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Primitive Neuroectodermal Tumor (PNET) of the kidney: a case report
Giorgio Pomara1, Francesco Cappello, Maria G Cuttano
1Department of Surgery, Urology Section S, Chiara Hospital, University of Pisa, Pisa, Italy. g.pomara@libero.it
BMC Cancer
|February 19, 2004
Summary
A rare case of kidney Primitive Neuroectodermal Tumor (PNET) in a young woman showed aggressive behavior but was successfully treated with surgery. Complete remission was observed at 24 months, highlighting effective management strategies for this uncommon cancer.
Area of Science:
- Nephrology
- Oncology
- Pathology
Background:
- Primitive Neuroectodermal Tumor (PNET) of the kidney is a rare and aggressive malignancy.
- Few cases are documented, often presenting with nonspecific symptoms.
- This report details a unique case in a 27-year-old female.
Observation:
- A radical nephrectomy with lymphadenectomy was performed.
- Immunohistochemistry revealed diffuse CD99 positivity.
- Other markers (Pankeratin, cytokeratin AE1/AE3, vimentin, desmin, S100, cromogranin) were negative.
Findings:
- The patient achieved no residual or recurrent tumor at 24-month follow-up.
- Diagnosis was confirmed by clinical presentation, macroscopic appearance, histological pattern, and immunophenotype.
- CD99 positivity is a key diagnostic indicator for PNET.
Implications:
- Accurate diagnosis of renal PNET is crucial due to its aggressive nature.
- Distinguishing PNET from Ewing's sarcoma is challenging but important for prognosis.
- Successful surgical management offers a positive outlook for patients with renal PNET.