Results of Multimodal Treatment of Two Patients with Thoracic Primitive Neuroectodermal Tumor. Is Surgery Really Helpful for Survival?

Introduction

Primitive neuroectodermal tumor (PNET) was first described by Askin et al. [[1]]. Previously, it was thought to originate from peripheral nerves and was referred to as neuroepithelioma [[2]]. Although the tumor was classified among the thoracic autonomic neurogenic tumors by Askin et al. [[1]], it does not produce biologically active substances which can be detected in blood or urine. PNET are classified as belonging to the group of malignant tumors with small round cells [[3]]. Most patients with PNET are adolescents or young adults, and the majority of them are younger than 30 years of age [[2]]. Despite combined chemoradiotherapy and surgical resection, the prognosis is generally poor due to local recurrence and distant metastases [[4]].

We report on two cases with PNET located in the thorax and present an overview of the clinical findings, treatment choices and outcomes for thoracic PNET on the basis of the data of two patients treated in our clinic.

References

• 1 Askin F B, Rosai J, Sibley R K, Dehner L P, McAlister W H. Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis.  Cancer. 1979;  43 2438-2451
• 2 Weiss S W, Goldblum J R. Primitive neuroectodermal tumors and related lesions. Weiss SW, Goldblum JR Soft Tissue Tumors. 4th edn. St. Louis, MI; Mosby Inc. 2001: 1265-1323
• 3 Dehner L P. Primitive neuroectodermal tumor and Ewing's sarcoma.  Am J Surg Pathol. 1993;  17 1-13
• 4 Schulman H, Newman-Heinman N, Kurtzbart E, Maor E, Zirkin H, Laufer L. Thoracoabdominal peripheral primitive neuroectodermal tumors in childhood: radiological features.  Eur Radiol. 2000;  10 1649-1652
• 5 Jurgens H, Bier V, Harms D, Beck J, Brandeis W, Etspuler G, Gadner H, Schmidt D, Treuner J, Winkler K. Malignant peripheral neuroectodermal tumors: a retrospective analysis of 42 patients.  Cancer. 1988;  61 349-357
• 6 Hage R, Duurkens V A, Seldenrijk C A, Brutel de la Riviere A, van Swieten H A, van den Bosch J M. Primitive neuroectodermal tumor: report of two cases and review of the literature.  J Thorac Cardiovasc Surg. 2002;  124 833-836
• 7 Kushner B H, Hajdu S I, Gulati S C, Erlandson R A, Exelby P R, Lieberman P H. Extracranial primitive neuroectodermal tumors: the Memorial Sloan-Kettering Cancer Center experience.  Cancer. 1991;  67 1825-1829