Objective: Primitive neuroectodermal tumors PNET of the kidney are very rare tumors with aggressive behavior. They usually are associated with poor prognosis. Case: A 26-year old male patient presented to our clinic with right flank pain, microscopic hematuria and moderate-heavy nausea complaints. CT imaging studies showed a tumor with 17x13x18 cm dimensions. A mass excision was not possible caused by tumor spread to liver surface and duodenum and the tumor could not be separated from these surfaces. The operation was finished with an excisional biopsy. The patient received 7 cycles of alternating IE/VAC chemotherapy sessions. At 5th month of follow-up, PET-CT scans showed that the tumor was regressed to 8x6x7 cm dimensions. Following this outcome, radical nephrectomy was performed without risking any other organs. Conclusion: Neoadjuvant chemotherapy can be employed prior to radical nephrectomy in such cases in order to radically/efficiently/ drastically reduce the tumor mass
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