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Retroperitoneal primitive neuroectodermal tumour (PNET). A case report and review of the literature
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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[[ce:hsp sp="0.25"/]]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.
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[[ce:hsp sp="0.25"/]]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.
Keywords
Primitive neuroectodermal tumour; Neuroectodermal tumour; External beam radiotherapy; Multimodality treatment; Radiosensitivity
About this articleTitle
Retroperitoneal primitive neuroectodermal tumour (PNET). A case report and review of the literature
Journal
Reports of Practical Oncology and Radiotherapy
Issue
Pages
221-224
Published online
2009-11-01
DOI
10.1016/j.rpor.2009.11.002
Bibliographic record
Rep Pract Oncol Radiother 2009;14(6):221-224.
Keywords
Primitive neuroectodermal tumour
Neuroectodermal tumour
External beam radiotherapy
Multimodality treatment
Radiosensitivity
Authors
Lena Marinova