Pediatric kidney tumours

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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:

  1. Wilms tumour.
  2. Clear cell sarcoma of the kidney.

Other renal tumours (not covered in this article):

The renal translocation carcinomas are covered in:

Specific tumours

Wilms tumour

  • AKA nephroblastoma, AKA Wilms' tumour.

Metanephric stromal tumour

  • Abbreviated MST.

General

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:

Images:

Metanephric adenofibroma

General

  • Adults and children.

Microsopic

Features:[1]

Mesoblastic nephroma

General

  • Almost exclusively in infants.

Subclassified:

  1. Classic.
  2. Cellular.
  3. Mixed.

Gross

  • Renal sinus infiltration - common.

Microscopic

Classic

Features:[3]

  • Spindle cells in fascicles.
  • Infiltrative border.

Cellular

Features:[3]

Mixed

  • Like the name implies - both classic pattern and cellular pattern areas are present.[3]

Molecular

Cellular mesoblastic nephroma:[4]

Clear cell sarcoma of the kidney

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:[5]

  • Vimentin +ve.
  • Desmin -ve.
  • Smooth muscle actin -ve.
  • NSE -ve.
  • S-100 -ve.

Molecular

  • t(10;17)(q22;p13) - reported in 6 of 50 cases.[6]

Renal rhabdoid tumour

  • AKA rhabdoid tumour of the kidney, abbreviated RTK.

Renal cell carcinoma

General

Papillary RCC

See: (adult) renal cell carcinoma.
  • Most common form of RCC in children.

Clear cell RCC

See: (adult) renal cell carcinoma.

See also

References

  1. 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. 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. 3.0 3.1 3.2 3.3 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.
  4. Rudzinski, ER.; Lockwood, CM.; Stohr, BA.; Vargas, SO.; Sheridan, R.; Black, JO.; Rajaram, V.; Laetsch, TW. et al. (Jul 2018). "Pan-Trk Immunohistochemistry Identifies NTRK Rearrangements in Pediatric Mesenchymal Tumors.". Am J Surg Pathol 42 (7): 927-935. doi:10.1097/PAS.0000000000001062. PMID 29683818.
  5. 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.
  6. O'Meara, E.; Stack, D.; Lee, CH.; Garvin, AJ.; Morris, T.; Argani, P.; Han, JS.; Karlsson, J. et al. (May 2012). "Characterization of the chromosomal translocation t(10;17)(q22;p13) in clear cell sarcoma of kidney.". J Pathol 227 (1): 72-80. doi:10.1002/path.3985. PMID 22294382.