czernobilsky B, Mann SA, Cheng L
Pathol Res Pract 2020 Nov;216(11):153198. Epub 2020 Sep 5 doi: 10.1016/j.prp.2020.153198. PMID: 33002849
Perspectives on testicular sex cord-stromal tumors and those composed of both germ cells and sex cord-stromal derivatives with a comparison to corresponding ovarian neoplasms.
Roth LM, Lyu B, Cheng L
Hum Pathol 2017 Jul;65:1-14. Epub 2017 Apr 23 doi: 10.1016/j.humpath.2017.04.009. PMID: 28445692
Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391
Sex cord-stromal tumors of the ovary and testis: their similarities and differences with consideration of selected problems.
Young RH
Mod Pathol 2005 Feb;18 Suppl 2:S81-98. doi: 10.1038/modpathol.3800311. PMID: 15502809

Therapy

A retrospective study of reproductive outcomes after fertility-sparing surgery and postoperative adjuvant chemotherapy in malignant ovarian germ cell tumors and sex cord-stromal tumors.
Zhang N, Chen R, Hua K, Zhang Y
J Ovarian Res 2017 Jul 27;10(1):52. doi: 10.1186/s13048-017-0348-x. PMID: 28750644Free PMC Article
Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391
Malignant ovarian germ cell tumors: a single-institution experience.
Cicin I, Eralp Y, Saip P, Ayan I, Kebudi R, Iyibozkurt C, Tuzlali S, Gorgun O, Topuz E
Am J Clin Oncol 2009 Apr;32(2):191-6. doi: 10.1097/COC.0b013e3181841f2e. PMID: 19307952
Prognostic factors and role of salvage surgery in chemorefractory ovarian germ cell malignancies: a study in Chinese patients.
Li J, Yang W, Wu X
Gynecol Oncol 2007 Jun;105(3):769-75. Epub 2007 Apr 24 doi: 10.1016/j.ygyno.2007.02.032. PMID: 17459461

Prognosis

Depression and anxiety in women with malignant ovarian germ cell (MOGCT) and sex cord stromal tumors (SCST): an analysis of the AGO-CORSETT database.
Bossart M, Plett H, Krämer B, Braicu E, Czogalla B, Klar M, Singer S, Mayr D, Staebler A, du Bois A, Kommoss S, Link T, Burges A, Heitz F, Grube M, Trillsch F, Harter P, Wimberger P, Buderath P, Hasenburg A
Arch Gynecol Obstet 2023 Apr;307(4):1155-1162. Epub 2022 Sep 21 doi: 10.1007/s00404-022-06781-0. PMID: 36127525Free PMC Article
A retrospective study of reproductive outcomes after fertility-sparing surgery and postoperative adjuvant chemotherapy in malignant ovarian germ cell tumors and sex cord-stromal tumors.
Zhang N, Chen R, Hua K, Zhang Y
J Ovarian Res 2017 Jul 27;10(1):52. doi: 10.1186/s13048-017-0348-x. PMID: 28750644Free PMC Article
Sex-Cord Stromal Tumors in Children and Teenagers: Results of the TGM-95 Study.
Fresneau B, Orbach D, Faure-Conter C, Verité C, Castex MP, Kalfa N, Martelli H, Patte C
Pediatr Blood Cancer 2015 Dec;62(12):2114-9. Epub 2015 Jul 23 doi: 10.1002/pbc.25614. PMID: 26206391
Malignant ovarian germ cell tumors: a single-institution experience.
Cicin I, Eralp Y, Saip P, Ayan I, Kebudi R, Iyibozkurt C, Tuzlali S, Gorgun O, Topuz E
Am J Clin Oncol 2009 Apr;32(2):191-6. doi: 10.1097/COC.0b013e3181841f2e. PMID: 19307952
Sex cord-stromal tumors of the ovary and testis: their similarities and differences with consideration of selected problems.
Young RH
Mod Pathol 2005 Feb;18 Suppl 2:S81-98. doi: 10.1038/modpathol.3800311. PMID: 15502809

Clinical prediction guides

Depression and anxiety in women with malignant ovarian germ cell (MOGCT) and sex cord stromal tumors (SCST): an analysis of the AGO-CORSETT database.
Bossart M, Plett H, Krämer B, Braicu E, Czogalla B, Klar M, Singer S, Mayr D, Staebler A, du Bois A, Kommoss S, Link T, Burges A, Heitz F, Grube M, Trillsch F, Harter P, Wimberger P, Buderath P, Hasenburg A
Arch Gynecol Obstet 2023 Apr;307(4):1155-1162. Epub 2022 Sep 21 doi: 10.1007/s00404-022-06781-0. PMID: 36127525Free PMC Article
The effect of fertility-sparing surgery on sexuality and global quality of life in women with malignant ovarian germ cell and sex cord stromal tumors: an analysis of the CORSETT database of the AGO study group.
Hasenburg A, Plett H, Krämer B, Braicu E, Czogalla B, Bossart M, Singer S, Mayr D, Staebler A, du Bois A, Kommoss S, Link T, Burges A, Heitz F, Keul J, Trillsch F, Harter P, Wimberger P, Buderath P, Klar M
Arch Gynecol Obstet 2021 Dec;304(6):1541-1549. Epub 2021 Jul 21 doi: 10.1007/s00404-021-06019-5. PMID: 34287678Free PMC Article
Germ-line deletion in DICER1 revealed by a novel MLPA assay using synthetic oligonucleotides.
Sabbaghian N, Srivastava A, Hamel N, Plourde F, Gajtko-Metera M, Niedziela M, Foulkes WD
Eur J Hum Genet 2014 Apr;22(4):564-7. Epub 2013 Sep 25 doi: 10.1038/ejhg.2013.215. PMID: 24065110Free PMC Article
Fine needle aspiration cytology in ovarian lesions: an institutional experience of 584 cases.
Gupta N, Rajwanshi A, Dhaliwal LK, Khandelwal N, Dey P, Srinivasan R, Nijhawan R
Cytopathology 2012 Oct;23(5):300-7. Epub 2011 Aug 25 doi: 10.1111/j.1365-2303.2011.00896.x. PMID: 21883539
Malignant ovarian germ cell tumors: a single-institution experience.
Cicin I, Eralp Y, Saip P, Ayan I, Kebudi R, Iyibozkurt C, Tuzlali S, Gorgun O, Topuz E
Am J Clin Oncol 2009 Apr;32(2):191-6. doi: 10.1097/COC.0b013e3181841f2e. PMID: 19307952

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