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Malignant Testicular Sex Cord-Stromal Tumor

MedGen UID:
271770
•Concept ID:
C1515289
•
Neoplastic Process

Definition

A malignant sex cord-gonadal stromal tumor that arises from the testis. It is characterized by cellular pleomorphism, anaplastic features, increased mitotic activity, and vascular invasion. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant Testicular Sex Cord-Stromal Tumor

Professional guidelines

PubMed

Rare benign and malignant testicular lesions: histopathology and management.
Kern SQ, Speir RW, Akgul M, Cary C
Curr Opin Urol 2020 Mar;30(2):235-244. doi: 10.1097/MOU.0000000000000715. PMID: 31922967
Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841
Gynaecomastia--pathophysiology, diagnosis and treatment.
Narula HS, Carlson HE
Nat Rev Endocrinol 2014 Nov;10(11):684-98. Epub 2014 Aug 12 doi: 10.1038/nrendo.2014.139. PMID: 25112235

Recent clinical studies

Etiology

Testicular Tumors: A Contemporary Update on Morphologic, Immunohistochemical and Molecular Features.
Al-Obaidy KI, Idrees MT
Adv Anat Pathol 2021 Jul 1;28(4):258-275. doi: 10.1097/PAP.0000000000000302. PMID: 33871428
Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841
Androgen-Secreting Ovarian Tumors.
Macut D, Ilić D, Mitrović Jovanović A, Bjekić-Macut J
Front Horm Res 2019;53:100-107. Epub 2019 Sep 9 doi: 10.1159/000494906. PMID: 31499493
DICER1 and Associated Conditions: Identification of At-risk Individuals and Recommended Surveillance Strategies.
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
Testicular Seminoma and Its Mimics: From the Radiologic Pathology Archives.
Marko J, Wolfman DJ, Aubin AL, Sesterhenn IA
Radiographics 2017 Jul-Aug;37(4):1085-1098. Epub 2017 Jun 2 doi: 10.1148/rg.2017160164. PMID: 28574809Free PMC Article

Diagnosis

Testicular Tumors: New Developments in Germ Cell and Sex Cord Stromal Tumors.
Dashora A, Wagner T, Berney DM
Surg Pathol Clin 2022 Dec;15(4):729-743. Epub 2022 Oct 13 doi: 10.1016/j.path.2022.07.007. PMID: 36344186
An introduction to the WHO 5th edition 2022 classification of testicular tumours.
Berney DM, Cree I, Rao V, Moch H, Srigley JR, Tsuzuki T, Amin MB, Comperat EM, Hartmann A, Menon S, Netto GJ, Rubin MA, Turajlic S, Raspollini MR, Tickoo SK
Histopathology 2022 Oct;81(4):459-466. Epub 2022 Jul 1 doi: 10.1111/his.14675. PMID: 35502823Free PMC Article
Testicular Tumors: A Contemporary Update on Morphologic, Immunohistochemical and Molecular Features.
Al-Obaidy KI, Idrees MT
Adv Anat Pathol 2021 Jul 1;28(4):258-275. doi: 10.1097/PAP.0000000000000302. PMID: 33871428
Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841
Testicular Seminoma and Its Mimics: From the Radiologic Pathology Archives.
Marko J, Wolfman DJ, Aubin AL, Sesterhenn IA
Radiographics 2017 Jul-Aug;37(4):1085-1098. Epub 2017 Jun 2 doi: 10.1148/rg.2017160164. PMID: 28574809Free PMC Article

Therapy

Testicular tumors of adrenogenital syndrome: From physiopathology to therapy.
Naouar S, Braiek S, El Kamel R
Presse Med 2017 Jun;46(6 Pt 1):572-578. Epub 2017 May 23 doi: 10.1016/j.lpm.2017.05.006. PMID: 28549629
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
Testicular sex cord stromal tumors: analysis of patients from the MAKEI study.
Hofmann M, Schlegel PG, Hippert F, Schmidt P, von-Schweinitz D, Leuschner I, Göbel U, Calaminus G, Schneider DT; MAKEI study group
Pediatr Blood Cancer 2013 Oct;60(10):1651-5. Epub 2013 Jun 3 doi: 10.1002/pbc.24607. PMID: 23733594
Chemotherapy of ovarian germ cell tumors and sex cord stromal tumors.
Gershenson DM
Semin Surg Oncol 1994 Jul-Aug;10(4):290-8. doi: 10.1002/ssu.2980100410. PMID: 8091071
Testicular tumours.
Peckham MJ, McElwain TJ
Clin Endocrinol Metab 1975 Nov;4(3):665-92. doi: 10.1016/s0300-595x(75)80052-1. PMID: 58746

Prognosis

An introduction to the WHO 5th edition 2022 classification of testicular tumours.
Berney DM, Cree I, Rao V, Moch H, Srigley JR, Tsuzuki T, Amin MB, Comperat EM, Hartmann A, Menon S, Netto GJ, Rubin MA, Turajlic S, Raspollini MR, Tickoo SK
Histopathology 2022 Oct;81(4):459-466. Epub 2022 Jul 1 doi: 10.1111/his.14675. PMID: 35502823Free PMC Article
Promising Immunotherapy in Metastatic Testicular Sex Cord Stromal Tumours After First-Line Chemotherapy.
Shang B, Cao C, Jiang W, Shi H, Bi X, Cui C, Shou J, Zheng S, Zhang J, Zhou A, Li C, Ma J
Front Immunol 2021;12:720359. Epub 2022 Jan 10 doi: 10.3389/fimmu.2021.720359. PMID: 35082775Free PMC Article
Rare benign and malignant testicular lesions: histopathology and management.
Kern SQ, Speir RW, Akgul M, Cary C
Curr Opin Urol 2020 Mar;30(2):235-244. doi: 10.1097/MOU.0000000000000715. PMID: 31922967
Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841
Ovarian Sex Cord-Stromal Tumors.
Schultz KA, Harris AK, Schneider DT, Young RH, Brown J, Gershenson DM, Dehner LP, Hill DA, Messinger YH, Frazier AL
J Oncol Pract 2016 Oct;12(10):940-946. doi: 10.1200/JOP.2016.016261. PMID: 27858560Free PMC Article

Clinical prediction guides

Risk Factors and Treatment Outcomes of 1,375 Patients with Testicular Leydig Cell Tumors: Analysis of Published Case Series Data.
Fankhauser CD, Grogg JB, Hayoz S, Wettstein MS, Dieckmann KP, Sulser T, Bode PK, Clarke NW, Beyer J, Hermanns T
J Urol 2020 May;203(5):949-956. Epub 2019 Dec 17 doi: 10.1097/JU.0000000000000705. PMID: 31845841
Ovarian Sex Cord-Stromal Tumors.
Schultz KA, Harris AK, Schneider DT, Young RH, Brown J, Gershenson DM, Dehner LP, Hill DA, Messinger YH, Frazier AL
J Oncol Pract 2016 Oct;12(10):940-946. doi: 10.1200/JOP.2016.016261. PMID: 27858560Free PMC Article
Clinical outcomes of local and metastatic testicular sex cord-stromal tumors.
Silberstein JL, Bazzi WM, Vertosick E, Carver BS, Bosl GJ, Feldman DR, Bajorin DF, Motzer RJ, Al-Ahmadie H, Reuter VE, Sheinfeld J
J Urol 2014 Aug;192(2):415-9. Epub 2014 Feb 8 doi: 10.1016/j.juro.2014.01.104. PMID: 24518791Free PMC Article
Testicular and testicular adnexa tumors in the elderly.
Gigantino V, La Mantia E, Franco R, Cecere S, Rossetti S, Di Napoli M, Pisano C, Berretta M, Galzerano A, Botti G, Pignata S, Facchini G
Anticancer Drugs 2013 Mar;24(3):228-36. doi: 10.1097/CAD.0b013e32835a3598. PMID: 23059385
The toxicology of perfluorooctanoate.
Kennedy GL Jr, Butenhoff JL, Olsen GW, O'Connor JC, Seacat AM, Perkins RG, Biegel LB, Murphy SR, Farrar DG
Crit Rev Toxicol 2004 Jul-Aug;34(4):351-84. doi: 10.1080/10408440490464705. PMID: 15328768

Recent systematic reviews

Testis-sparing surgery in children with testicular tumors: A systematic review and meta-analysis.
Miao X, Li Y, Zhou T, Lv M
Asian J Surg 2021 Dec;44(12):1503-1509. Epub 2021 Apr 21 doi: 10.1016/j.asjsur.2021.03.016. PMID: 33893031
Testis-sparing surgery for testicular tumors in children: a 20 year single center experience and systematic review of the literature.
Bois JI, Vagni RL, de Badiola FI, Moldes JM, Losty PD, Lobos PA
Pediatr Surg Int 2021 May;37(5):607-616. Epub 2021 Jan 17 doi: 10.1007/s00383-020-04850-6. PMID: 33454815
Myoid gonadal tumor. Case series, systematic review, and Bayesian analysis.
Renne SL, Valeri M, Tosoni A, Bertolotti A, Rossi R, Renne G, Colecchia M
Virchows Arch 2021 Apr;478(4):727-734. Epub 2020 Nov 2 doi: 10.1007/s00428-020-02957-8. PMID: 33140129
Risk factors and treatment outcomes of 239 patients with testicular granulosa cell tumors: a systematic review of published case series data.
Grogg JB, Schneider K, Bode PK, Kranzbühler B, Eberli D, Sulser T, Beyer J, Lorch A, Hermanns T, Fankhauser CD
J Cancer Res Clin Oncol 2020 Nov;146(11):2829-2841. Epub 2020 Jul 27 doi: 10.1007/s00432-020-03326-3. PMID: 32719989Free PMC Article
Sertoli Cell Tumors of the Testes: Systematic Literature Review and Meta-Analysis of Outcomes in 435 Patients.
Grogg J, Schneider K, Bode PK, Kranzbühler B, Eberli D, Sulser T, Lorch A, Beyer J, Hermanns T, Fankhauser CD
Oncologist 2020 Jul;25(7):585-590. Epub 2020 Feb 11 doi: 10.1634/theoncologist.2019-0692. PMID: 32043680Free PMC Article

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