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[Primitive neuroectodermal tumor of kidney : a case report]
Naofumi Tsutsumi1, Takayuki Sumiyoshi, Motohiro Okamura
1The Department of Urology, Osaka Red Cross Hospital, Japan.
Hinyokika Kiyo. Acta Urologica Japonica
|July 6, 2013
Summary
A rare pediatric kidney cancer, primitive neuroectodermal tumor (PNET), presented as asymptomatic hematuria. Despite aggressive treatment, the patient developed metastases and died 18 months after diagnosis.
Area of Science:
- Pediatric Oncology
- Nephrology
- Medical Genetics
Background:
- Primitive neuroectodermal tumors (PNETs) are rare, aggressive malignancies.
- Kidney PNETs are exceptionally uncommon, particularly in adolescents.
- Early diagnosis and prompt treatment are crucial for managing aggressive pediatric cancers.
Observation:
- A 16-year-old male presented with asymptomatic gross hematuria, initially with normal ultrasound findings.
- Recurrent hematuria and severe back pain led to CT, revealing a large right renal tumor with inferior vena cava thrombus.
- Pathological diagnosis confirmed kidney PNET via immunostaining and gene analysis.
Findings:
- The patient underwent radical nephrectomy and embolectomy, followed by adjuvant chemotherapy.
- Pulmonary metastases were detected 10 months post-operation.
- Combined modality therapy (salvage chemotherapy, surgery, irradiation) was administered.
Implications:
- This case highlights the aggressive nature of kidney PNET in adolescents.
- It underscores the importance of thorough investigation for recurrent hematuria, even after initial normal findings.
- The limited efficacy of current treatment modalities necessitates research into novel therapeutic strategies for advanced kidney PNET.
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