GPR: 2019 - Week 41

From MGH Learn Pathology

Clinical history

3 year old female with abdominal pain and 10.1 cm mass of R kidney


GPR19-33 -Wilms tumor.jpg


Registered users must be logged in to access the interactive quiz.


GPR19-33 -Wilms tumor.jpg

 
Wilms Tumor (Nephroblastoma)
  • 98% of cases in children < 10 years of age
  • Peak incidence: 2-3 years
  • Overall survival >90%
  • Most tumors are unicentric
  • Usually sharply demarcated from surrounding parenchyma
  • Very often encapsulated
  • Often uniformly pale gray or tan with soft consistency
  • More whorled or firm if prominent stromal component
  • Hemorrhage, necrosis, cysts and lobular pattern are common
 

GPR19-33 -Malignant rhabdoid tumor.png

 

Malignant Rhabdoid Tumor

  • Highly malignant pediatric renal tumor with very poor prognosis
  • Mortality rate > 80% within 2 yrs of diagnosis
  • Mean age 1 yr
  • Typically large tumor (mean 9.6 cm)
  • Well circumscribed but non-encapsulated with ill-defined borders
  • Fleshy tumor often with hemorrhage and necrosis
(Photo credit-ExpertPath)
 

GPR19-33 -mesoblastic nephroma-gross-93-8231.jpg

 

Congenital Mesoblastic Nephroma

  • Spindle cell neoplasm of the kidney
  • Most common renal tumor in the first 3 months of life
  • Rarely occurs in children >2 yrs
  • Majority cured with surgery and have excellent outcome
  • Solitary, unilateral
  • Whorled or trabeculated gray/white cut surface
  • Indistinct tumor-kidney interface
  • May see hemorrhage, cysts or necrosis
 

GPR19-33 -cystic nephroma.jpg

 

Cystic Nephroma

  • Benign encapsulated neoplasm composed entirely of epithelial lined cysts and thin cyst septa
  • Pediatric lesion associated with DICER1 mutations
  • Typically occurs in those <4 yrs
  • Well circumscribed, encapsulated, diffusely cystic containing clear serous fluid
  • Solid areas may represent sarcomatous transformation
 



This week's Gross Pathology Roundup was presented by Kristine Marie Chaudet, MD on Mon, Oct 07, 2019.