Kidney Flashcards
Which patients with wilms tumor can have surgery only ?
The COG AREN0532 study showed that patients <2 years old with stage I favorable histology Wilms tumor weighing less than 550 grams have 90% event-free survival and 100% overall survival with surgery only and not adjuvant chemotherapy.
So just observe them!
How to managed RCC which is completly resected?
FISH evaluation showed a translocation involving TFE3, which is characteristic of translocation-RCC, the most common RCC subtype in children and adolescents.
For patients with completely resected disease, the standard treatment is observation without adjuvant therapy. This is true even for patients with lymph node involvement.
The COG AREN0321 study showed that pediatric and adolescent patients with stage III RCC with lymph node involvement had 80% event-free survival without adjuvant therapy.
For distant metastatic or unresectable disease, the agent of choice would be a tyrosine kinase inhibitor such as sunitinib, axitinib or cabozantinib +/- an immune checkpoint inhibitor.
Screening for BWS
Beckwith-Wiedemann syndrome (BWS). This is an overgrowth syndrome in which patients present with macroglossia, abdominal wall defects, unilateral overgrowth (hemihypertrophy), and enlarged organs.
These patients are at risk for embryonal tumors such as Wilms’ tumor and hepatoblastoma. Of genetic alterations associated with BWS, hypermethylation of 11p15 has the highest associated risk for developing Wilms’ tumor.
It is recommended that this screening US every 3 months and continue through age 7 years.
Vincristine, doxorubicin, cyclophosphamide, etoposide, and carboplatin.
Anaplastic wilms tumor
Clear cell sarcoma of kidneys.
BCOR internal tandem duplication is the most common molecular finding in clear cell sarcoma of the kidney (CCSK); about 90% of such tumors have this molecular finding.
CCSK has a distinct metastatic pattern compared to Wilms tumor characterized by spread to bone and brain. Although metastasis is uncommon at initial diagnosis, the brain and bone are common sites of recurrence, and it is important to assess these sites with baseline imaging.
