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Synovial sarcoma of nerve

dc.contributor.authorScheithauer, BW
dc.contributor.authorAmrami, KK
dc.contributor.authorFolpe, AL
dc.contributor.authorSilva, AI
dc.contributor.authorEdgar, MA
dc.contributor.authorWoodruff, JM
dc.contributor.authorLevi, AD
dc.contributor.authorSpinner, RJ
dc.date.accessioned2012-04-27T15:20:19Z
dc.date.available2012-04-27T15:20:19Z
dc.date.issued2011
dc.description.abstractTumors of peripheral nerve are largely neuroectodermal in nature and derived from 2 elements of nerve, Schwann or perineurial cells. In contrast, mesenchymal tumors affecting peripheral nerve are rare and are derived mainly from epineurial connective tissue. The spectrum of the latter is broad and includes lipoma, vascular neoplasms, hematopoietic tumors, and even meningioma. Of malignant peripheral nerve neoplasms, the vast majority are primary peripheral nerve sheath tumors. Malignancies of mesenchymal type are much less common. To date, only 12 cases of synovial sarcoma of nerve have been described. Whereas in the past, parallels were drawn between synovial sarcoma and malignant glandular schwannoma, an uncommon form of malignant peripheral nerve sheath tumor, molecular genetics have since clarified the distinction. Herein, we report 10 additional examples of molecularly confirmed synovial sarcoma, all arising within minor or major nerves. Affecting 7 female and 3 male patients, 4 tumors occurred in pediatric patients. Clinically and radiologically, most lesions were initially thought to be benign nerve sheath tumors. On reinterpretation of imaging, they were considered indeterminate in nature with some features suspicious for malignancy. Synovial sarcoma of nerve, albeit rare, seems to behave in a manner similar to its more common, soft tissue counterpart. Those affecting nerve have a variable prognosis. Definitive recommendations regarding surgery and adjuvant therapies await additional reports and long-term follow-up. The literature is reviewed and a meta-analysis is performed with respect to clinicopathologic features versus outcome.por
dc.identifier.citationHum Pathol. 2011;42(4):568-77.por
dc.identifier.urihttp://hdl.handle.net/10400.23/216
dc.language.isoengpor
dc.peerreviewedyespor
dc.publisherElsevierpor
dc.subjectNeoplasias do Sistema Nervoso Periféricopor
dc.subjectSarcoma Sinovialpor
dc.titleSynovial sarcoma of nervepor
dc.typejournal article
dspace.entity.typePublication
rcaap.rightsopenAccesspor
rcaap.typearticlepor

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