Pediatric kidney tumours
This article cover pediatric kidney tumours. Adult kidney tumour are covered in kidney tumours article. An introduction to pediatric pathology is in the pediatric pathology article.
Overview
These are diagnoses pediatric pathologists make.
The most common ones are:
Other renal tumours (not covered in this article):
- Postneuroblastoma RCC.
- Neuroblastoma.
- PNET.
- Synovial sarcoma.
- Lymphoma.
The translocation carcinomas are covered in the kidney tumours article
Specific tumours
Wilms tumour
- AKA nephroblastoma, AKA Wilms' tumour.
Main article: Wilms tumour
Metanephric stromal tumour
- Abbreviated MST.
General
- Infants and children.
- Diagnosed as mesoblastic nephroma in the past.[1]
- Benign and rare.[2]
Gross
Features:[1]
- Usu. renal medulla.
- Solid or cystic.
Microscopic
Features:[1]
- "Collarettes" - tumour cells surround blood vessels or renal tubules.
- Spindle cells/stellate cells.
- Variable cellular density - imparts a nodular appearance at low power.
- Indistinct cell borders/cytoplasm.
- Induces epithelioid morphology in smooth muscle cells.
Notes:
- +/-Heterologous elements.
DDx:
- Clear cell sarcoma of the kidney.[2]
- Mesoblastic nephroma.
Images:
Metanephric adenofibroma
General
- Adults and children.
Microsopic
Features:[1]
- Has features of:
- Stromal component.
- Similar to metanephric stromal tumour.
- Epithelial nodules.
- Similar to metanephric adenoma.
- Stromal component.
Mesoblastic nephroma
General
- Almost exclusively in infants.
Subclassified:
- Classic.
- Cellular.
- Mixed.
Gross
- Renal sinus infiltration - common.
Microscopic
Classic
Features:[3]
- Spindle cells in fascicles.
- Infiltrative border.
Cellular
Features:[3]
- Plump cells with vesicular nuclei.
- Well-defined border.
- Mitotically active.
Mixed
- Like the name implies - both classic pattern and cellular pattern areas are present.[3]
Molecular
Cellular mesoblastic nephroma:
- t(12:15)(p13;q25) ETV6/NTRK3.
- Same translocation if found in infantile fibrosarcoma.
Clear cell sarcoma of the kidney
- Not to be confused with clear cell sarcoma.
General
- Common pediatric renal tumour - second only to Wilms tumour.
- Aggressive.
Gross
- Renal medulla.
- +/-Cystic.
Image:
Microscopic
Features:[3]
- Polygonal cells/Stellate cells.
- Indistinct cell borders/cytoplasm; clear background.
- No nucleoli.
Images:
IHC
Features:[4]
- Vimentin +ve.
- Desmin -ve.
- Smooth muscle actin -ve.
- NSE -ve.
- S-100 -ve.
Renal rhabdoid tumour
- AKA rhabdoid tumour of the kidney, abbreviated RTK.
General
- Similar to extrarenal malignant rhabdoid tumour.[3]
- Arises from renal medulla.
- May be associated with a CNS tumour.
Microscopic
- Variable architecture.
- Round cells.
- Abundant cytoplasm with eosinophilic inclusions.
- Eccentric vesicular nucleus.
- Prominent nucleolus -- key feature.
Images:
IHC
- INI1 -ve.
Renal cell carcinoma
General
- Similar to in adults.
- Clear cell RCC not common - a tumour with the clear cell histomorphology is more often a renal tumour with Xp11.2 translocation.
Papillary RCC
- Most common form of RCC in children.
Clear cell RCC
See also
References
- ↑ 1.0 1.1 1.2 1.3 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 283. ISBN 978-0781765275.
- ↑ 2.0 2.1 Palese, MA.; Ferrer, F.; Perlman, E.; Gearhart, JP. (Sep 2001). "Metanephric stromal tumor: a rare benign pediatric renal mass.". Urology 58 (3): 462. PMID 11549506.
- ↑ 3.0 3.1 3.2 3.3 3.4 3.5 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 284. ISBN 978-0781765275.
- ↑ Viswanathan, S.; Dave, BK.; Desai, SB. (Apr 2007). "Clear cell sarcoma of the kidney--a study of seven cases over a period of three years.". Indian J Pathol Microbiol 50 (2): 270-3. PMID 17883043.
- ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 627. ISBN 978-0781765275.