Cystic nephroma

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

ID	`DIS-CYSTIC-NEPHROMA`
Type	Disease
Aliases	Cystic partially differentiated nephroblastoma (when overlap with Wilms-spectrum considered)DICER1-associated cystic nephromaMultilocular cystic nephromaPediatric cystic nephromaКістозна нефрома
Status	reviewed 2026-05-20 \| pending_clinical_signoff
Diseases	None declared
Sources	`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.

Source YAML

icd_o_3_morphology	8959/1
icd_o_3_topography	["C64"]
icd_10	D30.0
who_classification	WHO 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)

Capabilities

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).

Used By

Disease

DIS-PINEOBLASTOMA - Pineoblastoma
DIS-PITUITARY-BLASTOMA - Pituitary blastoma
DIS-PLEUROPULMONARY-BLASTOMA - Pleuropulmonary blastoma (PPB)
DIS-SERTOLI-LEYDIG-OVARIAN - Sertoli-Leydig cell tumor of the ovary

Sources

SRC-SCHULTZ-DICER1-2018 - DICER1 and Associated Conditions: Identification of At-Risk Individuals and Recommended S...
SRC-STEWART-DICER1-REVIEW-2019 - Neoplasm Risk Among Individuals With a Pathogenic Germline Variant in DICER1