Retroperitoneal primitive neuroectodermal tumour (PNET). A case report and review of the literature

Abstract

Purpose We report a clinical case and present a brief review of the literature of peripheral primitive neuroectodermal tumour (PNET) as a rare disease. We discuss the difficult clinical and pathological diagnosis and the multidisciplinary approach to treatment of PNET. We debate radiosensitivity of extracranial recurrent retroperitoneal PNET. Methods and materials External beam radiation therapy was applied for a non-resectable local recurrence of retroperitoneal PNET in a 74-year-old woman. There were no distant metastases and our patient has refused chemotherapy. Results Local tumour control (LTC) was achieved after administration of a total dose of 60 Gy in 30 fractions by external beam 60 Cobalt radiotherapy. Conclusions PNET is an aggressive malignant tumour infiltrating lymphatics and metastasizing haematogenously. It requires a multimodality treatment. Late local recurrence of extracranial retroperitoneal PNET has shown high radiosensitivity, so local tumour irradiation could be a radical treatment even in non-resectable cases.