Session Item

Renal primitive neuro-ectodermal tumour (rPNET)is a rare subset of extra skeletal ewings and presents as an aggressive clinical course and worse outcomes. We present our experience in the management of rPNET in terms of the clinical outcomes. 

Between June 2012 and June 2021, patient information was retrospectively evaluated and a total of 12 patients were assessed in terms of the disease characteristics, treatment delivered, clinical and toxicity outcomes. The survival outcomes were noted in the predefined proforma. 

With a median follow up 12 months among the patient cohort, majority of them were males (8/12; 66.7%) with a median age of 29.6 years (range 18 – 51 years). Among the presentations, flank pain and haematuria constituted the bulk of symptoms. The median tumor size was 12.6 cm (range, 4.8-26.4 cm). EWSR1 positivity was found in 6 patients and negative in 2 among the total 8 patients who were tested. The most common sites for metastases were bone, lung and liver with 6 patients presenting with distant metastases. Seven patients received neoadjuvant chemotherapy with VAC and the rest received it after surgery. Radiotherapy was given in six patients. On assessing the survival, 6 patients were alive and 5 died due to the disease metastases and one due to chemotoxicity. The median survival was 18.1 months. 

Renal PNETs are chemotherapy sensitive disease entity and radiation seems to play a role in multimodality treatment which combined with surgery can provide improved local control. The tumour is aggressive in nature with local recurrence and distant metastases. We need prospective studies to better understand the role of treatment modalities and the clinical outcomes.