Vol. 5, Issue 7 (2019)
Primary neuroectodermal tumors of kidney: Our experience at institute of kidney disease and research centre-institute of transplantation sciences
Author(s): Dr. Raghuveer Machiraju, Dr. Sayeed Jamal Rizvi, Dr. Pranjal R Modi
Abstract: Objectives: Primary Neuroectodermal Tumours (PNET) are rare and highly aggressive tumours. These tumours are diagnosed by means of histopathology and aided by Immunohistochemistry and cytogenetics. Multimodality treatment is needed for treating these tumours. The purpose of this study is to review our experience in diagnosis and treatment PNET of kidney. Materials and Methods: We retrospectively reviewed the data of all the patients treated for PNET of kidney since January 2016 to till July 2018 at our institute. Results: A total of six pts have been treated for PNET of kidney. Five were females and one male. Out of six patients, three had venous involvement. All the pts underwent radical nephrectomy and also IVC thrombectomy in three cases. Diagnosis of PNET made by HPE and supported by IHC. Post-surgery all six pts were given chemotherapy and two of them were given radiotherapy as well. All the six are alive till date and with no recurrence till date. Conclusions: In general, renal PNET is a highly aggressive and have poor prognosis. In adolescents and young adults with a suspicious renal mass, a diagnosis of renal PNET should be always considered. The golden standard to diagnosis renal PNET is based on histologic and immune histochemical features, supported by cytogenetic analysis. Multimodality treatment consisting of surgery, chemotherapy and radiotherapy is recommended to manage the disease.
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