| Literature DB >> 26743922 |
Mariangela Novello1, Concezio Di Rocco2,3, Gianpiero Tamburrini2, Paolo Frassanito2, Daniel T Aguirre3, Andrew E Rosenberg4, Antonella Coli5.
Abstract
INTRODUCTION: Primary, adult-type bone fibrosarcoma is an uncommon, malignant spindle-cell tumor of fibroblastic origin, rarely affecting children. Most frequently diagnosed among bone malignancies in the past, improved diagnostic techniques and further restrictive classification criteria have currently made the diagnosis of fibrosarcoma very unusual. CASE REPORT: We hereby report the case of a 7-year-old child with a right frontal swelling mass. A computed tomography scan showed an osteolytic lesion of the right frontal bone, involving the diploe and the outer table of the skull. An en bloc surgical excision, followed by a thorough immunohistological evaluation, led to the diagnosis of fibroblastic proliferation, with low cellularity and minimal atypias. The patient had four recurrences during the 4-year follow-up. With an increasing histological grade at recurrences, a diagnosis of adult-type fibrosarcoma was made.Entities:
Keywords: Fibrosarcoma; Frontal bone; Immunohistochemistry; Molecular biology; Pediatric; Tumor progression
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Year: 2016 PMID: 26743922 DOI: 10.1007/s00381-015-2998-6
Source DB: PubMed Journal: Childs Nerv Syst ISSN: 0256-7040 Impact factor: 1.475