raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:363489

Sex cord-stromal tumor of testis

Synonyms: Testicular sex cord-stromal tumor

Type
Disease
Group
Disorder
Inheritance
Not applicable
Age of onset
Adolescent, Adult
Status
active

A group of rare tumors of testis comprising neoplasms of pure sex cord or pure stromal type, or neoplasms with admixtures of both components in various proportions and degree of differentiation. The tumors usually present as a painless testicular mass, although some may be associated with endocrine manifestations such as precocious puberty, gynecomastia, or erectile dysfunction. Malignant behavior is seen only in a small percentage of these tumors.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enSex cord-stromal tumor of testisTesticular sex cord-stromal tumor
frTumeur testiculaire des cordons sexuels et du stroma
deKeimstrang-Stromatumor, testikulärerKeimstrang-Stromatumor des Hodens
esTumor testicular de los cordones sexuales
itTumore testicolare dello stroma e dei cordoni sessualiTumore dello stroma e dei cordoni sessuali del testicolo
nlSex cord-stromale tumor van testisSex cord-stromale tumor van teelbal · Testiculaire sex cord-stromale tumor
ptTumor testicular do estroma dos cordões sexuais
plGuz jądra ze sznurów płciowych i podścieliska= en
csSex cord stromální nádory varlatTestikulární sex cord stromální nádor
ukПухлина сімяного канатика-стромальна пухлина яєчкаСтромальна пухлина сімяного канатика яєчка
zh睾丸性索间质瘤睾丸性索间质肿瘤
trTestisin seks kord stromal tümörüTestis cinsiyet kord stromal tümörü

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10D40.1BTNTattributedvalidated
MONDO0003125Evalidated
UMLSC3840076Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

23 records
TypeClassValueAreaQualificationValidationSource
Annual incidence<1 / 1 000 0000.02 / 100 000EuropeValue and classValidated22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]
Lifetime Prevalence1-9 / 1 000 0000.44 / 100 000EuropeValue and classValidated22033323[PMID]_RARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.049 / 100 000AustriaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.048 / 100 000BelgiumValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.01 / 100 000BulgariaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.034 / 100 000CroatiaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.016 / 100 000Czech RepublicValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.018 / 100 000EstoniaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.012 / 100 000FinlandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.02 / 100 000GermanyValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.018 / 100 000IrelandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.027 / 100 000ItalyValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.016 / 100 000LatviaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.008 / 100 000LithuaniaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.022 / 100 000NorwayValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.013 / 100 000PolandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.01 / 100 000PortugalValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.037 / 100 000SlovakiaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.006 / 100 000SloveniaValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.021 / 100 000SpainValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.023 / 100 000SwitzerlandValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.012 / 100 000NetherlandsValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]
Annual incidence<1 / 1 000 0000.023 / 100 000United KingdomValue and classValidatedRARECARE surveillance of rare cancers in Europe[OTHER]

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card