Skull base surgery for tumors in children: long-term clinical and functional outcome

Abstract

<jats:sec> <jats:title>Object</jats:title> <jats:p>Skull base tumors in children are rare but require complex approaches with potential morbidity to the developing craniofacial skeleton, in addition to tumor-related morbidity. Reports of long-term clinical and functional outcome following skull base approaches in children are scarce. The authors report long-term outcome in children with tumors undergoing multidisciplinary skull base surgery.</jats:p></jats:sec> <jats:sec> <jats:title>Methods</jats:title> <jats:p>A retrospective analysis was undertaken of children undergoing surgery at a single institution between 1998 and 2008 for benign and malignant lesions of the anterior, middle, or posterior cranial base. Patients with craniopharyngioma, pituitary tumors, and optic glioma were excluded. Histology, surgical morbidity, length of hospital stay, progression-free survival, and adjuvant therapy were recorded. Functional and cognitive outcome was assessed prospectively using the Late Effects Severity Score (LESS).</jats:p></jats:sec> <jats:sec> <jats:title>Results</jats:title> <jats:p>Twenty-three children ranging in age from 13 months to 15 years underwent skull base approaches for resection of tumors during the study period. The median follow-up duration was 60 months. Tumor types included meningioma, schwannoma, rhabdomyosarcoma, neuroblastoma, angiofibroma, and chordoma. Complete resection was achieved in 12 patients (52%). Thirteen patients (57%) had benign histology. The median hospital stay was 7 days. There were 3 deaths, 1 perioperative and 2 from tumor progression. Two patients had CSF leakage (9%) and 2 developed meningitis. Two children (9%) had residual neurological deficit at last follow-up evaluation. Thirteen (59%) of 22 surviving patients received adjuvant therapy. The majority of the patients remain in mainstream education and 19 of the 20 surviving children have an LESS of 3 or lower.</jats:p></jats:sec> <jats:sec> <jats:title>Conclusions</jats:title> <jats:p>Children tolerate complex skull base procedures well, with minimal surgical-related morbidity as well as good long-term tumor control rates and functional outcomes from maximal safe resection combined with adjuvant treatment when required.</jats:p></jats:sec>

125I interstitial brachytherapy in management of pediatric skull base tumors

“…Pediatric skull base tumors are rare and consist of a variety of histology types . Malignant tumors account for 27% to 54% of all pediatric skull base tumors, of which sarcoma is the most common type .…”

“…There is no consensus in treatment for pediatric skull base tumors; thus, they are a challenge for oncologists, pediatric surgeons, and radiation practitioners. Multidisciplinary therapy, including surgery, radiotherapy, and chemotherapy, has become a treatment modality for pediatric skull base tumors . However, children are known to be more sensitive to radiation‐induced malignancies than adults by a factor of more than 10 times, and long‐term radiotherapy‐related complications, such as facial growth disorders and dental abnormalities in pediatric patients, have been frequently reported .…”

Endoscopic endonasal surgery for benign fibro‐osseous lesions of the pediatric skull base

“…This has the potential for brain injury with resultant encephalomalacia and long‐term cognitive changes. A 2013 study by Hayhurst et al evaluated the long‐term functional and cognitive outcomes using the Late Effects Severity Score (LESS) of pediatric patients who had undergone open skull base tumor resection . Eight of 23 patients had elevated LESS scores, indicating late neurologic, endocrine, visual/auditory, or other effects with long‐term follow‐up (average 60 months).…”

36 Pediatric Skull Base

“…The survival rate was 87% and the rate of permanent neurologic morbidity was 9%. 84 In another series of 26 cases of skull base tumors, a 30.7% complication rate of permanent deficits was reported, with a 92% complete resection rate and 81% tumor-free survival rate at 2 years postoperatively. 2 A retrospective series of 67 children operated for skull base pathologies during a 6-year period 85 reported that 80% of children were alive and well after 2½ years, with no severe postoperative complications.…”

“…3 • Even if skull base tumors in children are more aggressive, minimal surgical-related morbidities and good long-term tumor control have been reported. 2,70,83,84 Outcomes • In a series of 55 children who underwent skull base approaches, a 11% rate of permanent neurologic morbidity was reported. 70 • In a series of 23 pediatric patients who underwent skull base approaches for resection of tumors with a median follow-up of 60 months, gross total resection was achieved in 52%.…”