PEDIATRICS CLINICAL RESEARCH
Authors: J. C. Murray, N. Rainusso, R. A. Roberts, A. M. Gomez, R. Egler, H. Russell, M. F. Okcu, S. Gururangan, J. Fangusaro, T. Young-Poussaint, S. Lesh, A. Onar, R. Gilbertson, R. Packer, R. McLendon, H. S. Friedman, J. Boyett, L. E. Kun, R. Venkatramani, K. Haley, F. Gilles, R. Sposto, L. Ji, R. Olshefski, J. Garvin, T. Tekautz, G. Kennedy, R. Rassekh, T. Moore, S. Gardner, J. Allen, R. Shore, C. Moertel, M. Atlas, J. Lasky, J. Finlay, E. T. Valera, M. S. Brassesco, C. A. Scrideli, R. S. Oliveira, H. R. Machado, L. G. Tone, J. L. Finlay, S. Kreimer, J. Dagri, J. Grimm, S. Bluml, B. Britt, G. Dhall, F. Gilles, J. L. Finlay, R. J. Brown, G. Dhall, A. Shah, N. Kapoor, H. Abdel-Azim, A. A. N. Rao, D. Wallace, J. Boyett, A. Gajjar, R. J. Packer, M. L. Pearlman, Z. Sadighi, R. Bingham, T. Vats, S. Khatua, R. H. Ko, S. O'Neil, R. S. Lavey, J. L. Finlay, G. Dhall, T. B. Davidson, F. Gilles, J. Tovar, J. Grimm, K. Wong, A. Olch, G. Dhall, J. L. Finlay, J. C. Murray, J. H. Honeycutt, D. J. Donahue, H. W. Head, A. J. Alles, A. Ray, M. Pearlman, T. Vats, S. Khatua, J. Baskin, I. Qaddoumi, M. S. Ahchu, S. F. Alabi, I. C. Arambu, M. Castellanos, Y. Gamboa, R. Martinez, M. Montero, E. Ocampo, S. C. Howard, J. L. Finlay, A. Broniscer, S. D. Baker, J. N. Baker, A. P. Panandiker, A. Onar-Thomas, T. K. Chin, T. E. Merchant, A. Davidoff, S. C. Kaste, A. Gajjar, C. F. Stewart, J. Espinoza, K. Haley, N. Patel, G. Dhall, S. Gardner, A. Jeffrey, J. Torkildson, A. Cornelius, R. Rassekh, A. Bedros, M. Etzl, J. Garvin, K. Pradhan, R. Corbett, M. Sullivan, G. McGowage, D. Puccetti, D. Stein, R. Jasty, L. Ji, R. Sposto, J. L. Finlay, R. Antony, S. Gardner, M. Patel, K. E. Wong, B. Britt, G. Dhall, J. Grimm, M. Krieger, G. McComb, F. Gilles, R. Sposto, J. L. Finlay, T. B. Davidson, P. A. Sanchez-Lara, L. M. Randolph, M. D. Krieger, S. Wu, A. Panigrahy, H. Shimada, A. Erdreich-Epstein, D. M. Puccetti, N. Patel, T. Kennedy, S. Salamat, Y. Bradfield, H. J. Park, J. H. Yoon, H. S. Ahn, H. Y. Shin, S. K. Kim, H. J. Im, Y. S. Ra, S. C. Won, H. J. Baek, K. W. Sung, J. O. Hah, Y. T. Lim, G. S. Lee, Y. H. Lee, H. S. Kim, J. K. Park, M. K. Kim, J. E. Park, N. G. Chung, H. S. Choi, C. J. Campen, P. G. Fisher, M. I. Ruge, T. Simon, B. Suchorska, R. Lehrke, C. Hamisch, F. Koerber, H. Treuer, F. Berthold, V. Sturm, J. Voges, T. B. Davidson, J. L. Finlay, G. Dhall, M. Kirsch, C. Lindner, G. Schackert, R. J. Brown, M. Krieger, G. Dhall, J. L. Finlay
Journal: Neuro-Oncology (2011)

INTRODUCTION: Primitive neuroectodermal tumor (PNET) is the most common primary malignant childhood brain tumor and is usually seen as an intraparenchymal lesion, typically arising in the cerebellum (as a medulloblastoma).Peripheral PNET (PPNET), a member of the Ewing's sarcoma (ES) family of tumors, is a tumor of putative parasympathetic neural histogenesis, typically developing in the soft tissue and skeletal structures of the trunk and extremities.We report here four cases of atypical primary ES/PPNET in very young children involving pericranial structures, two with significant intracranial involvement.METHODS: Following IRB approval, we compiled the clinical, imaging, and pathology records from four children with aggressive pericranial ES/PPNET.RESULTS: Ages at diagnosis were 1 day (female), 5 days (male), 12 months (male), and 13 months (female).Two patients had orbitofacial tumors with significant bony erosion but no intracranial extension, one patient had a temporal bone lesion with significant middle cranial fossa extension, and one patient had an intracranial temporal lobe-based tumor without extracranial extension.Immunohistochemical evidence of the EWS/FLI-1 chimeric protooncogene, typical of PPNET, was identified in one patient; genetic analysis in two patients revealed EWSR1 gene rearrangement in one patient and a novel t(20;22)(q11.2;q12)translocation in the other.All tumors were relatively large (for body size), measuring 3-8 cm in maximal dimension.Treatment included tumor resection and sarcoma-style combination chemotherapy.Two patients are alive and continue therapy, whereas two have died of sepsis and progressive disease at 3 and 6 months after diagnosis, respectively.CONCLUSIONS: Pericranial primary ES/PPNET is a rare tumor, heretofore typically described only in pre-school and school-aged children.Our study suggests this entity can occur in very young children, in whom it has a poor prognosis.It appears to be more infiltrative and to violate the usual tissue compartments.Further awareness and reporting of this rare tumor will aid understanding of histogenesis, prognosis, and optimal therapy.