Tumor de células de Sertoli-Leydig do ovário
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| ID | DIS-SERTOLI-LEYDIG-OVARIAN |
|---|---|
| Tipo | Doença |
| Alegais | Androblastoma do ovárioTumor de células Sertoli-Leydig associado ao DICER1Tumor de estroma do cordão do sexo ovárico — Subtipo Sertoli-LeydigSLCTTumor de células de Sertoli-LeydigСертолі-Лейдиг клітинна пухлина яєчника |
| Estado | reviewed 2026-05-20 | pending_clinical_signoff |
| Doenças | Nenhuma declaração |
| Fontes | SRC-COG SRC-NCCN-PEDIATRIC-SARCOMA |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8631/1 |
|---|---|
| icd_o_3_topography | ["C56"] |
| icd_10 | C56 |
| who_classification | WHO Female Genital Tumours 5th ed (2020): Sertoli-Leydig cell tumour of the ovary; grading by Sertoli cell differentiation (well-differentiated → intermediate → poorly differentiated → retiform) with associated prognosis |
Notas
Ovarian Sertoli-Leydig cell tumor in any child / adolescent / young adult should trigger DICER1 germline testing — the proportion of DICER1-positive cases is high (~30-60%, higher in younger and poorly-differentiated cases). Diagnosis in a postmenopausal woman has a lower (though non-zero) DICER1-positive yield. Treatment: fertility-sparing unilateral salpingo-oophorectomy (USO) with staging for stage IA well-differentiated tumors in young women; total hysterectomy + bilateral salpingo-oophorectomy (TAH-BSO) for postmenopausal patients or high-grade / advanced-stage disease. Adjuvant chemotherapy (BEP — bleomycin, etoposide, cisplatin — similar to germ-cell tumour regimens) for stage IC and above, or poorly-differentiated / retiform variants. Androgen excess resolves dramatically post-surgery (virilization symptoms regress but voice deepening may be permanent). Lifelong DICER1-syndrome surveillance for germline-positive survivors: contralateral ovary US q6-12 months (synchronous and metachronous DICER1-driven SLCTs reported), chest imaging (PPB risk in survivors' offspring + own residual risk), thyroid US, and abdominal-pelvic US.
Usado por
Doença
DIS-CYSTIC-NEPHROMA- Nefroma císticaDIS-PINEOBLASTOMA- PineoblastomaDIS-PITUITARY-BLASTOMA- Blastoma de pituitáriaDIS-PLEUROPULMONARY-BLASTOMA- Blastoma pleuropulmonar (BPP)