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Néphrome kystique

Vue déterministe de l'entité source de la LJAM. L'autorité clinique demeure avec les ID de la source cités et l'état d'approbation de l ́examinateur.

IDDIS-CYSTIC-NEPHROMA
TypeMaladie
Alias
Néphroblastome kystique partiellement différencié (lorsque le chevauchement avec le spectre Wilms est considéré)Néphrome kystique associé à DICER1Néphrome cystique multiloculaireNéphrome cystique pédiatriqueКістозна нефрома
Étatreviewed 2026-05-20 | pending_clinical_signoff
MaladiesAucune déclaration
SourcesSRC-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.

Source YAML

icd_o_3_morphology8959/1
icd_o_3_topography["C64"]
icd_10D30.0
who_classificationWHO Urinary and Male Genital Tumours 5th ed (2022): Cystic nephroma — benign / borderline (Grade-low) multilocular cystic kidney lesion of childhood; differential with cystic partially differentiated nephroblastoma (Wilms-spectrum)

Capacités

Notes

Pediatric cystic nephroma is a strong DICER1-syndrome signal — diagnosis in any child should trigger DICER1 germline testing AND cascade-testing of parents + siblings. Beckwith-Wiedemann syndrome is a separate (non- DICER1) etiology that shares the Wilms-spectrum abdominal-tumour surveillance protocol (abdominal US q3-4 months from birth to age 7; AFP q2-3 months from birth to age 4 — focused on hepatoblastoma). Treatment: maximal safe surgical resection (nephron-sparing partial nephrectomy when anatomically feasible; total nephrectomy if not). Histopathology after resection is critical — distinguishes pure cystic nephroma (benign-borderline, surveillance only) from cystic partially differentiated nephroblastoma (Wilms-spectrum, requires adjuvant chemo per stage) and from progression to anaplastic sarcoma of the kidney (high-grade malignant, requires multimodal therapy). Lifelong DICER1-syndrome surveillance for germline-positive survivors: chest imaging (PPB risk), abdominal-pelvic US (ovarian, Sertoli-Leydig risk in girls), thyroid US, and CNS imaging (pineoblastoma, pituitary blastoma risk under age 5).

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Maladie

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