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Mixed germ cell-sex cord-stromal tumor

MedGen UID:
232941
•Concept ID:
C1321220
•
Neoplastic Process
Synonyms: Mixed Germ Cell-Sex Cord Neoplasm; mixed germ cell-Sex cord neoplasm; Mixed Germ Cell-Sex Cord Tumor; mixed germ cell-Sex cord tumor; mixed germ cell-Sex cord tumour; Mixed Germ Cell-Sex Cord-Stromal Neoplasm; mixed germ cell-Sex cord-stromal neoplasm; Mixed Germ Cell-Sex Cord-Stromal Tumor; mixed germ cell-sex cord-stromal tumor; mixed germ cell-Sex cord-stromal tumor; mixed germ cell-sex cord-stromal tumor (morphologic abnormality); mixed germ cell-Sex cord-stromal tumour; mixed germ cell-sex cord-stromal tumour (morphologic abnormality)
SNOMED CT: Mixed germ cell-sex cord-stromal tumor (406096006)
 
Monarch Initiative: MONDO:0002478

Definition

A biphasic neoplasm that arises from the ovary or the testis. It is characterized by the presence of neoplastic germ cells and neoplastic sex cord-stromal cells. It includes the gonadoblastoma and mixed germ cell-sex cord stromal tumor, unclassifiable. [from NCI]

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
Mixed germ cell-sex cord stromal tumor of the testis with an intratubular component: a problem in differential diagnosis.
Roth LM, Cheng L
Hum Pathol 2016 May;51:51-6. Epub 2016 Jan 7 doi: 10.1016/j.humpath.2015.12.016. PMID: 27067782

Recent clinical studies

Etiology

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
Novel insights into the mixed germ cell-sex cord stromal tumor of the testis: detection of chromosomal aneuploidy and further morphological evidence supporting the neoplastic nature of the germ cell component.
Michalova K, McKenney JK, Kristiansen G, Steiner P, Grossmann P, Putzova M, Martinek P, Chottova-Dvorakova M, Michal M, Hes O, Michal M
Virchows Arch 2020 Nov;477(5):615-623. Epub 2020 May 23 doi: 10.1007/s00428-020-02843-3. PMID: 32447491
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

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
Mixed germ cell-sex cord stromal tumor of the testis with an intratubular component: a problem in differential diagnosis.
Roth LM, Cheng L
Hum Pathol 2016 May;51:51-6. Epub 2016 Jan 7 doi: 10.1016/j.humpath.2015.12.016. PMID: 27067782
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
A mixed germ cell-sex cord-stromal tumor of the ovary with retiform tubular structure: a case report.
Tokuoka S, Aoki Y, Hayashi Y, Yokoyama T, Ishii T
Int J Gynecol Pathol 1985;4(2):161-70. doi: 10.1097/00004347-198506000-00008. PMID: 2991152

Prognosis

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
Neoplastic potential of germ cells in relation to disturbances of gonadal organogenesis and changes in karyotype.
Słowikowska-Hilczer J, Romer TE, Kula K
J Androl 2003 Mar-Apr;24(2):270-8. doi: 10.1002/j.1939-4640.2003.tb02672.x. PMID: 12634315

Clinical prediction guides

Mixed germ cell-sex cord stromal tumor of the testis with an intratubular component: a problem in differential diagnosis.
Roth LM, Cheng L
Hum Pathol 2016 May;51:51-6. Epub 2016 Jan 7 doi: 10.1016/j.humpath.2015.12.016. PMID: 27067782
Monosomy 22 in a mixed germ cell-sex cord-stromal tumor of the ovary.
Speleman F, Dermaut B, De Potter CR, Van Gele M, Van Roy N, De Paepe A, Laureys G
Genes Chromosomes Cancer 1997 Jul;19(3):192-4. PMID: 9219001

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