Primitive neuroectodermal tumor of kidney mimicking as an inflammatory renal mass

Abstract

Introduction: Renal primitive neuroectodermal tumor (PNET) is a rare and aggressive renal tumor with few reported cases in the literature.

Observations: We report a case of a 23-year-old male patient who initially presented with features of an inflammatory renal space occupying lesion (SOL) on clinical evaluation and imaging. Guided fine-needle aspiration cytology from renal mass revealed poorly differentiated neoplasm. Left open radical nephrectomy was performed. Final histopathology examination, despite the absence of clinical, radiological and gross features was consistent with a diagnosis of renal PNET. Such uncommon presentation of renal PNET has been rarely reported in the literature. Our patient then received six cycles of adjuvant chemotherapy (vincristine 1.5 mg/m² on day 1, doxorubicin 20 mg/m² on days 1–3, etoposide 150 mg/m² on days 1–3, and ifosfamide 3 g/m² on days 1–3 with mesna every 21 days). The patient developed multiorgan metastasis and progressive disease after remaining disease-free for 14 months.

Conclusion: Renal PNET should be kept in the differentials of a renal SOL presenting in adolescents and young adults. All diagnostic modalities concerning SOL of the kidney must be interpreted with caution in order for the appropriate management. Punctures for cytology can be indicated in select cases. Histopathology, immuno histochemistry supported by cytogenetic studies are required for the exact diagnosis of renal PNET. Multidisciplinary approach consisting of surgery, chemotherapy, and radiotherapy is recommended to manage this condition in view of its aggressive nature and poor prognosis.

Keywords: Renal; PNET; Imaging