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Segmental cystic kidney tumours in children
Seppo Taskinen1, Jouko Lohi, Reetta Kivisaari
1Division of Pediatric Surgery, Helsinki University Hospital, Helsinki, Finland. seppo.taskinen@hus.fi
Scandinavian Journal of Urology and Nephrology
|December 9, 2009
Summary
Segmental cystic kidney tumors are rare in children. Diagnosis is challenging, but complete resection is crucial if a neoplastic tumor is suspected.
Area of Science:
- Pediatric Urology
- Pediatric Oncology
- Renal Pathology
Background:
- Segmental cystic renal tumors are exceptionally rare in pediatric populations.
- This study details the experience with these rare pediatric kidney tumors.
Observation:
- Four pediatric cases (3 female, 1 male) under 3 years old were identified.
- All patients underwent kidney-sparing surgery for segmental cystic tumors.
- Two cases were neoplastic (cystic nephroma, cystic nephroblastoma) and two were non-neoplastic (localized cystic disease, segmental ADPKD).
Findings:
- Neoplastic tumors exhibited a fibrous capsule on MRI and during surgery, distinguishing them from non-neoplastic lesions.
- Neoplastic cases were associated with other conditions: cystic pleuropulmonary blastoma and Muli-vey nanism.
- Non-neoplastic cases included localized cystic disease and segmental adult type autosomal dominant polycystic kidney disease.
Implications:
- Preoperative and perioperative diagnosis of segmental cystic kidney tumors in children presents significant challenges.
- Complete surgical resection is mandatory when a neoplastic etiology is suspected for these rare pediatric renal tumors.
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