Related Experiment Videos
Is primitive neuroectodermal tumor of the kidney a distinct entity?
C Rodriguez-Galindo1, N M Marina, B D Fletcher
1Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38105-2794, USA.
Cancer
|June 1, 1997
Summary
Primitive neuroectodermal tumors (PNETs) of the kidney are rare but distinct renal neoplasms. These tumors often present at advanced stages with poor treatment outcomes in young patients.
Area of Science:
- Oncology
- Pediatric Oncology
- Nephrology
Background:
- Primitive neuroectodermal tumors (PNETs) are neoplasms of presumed neuroectodermal origin.
- PNETs commonly manifest as bone or soft tissue masses in adolescents and young adults.
- Kidney PNETs are rare, with limited prior case reports.
Observation:
- Four pediatric patients with PNET of the kidney were identified and treated.
- Patients presented with unilateral renal masses and widespread metastatic disease.
- Metastatic sites included lymph nodes, lungs, bone, liver, and bone marrow.
Findings:
- Patients ranged from 4-20 years old.
- Treatment involved surgery, radiotherapy, and multiagent chemotherapy.
- Three of four patients succumbed to progressive disease within 14 months.
Implications:
- PNET of the kidney is a distinct clinicopathologic entity.
- It should be considered in the differential diagnosis of renal tumors in pediatric and young adult populations.
- The entity is associated with advanced disease at presentation and limited therapeutic response.