Zystisches Nephrom

Deterministische Ansicht der Quelle YAML Entität. Klinische Autorität bleibt mit den zitierten Quell-IDs und Rezensenten Sign-off Zustand.

ID	`DIS-CYSTIC-NEPHROMA`
Art	Erkrankung
Aliase	Zystisches teilweise differenziertes Nephroblastom (bei Überlappung mit Wilms-Spektrum)DICER1-assoziiertes zystisches NephromMultilokules zystisches NephromaKinderzystisches NephromaКістозна нефрома
Status	reviewed 2026-05-20 \| pending_clinical_signoff
Erkrankungen	None declared
Quellen	`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.

Quelle: YAML

icd_o_3_morphology	8959/1
icd_o_3_topography	["C64"]
icd_10	D30.0
who_classification	WHO Harn- und männliche Genitaltumore 5. ed (2022): Zystisches Nephroma — gutartig/borderline (Grade-low) multilocular zystische Nierenläsion der Kindheit; Differential mit zystischer teilweise differenzierter Nephroblastom (Wilms-Spektrum)

Fähigkeiten

Anmerkungen

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).

Verwendet von

Erkrankung

DIS-PINEOBLASTOMA - Pineoblastom
DIS-PITUITARY-BLASTOMA - Hypophysisches Blastom
DIS-PLEUROPULMONARY-BLASTOMA - Pleuropulmonales Blastom (PPB)
DIS-SERTOLI-LEYDIG-OVARIAN - Sertoli-Leydig Zelltumor des Eierstocks

Quellen

SRC-SCHULTZ-DICER1-2018 - DICER1 und damit verbundene Bedingungen: Identifizierung von At-Risk-Personen und empfohlene S...
SRC-STEWART-DICER1-REVIEW-2019 - Neoplasma-Risiko bei Personen mit einer pathogenen Germline-Variante in DICER1