Peripheral primitive neuroectodermal tumors of bone. A review of three cases.

Abstract

The recently individualized and still incompletely understood family of peripheral neuroectodermal tumors encompasses several tumor types, of which some have a predilection for bone. Immunocytochemical studies are essential and usually provide the diagnosis. A t(11;22)(q24;q12) translocation is present in over 80% of cases. Ewing's sarcoma is now viewed as an undifferentiated form of peripheral neuroectodermal tumor, and both tumors require management with combination chemotherapy plus radiation therapy and/or surgery. Contradictory data have been reported regarding the comparative prognosis of peripheral neuroectodermal tumor and Ewing's sarcoma, indicating a need for further studies in large numbers of patients. We illustrate these points by three case-reports, two in girls diagnosed with a vertebral primary at five and nine years of age, respectively, and one in a man diagnosed with a pelvic primary at 29 years of age.

• Authors: B Augé, B Kantelip, H Chataigner, E Toussirot, F Michel, M Onimus, D Wendling
• Journal: Revue du rhumatisme (English ed.) (Rev Rhum Engl Ed) Vol. 66 Issue 2 Pg. 92-6 (Feb 1999) ISSN: 1169-8446 [Print] France
• PMID: 10084168 (Publication Type: Case Reports, Journal Article, Review)

Topics

• Adult
• Biopsy
• Bone Neoplasms (diagnosis, therapy)
• Cervical Vertebrae (diagnostic imaging, pathology)
• Child
• Child, Preschool
• Combined Modality Therapy
• Diagnosis, Differential
• Fatal Outcome
• Female
• Follow-Up Studies
• Humans
• Ilium (diagnostic imaging, pathology)
• Magnetic Resonance Imaging
• Male
• Neoplasm Recurrence, Local
• Neuroectodermal Tumors, Primitive (diagnosis, therapy)
• Radiography
• Thoracic Vertebrae (diagnostic imaging, pathology)