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Renal Tumors of Childhood: Radiologic-Pathologic Correlation Part 2. The 2nd Decade: From the Radiologic Pathology

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Renal cell carcinoma (RCC) is more common than Wilms tumor in older children and adolescents. Understanding diverse pediatric renal tumor subtypes and their imaging features aids accurate diagnosis.

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Area of Science:

  • Pediatric Oncology
  • Radiology
  • Pathology

Background:

  • Malignant renal tumors are 7% of childhood cancers, with Wilms tumors dominating in younger children.
  • In older children and adolescents, renal cell carcinoma (RCC) surpasses Wilms tumor in incidence.
  • Pediatric RCC subtypes differ significantly from adult forms.

Purpose of the Study:

  • To differentiate pediatric renal tumors based on clinical, biologic, and histopathologic features.
  • To correlate these features with imaging findings for preoperative diagnosis.
  • To provide radiologists with a differential diagnosis for renal masses in older children and adolescents.

Main Methods:

  • Review of clinical presentation and histopathology of various pediatric renal tumors.
  • Analysis of imaging characteristics (CT, MRI) associated with specific subtypes.
  • Correlation of imaging findings with known biologic and histopathologic features.

Main Results:

  • Xp11.2 translocation RCC is the most common pediatric subtype, showing hyperattenuation and lymphadenopathy.
  • Papillary RCC presents with hypointensity and calcification; clear cell RCC is associated with von Hippel-Lindau disease.
  • Other entities include medullary carcinoma (sickle cell trait), angiomyolipoma (tuberous sclerosis), metanephric tumors, lymphoma, and PNET, each with distinct imaging signatures.

Conclusions:

  • Recognizing the unique histopathologic spectrum of pediatric renal tumors is crucial.
  • Specific imaging findings can suggest particular subtypes like Xp11.2 translocation RCC or papillary RCC.
  • Accurate preoperative differential diagnosis of pediatric renal masses relies on integrating clinical, histopathologic, and imaging data.