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Metastatic primitive neuroectodermal tumor of the kidney in adults
R Casella1, H Moch, C Rochlitz
1Urologic Clinic, University Hospital, Basel, Switzerland. Rcasella@www.uro.bcm.tm.edu
Objective:
Primitive neuroectodermal tumors (PNET) of the kidney are rare and highly aggressive malignancies. The purpose of our study was to present information about the management of patients with metastatic disease.
Methods:
The records of 2 patients (30-year-old female and 32-year-old male) with metastatic PNET of the kidney were reviewed and our data compared with the literature.
Results:
Neither clinical evaluation nor radiological methods allowed to distinguish PNET from renal cell carcinoma. Immunohistochemistry revealed strong positivity for CD99 in tumor 1 and weak positivity for NSE and vimentin in both tumors. In tumor 2, EWS/FLI1 translocation was detected by RT-PCR. Patient 1 underwent nephrectomy, seven cycles of polychemotherapy, two cycles of high-dose chemotherapy, autologous bone marrow rescue, radiotherapy of suspicious skeletal foci and is without evidence of recurrent disease 28 months after therapy. Patient 2 underwent six cycles of polychemotherapy, nephrectomy, high-dose chemotherapy with cyclophosphamide and abdominal radiotherapy. Because of relapse high-dose chemotherapy with stem cell rescue was not performed. He underwent three further cycles of polychemotherapy and died one year after diagnosis due to cerebral metastasis.
Conclusions:
The diagnosis of renal PNET must be considered in young patients with renal neoplasm, particularly those with advanced disease at presentation. Achieving exact diagnosis has important clinical consequences because polychemotherapy and high-dose chemotherapy may lead to dramatic tumor reduction or even complete remission.
Insights
Primitive neuroectodermal tumors (PNET) of the kidney are rare. Early diagnosis and aggressive treatment, including chemotherapy, are crucial for managing metastatic kidney PNET, offering potential for remission.
Area of Science:
- Oncology
- Pathology
- Medical Research
Background:
- Primitive neuroectodermal tumors (PNET) of the kidney are exceptionally rare and aggressive cancers.
- Metastatic disease presentation poses significant management challenges.
Observation:
- Clinical and radiological evaluations could not reliably differentiate renal PNET from renal cell carcinoma.
- Immunohistochemistry (CD99, NSE, vimentin) and RT-PCR (EWS/FLI1 translocation) were vital for accurate diagnosis.
- Two patients with metastatic renal PNET received varied treatment regimens, including surgery, polychemotherapy, and high-dose chemotherapy.
Findings:
- One patient achieved remission 28 months post-therapy with a comprehensive treatment plan.
- The other patient succumbed to cerebral metastasis despite aggressive treatment, highlighting the disease's severity.
- Treatment response varied significantly, with complete remission possible but relapse a considerable risk.
Implications:
- Renal PNET should be suspected in young patients with renal neoplasms, especially those presenting with advanced disease.
- Accurate diagnosis is critical, as intensive chemotherapy regimens can induce significant tumor reduction or complete remission.
- Further research into optimal management strategies for metastatic renal PNET is warranted.