Long-term Survival and Functional Improvement in Pediatric Intramedullary Spinal Cord Tumors

Keywords: spinal cord, outcome, children, spinal cord neoplasm, ependymoma

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     Intramedullary spinal cord tumors (IMSCT) comprise a rare subgroup of intraspinal tumors in the pediatric age group. Radical resection is recommended as first-line treatment but is associated with morbidity including risk of neurological decline and development of postoperative spinal deformity.
     We present our institutional experience in the management of pediatric IMSCTs, analyzing the roles of surgery, chemotherapy and radiation therapy.
     Thirty patients (male=18, female=12) were identified from 1975 to 2010. Mean age at presentation was 9.5yr (Range 0.5mo–20yr).
     Case records for pediatric patients (<21yr), treated at our institution for IMSCTs were analyzed.
      Histopathological diagnosis indicated predominance of low-grade astrocytomas (n=16; 53%), anaplastic astrocytomas (n=6, 20%) and ependymomas (n=5, 17%). An attempt at radical resection was made with intraoperative neurophysiological monitoring. Significant functional grade (McCormick scale) improvement was seen in 45% of patients. Overall improvement in functional grade significantly correlated with better functional grade at presentation. Incidence of secondary spinal deformity necessitating surgical fusion was 26% (n=8) and correlated significantly with younger age at presentation. Repeat surgical resection was required in 40% of patients in <2yr age group. Five yr, 10-yr and 20-yr survival rates were 83%, 65% and 41% respectively. Adjuvant chemotherapy and/or radiation therapy did not improve progression free survival and overall disease survival.
     This is a retrospective study.
     Overall disease prognosis for pediatric IMSCTs is favorable with functional improvement expected in a significant proportion of patients.
     Long term survival is associated with early diagnosis and aggressive surgical resection. Adjuvant therapies do not offer any significant therapeutic benefit.


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