A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University Health Sciences Center.
OBJECTIVE
This is a retrospective review of 397 benign and malignant peripheral neural sheath tumors (PNSTs) that were surgically treated between 1969 and 1999 at the Louisiana State University Health Sciences Center (LSUHSC). The surgical techniques and adjunctive treatments are presented, the tumors are classified with respect to type and prevalence at each neuroanatomical location, and the management of malignant PNSTs is reviewed.


METHODS
There were 361 benign PNSTs (91%). One hundred forty-one benign lesions were brachial plexus tumors: 54 schwannomas (38%) and 87 neurofibromas (62%), of which 55 (63%) were solitary neurofibromas and 32 (37%) were neurofibromatosis Type 1 (NF1)-associated neurofibromas. Among the brachial plexus lesions supraclavicular tumors predominated with 37 (69%) of 54 schwannomas; 34 (62%) of 55 solitary neurofibromas; and 19 (59%) of 32 NF1-associated neurofibromas. One hundred ten upper-extremity benign PNSTs consisted of 32 schwannomas (29%) and 78 neurofibromas (71%), of which 45 (58%) were sporadic neurofibromas and 33 (42%) were NF1-associated neurofibromas. Twenty-five benign PNSTs were removed from the pelvic plexus. Lower-extremity PNSTs included 32 schwannomas (38%) and 53 neurofibromas (62%), of which 31 were solitary neurofibromas and 22 were NF1-associated neurofibromas. There were 36 malignant PNSTs: 28 neurogenic sarcomas and eight other sarcomas (fibro-, spindle cell, synovial, and perineurial sarcomas).


CONCLUSIONS
The majority of tumors were benign PNSTs from the brachial plexus region. Most of the benign PNSTs in all locations were neurofibromas, with sporadic neurofibromas predominating. Similar numbers of schwannomas were found in the upper and lower extremities, whereas neurofibromas were more prevalent in the upper extremities. Despite aggressive limb-ablation or limb-sparing surgery plus adjunctive therapy, malignant PNSTs continue to be associated with high morbidity and mortality rates.
Kim
Daniel H
DH
Department of Neurosurgery, Stanford University Medical Center, Stanford, California 94305-5327, USA. neurokim@stanford.edu
Murovic
Judith A
JA
Tiel
Robert L
RL
Moes
Gregory
G
Kline
David G
DG
D000046:Academic Medical Centers; D001132:Arm / Q000294:innervation / Q000601:surgery; D014408:Biomarkers, Tumor / Q000032:analysis; D001917:Brachial Plexus / Q000473:pathology / Q000601:surgery; D020516:Brachial Plexus Neuropathies / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007001:Hypogastric Plexus / Q000473:pathology / Q000601:surgery*; D007866:Leg / Q000294:innervation / Q000601:surgery; D008145:Louisiana; D008279:Magnetic Resonance Imaging; D018317:Nerve Sheath Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D009442:Neurilemmoma / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D009455:Neurofibroma / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D009456:Neurofibromatosis 1 / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D018319:Neurofibrosarcoma / Q000175:diagnosis / Q000473:pathology / Q000601:surgery; D009460:Neurologic Examination; D010525:Peripheral Nerves / Q000473:pathology / Q000601:surgery; D010524:Peripheral Nervous System Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D014408:Biomarkers, Tumor