TY - JOUR
T1 - Neurocytoma-like neoplasm of the thoracic spine in a 15-month-old child presenting with diffuse leptomeningeal dissemination and communicating hydrocephalus
T2 - Case report
AU - Psarros, Thomas G.
AU - Swift, Dale
AU - Mulne, Arlynn F.
AU - Burns, Dennis K.
PY - 2005/8
Y1 - 2005/8
N2 - This unusual mixed glioneuronal neoplasm of the spine resembling central neurocytoma is only the second reported example of a neoplasm of this type involving the spinal cord and is, seemingly, the first to present with diffuse leptomeningeal dissemination and communicating hydrocephalus. This 15-month-old boy presented with somnolence, bilateral sixth nerve palsy, and lower-extremity weakness and was found to harbor a primary neoplasm within the thoracic spine and associated syrinx, widespread leptomeningeal dissemination, and communicating hydrocephalus. The patient underwent cerebrospinal fluid shunt placement, thoracic laminectomy for tumor debulking and biopsy, chemotherapy, and radiation therapy to the neuraxis. Immunohistochemically, the tumor was marked by evidence of mixed glial (glial fibrillary acidic protein-positive) and neuronal (synaptophysin-positive) differentiation. Mitotic activity was inconspicuous. No areas of vascular proliferation, necrosis, or marked nuclear pleomorphism were noted. Mixed glioneuronal neoplasms are a heterogeneous group of tumors whose biological potential remains incompletely defined. The present case illustrates the reality that some of these neoplasms can be clinically aggressive, despite comparatively bland histological features. The authors' goal is to acquaint neurosurgeons with the expanding spectrum of mixed glioneuronal neoplasms and with the potential of some of these lesions to pursue an aggressive clinical course.
AB - This unusual mixed glioneuronal neoplasm of the spine resembling central neurocytoma is only the second reported example of a neoplasm of this type involving the spinal cord and is, seemingly, the first to present with diffuse leptomeningeal dissemination and communicating hydrocephalus. This 15-month-old boy presented with somnolence, bilateral sixth nerve palsy, and lower-extremity weakness and was found to harbor a primary neoplasm within the thoracic spine and associated syrinx, widespread leptomeningeal dissemination, and communicating hydrocephalus. The patient underwent cerebrospinal fluid shunt placement, thoracic laminectomy for tumor debulking and biopsy, chemotherapy, and radiation therapy to the neuraxis. Immunohistochemically, the tumor was marked by evidence of mixed glial (glial fibrillary acidic protein-positive) and neuronal (synaptophysin-positive) differentiation. Mitotic activity was inconspicuous. No areas of vascular proliferation, necrosis, or marked nuclear pleomorphism were noted. Mixed glioneuronal neoplasms are a heterogeneous group of tumors whose biological potential remains incompletely defined. The present case illustrates the reality that some of these neoplasms can be clinically aggressive, despite comparatively bland histological features. The authors' goal is to acquaint neurosurgeons with the expanding spectrum of mixed glioneuronal neoplasms and with the potential of some of these lesions to pursue an aggressive clinical course.
KW - Communicating hydrocephalus
KW - Leptomeningeal dissemination
KW - Mixed glioneuronal neoplasm
KW - Neurocytoma
KW - Pediatric neurosurgery
UR - http://www.scopus.com/inward/record.url?scp=33644685636&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=33644685636&partnerID=8YFLogxK
M3 - Article
C2 - 16370289
AN - SCOPUS:33644685636
SN - 0022-3085
VL - 103 PEDIATRICS
SP - 184
EP - 190
JO - Journal of neurosurgery
JF - Journal of neurosurgery
IS - SUPPL. 2
ER -