title: Primary cerebral myxofibrosarcoma: clinical, morphologic, immunohistochemical, molecular, and ultrastructural study of an infrequent tumor in an extraordinary localization.
abstract: Herein, we describe an intracerebral primary low-grade myxofibrosarcoma occurring in a 9-year-old boy. The lesion measured 7 cm and occupied the left parieto-occipital region. A gross-total removal of the tumor was performed. Nine months later, radiologic follow-up revealed a local recurrence which was again surgically removed. The patient then underwent radiotherapy and chemotherapy. He was well and disease-free at 6 months follow-up. The tumor was composed of spindle, stellated, and multinucleated cells embedded in a myxoid background. Foci of increased cellularity, pleomorphism, and high mitotic rate were present. The tumor borders were sharply demarcated from the non-neoplastic nervous parenchyma. Immunohistochemical staining showed that the neoplastic cells were vimentine and CD34 positive. Fluorescence in-situ hybridization analyses did not show FUS and EWSR1 gene rearrangements. Primary intracranial myxofibrosarcomas are very rare (to the best of our knowledge, less than 10 published cases in the international literature). We believe each new case should be recorded to produce a better clinical, pathologic, molecular, prognostic, and therapeutic characterization of this lesion.
authors.affiliation: Department of Biomedicine, Careggi Hospital, Florence, Italy. ambuccoliero@unifi.it
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Buccoliero
authors.forename: Francesca
authors.initials: F
authors.lastname: Castiglione
authors.forename: Francesca
authors.initials: F
authors.lastname: Garbini
authors.forename: Duccio
authors.initials: D
authors.lastname: Rossi Degl'Innocenti
authors.forename: Daniela
authors.initials: D
authors.lastname: Moncini
authors.forename: Alessandro
authors.initials: A
authors.lastname: Franchi
authors.forename: Milena
authors.initials: M
authors.lastname: Paglierani
authors.forename: Antonella
authors.initials: A
authors.lastname: Simoni
authors.forename: Gianna
authors.initials: G
authors.lastname: Baroni
authors.forename: Danielli
authors.initials: D
authors.lastname: Daniele
authors.forename: Iacopo
authors.initials: I
authors.lastname: Sardi
authors.forename: Flavio
authors.initials: F
authors.lastname: Giordano
authors.forename: Federico
authors.initials: F
authors.lastname: Mussa
authors.forename: Maurizio
authors.initials: M
authors.lastname: Aricò
authors.forename: Lorenzo
authors.initials: L
authors.lastname: Genitori
authors.forename: Gian Luigi
authors.initials: GL
authors.lastname: Taddei
mesh_terms: D001932:Brain Neoplasms / Q000175:diagnosis* / Q000601:surgery; D002648:Child; D005354:Fibrosarcoma / Q000175:diagnosis* / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D008279:Magnetic Resonance Imaging; D008297:Male; D014057:Tomography, X-Ray Computed