Tumors of the skull base in children and adolescents

Restricted access

Object. Published data obtained in children with tumors of the skull base are sparse. In the majority of the available reports, the authors focus on the technical application of skull base approaches, but they contribute a paucity of information on the management of specific tumors, especially malignant skull base lesions. The purposes of this report are to increase the collective experience with the treatment of these tumors and to identify successful management paradigms.

Methods. The authors retrospectively reviewed the clinical records, pathological reports, and diagnostic images obtained in 24 children (≤ 19 years of age) with tumors arising from the cranial base in whom resection was part of their management between 1992 and 2002. Surgery-related complications and outcomes were analyzed with regard to tumor type and surgical approach.

The median age of the group was 14 years. Tumors involved the anterior skull base in eight (33%), the middle skull base in 10 (42%), both the anterior and middle skull base in four (17%), and the posterior skull base in two patients (8%). Benign lesions were discovered in 11 patients (46%) and malignant neoplasms in 13 (54%). The tumors were most commonly of mesenchymal origin (21 [87.5%] of 24 tumors). Thirty surgical procedures were performed using a number of skull base approaches. A gross-total resection was achieved in 23 procedures (77%) and a subtotal resection in five (17%); a biopsy procedure was performed in one case; and the disease process could not be accessed in one case. One patient died in the perioperative period. Minor complications (Karnofsky Performance Scale score ≥ 90, no prolongation of hospital stay, and no further surgery needed) occurred following 10 (33%) of the 30 surgical procedures. These affected 10 (42%) of the 24 patients and resulted in persistent or prolonged deficits in only five patients (21%). In nine patients (38%) the tumor recurred after a mean duration of 23 months.

Conclusions. Skull base tumors in children affect mainly the anterior and middle cranial fossa. Sarcomas account for the majority of malignant tumors. Treatment of skull base tumors in children and adolescents needs to be tailored to patient age, tumor location, and tumor type.

Article Information

Contributor Notes

Address reprint requests to: Franco DeMonte, M.D., F.R.C.S.(C)., Department of Neurosurgery, Box 442, M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030. email: fdemonte@mdanderson.org.

© AANS, except where prohibited by US copyright law.

Headings
References
  • 1.

    Al-Mefty OBorba LA: Skull base chordomas: a management challenge. J Neurosurg 86:1821891997Al-Mefty O Borba LA: Skull base chordomas: a management challenge. J Neurosurg 86:182–189 1997

    • Search Google Scholar
    • Export Citation
  • 2.

    Alshail ERutka JTDrake JMet al: Utility of frameless stereotaxy in the resection of skull base and basal cerebral lesions in children. Skull Base Surg 8:29381998Alshail E Rutka JT Drake JM et al: Utility of frameless stereotaxy in the resection of skull base and basal cerebral lesions in children. Skull Base Surg 8:29–38 1998

    • Search Google Scholar
    • Export Citation
  • 3.

    Bales CKotapka MLoevner LAet al: Craniofacial resection of advanced juvenile nasopharyngeal angiofibroma. Arch Otolaryngol Head Neck Surg 128:107110782002Bales C Kotapka M Loevner LA et al: Craniofacial resection of advanced juvenile nasopharyngeal angiofibroma. Arch Otolaryngol Head Neck Surg 128:1071–1078 2002

    • Search Google Scholar
    • Export Citation
  • 4.

    Benk VLiebsch NJMunzenrider JEet al: Base of skull and cervical chordomas in children treated by high-dose irradiation. Int J Radiat Oncol Biol Phys 31:5775811995Benk V Liebsch NJ Munzenrider JE et al: Base of skull and cervical chordomas in children treated by high-dose irradiation. Int J Radiat Oncol Biol Phys 31:577–581 1995

    • Search Google Scholar
    • Export Citation
  • 5.

    Blatt JSnyderman CWollman MRet al: Delayed resection in the management of non-orbital rhabdomyosarcoma of the head and neck in childhood. Med Pediatr Oncol 28:2942981997Blatt J Snyderman C Wollman MR et al: Delayed resection in the management of non-orbital rhabdomyosarcoma of the head and neck in childhood. Med Pediatr Oncol 28:294–298 1997

    • Search Google Scholar
    • Export Citation
  • 6.

    Borba LABAl-Mefty OMrak REet al: Cranial chordomas in children and adolescents. J Neurosurg 84:5845911996Borba LAB Al-Mefty O Mrak RE et al: Cranial chordomas in children and adolescents. J Neurosurg 84:584–591 1996

    • Search Google Scholar
    • Export Citation
  • 7.

    Brockmeyer DLArthur ASGruber DPet al: Anterior skull base tumors in Batjer HHLoftus CM (eds): Textbook of Neurological Surgery. Philadelphia: Lippincott Willams & Wilkins2003 Vol 1 pp 10411045Brockmeyer DL Arthur AS Gruber DP et al: Anterior skull base tumors in Batjer HH Loftus CM (eds): Textbook of Neurological Surgery. Philadelphia: Lippincott Willams & Wilkins 2003 Vol 1 pp 1041–1045

    • Search Google Scholar
    • Export Citation
  • 8.

    Bruce DA: Skull base tumors in children in Albright LPollack IAdelson D (eds): Principles and Practice of Pediatric Neurosurgery. New York: Thieme Medical Publishers1999 pp 663684Bruce DA: Skull base tumors in children in Albright L Pollack I Adelson D (eds): Principles and Practice of Pediatric Neurosurgery. New York: Thieme Medical Publishers 1999 pp 663–684

    • Search Google Scholar
    • Export Citation
  • 9.

    Cohen ZRMarmor EFuller GNet al: Misdiagnosis of olfactory neuroblastoma. Neurosurg Focus 12(5):Article 32002Cohen ZR Marmor E Fuller GN et al: Misdiagnosis of olfactory neuroblastoma. Neurosurg Focus 12(5):Article 3 2002

    • Search Google Scholar
    • Export Citation
  • 10.

    DeMonte FDiaz E JrCallender Det al: Transmandibular, circumglossal, retropharyngeal approach for chordomas of the clivus and upper cervical spine. Technical note. Neurosurg Focus 10(3):Article 102001DeMonte F Diaz E Jr Callender D et al: Transmandibular circumglossal retropharyngeal approach for chordomas of the clivus and upper cervical spine. Technical note. Neurosurg Focus 10(3):Article 10 2001

    • Search Google Scholar
    • Export Citation
  • 11.

    DeMonte FTabrizi PCulpepper SAet al: Ophthalmological outcome after orbital entry during anterior and anterolateral skull base surgery. J Neurosurg 97:8518562002DeMonte F Tabrizi P Culpepper SA et al: Ophthalmological outcome after orbital entry during anterior and anterolateral skull base surgery. J Neurosurg 97:851–856 2002

    • Search Google Scholar
    • Export Citation
  • 12.

    Eder HGLeber KAEustacchio Set al: The role of gamma knife radiosurgery in children. Childs Nerv Syst 17:3413472001Eder HG Leber KA Eustacchio S et al: The role of gamma knife radiosurgery in children. Childs Nerv Syst 17:341–347 2001

    • Search Google Scholar
    • Export Citation
  • 13.

    Eppley BLSadove AMHavlik RJ: Resorbable plate fixation in pediatric craniofacial surgery. Plast Reconstr Surg 100:1131997Eppley BL Sadove AM Havlik RJ: Resorbable plate fixation in pediatric craniofacial surgery. Plast Reconstr Surg 100:1–13 1997

    • Search Google Scholar
    • Export Citation
  • 14.

    Farkas LGPosnick JCHreczko TM: Anthropometric growth study of the head. Cleft Palate Craniofac J 29:3033081992Farkas LG Posnick JC Hreczko TM: Anthropometric growth study of the head. Cleft Palate Craniofac J 29:303–308 1992

    • Search Google Scholar
    • Export Citation
  • 15.

    Fearon JAMunro IRBruce DA: Observations on the use of rigid fixation for craniofacial deformities in infants and young children. Plast Reconstr Surg 95:6346381995Fearon JA Munro IR Bruce DA: Observations on the use of rigid fixation for craniofacial deformities in infants and young children. Plast Reconstr Surg 95:634–638 1995

    • Search Google Scholar
    • Export Citation
  • 16.

    Fearon JAMunro IRBruce DAet al: Massive teratomas involving the cranial base: treatment and outcome—a two-center report. Plast Reconstr Surg 91:2232281993Fearon JA Munro IR Bruce DA et al: Massive teratomas involving the cranial base: treatment and outcome—a two-center report. Plast Reconstr Surg 91:223–228 1993

    • Search Google Scholar
    • Export Citation
  • 17.

    Gadwal SRFanburg-Smith JCGannon FHet al: Primary chondrosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 14 cases with a review of the literature. Cancer 88:218121882000Gadwal SR Fanburg-Smith JC Gannon FH et al: Primary chondrosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 14 cases with a review of the literature. Cancer 88:2181–2188 2000

    • Search Google Scholar
    • Export Citation
  • 18.

    Gay ESekhar LNRubinstein Eet al: Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients. Neurosurgery 36:8878971995Gay E Sekhar LN Rubinstein E et al: Chordomas and chondrosarcomas of the cranial base: results and follow-up of 60 patients. Neurosurgery 36:887–897 1995

    • Search Google Scholar
    • Export Citation
  • 19.

    Goldberg DSBartlett SPYu JCet al: Critical review of microfixation in pediatric craniofacial surgery. J Craniofac Surg 6:3013081995Goldberg DS Bartlett SP Yu JC et al: Critical review of microfixation in pediatric craniofacial surgery. J Craniofac Surg 6:301–308 1995

    • Search Google Scholar
    • Export Citation
  • 20.

    Guyuron BDagys APMunro IRet al: Effect of irradiation on facial growth: a 7- to 25-year follow-up. Ann Plast Surg 11:4234271983Guyuron B Dagys AP Munro IR et al: Effect of irradiation on facial growth: a 7- to 25-year follow-up. Ann Plast Surg 11:423–427 1983

    • Search Google Scholar
    • Export Citation
  • 21.

    Hayasaka SSekimoto MShibasaki Het al: Ophthalmic complications in patients with malignant tumors of the nose and paranasal sinuses. Ann Ophthalmol 24:4294331992Hayasaka S Sekimoto M Shibasaki H et al: Ophthalmic complications in patients with malignant tumors of the nose and paranasal sinuses. Ann Ophthalmol 24:429–433 1992

    • Search Google Scholar
    • Export Citation
  • 22.

    Healy GBUpton JBlack PMet al: The role of surgery in rhabdomyosarcoma of the head and neck in children. Arch Otolaryngol Head Neck Surg 117:118511881991Healy GB Upton J Black PM et al: The role of surgery in rhabdomyosarcoma of the head and neck in children. Arch Otolaryngol Head Neck Surg 117:1185–1188 1991

    • Search Google Scholar
    • Export Citation
  • 23.

    Holmes ADKlug GLBreidahl AF: The surgical management of osseous cranial base tumours in children. Aust N Z J Surg 67:7227301997Holmes AD Klug GL Breidahl AF: The surgical management of osseous cranial base tumours in children. Aust N Z J Surg 67:722–730 1997

    • Search Google Scholar
    • Export Citation
  • 24.

    Hug EBSweeney RANurre PMet al: Proton radiotherapy in management of pediatric base of skull tumors. Int J Radiat Oncol Biol Phys 52:101710242002Hug EB Sweeney RA Nurre PM et al: Proton radiotherapy in management of pediatric base of skull tumors. Int J Radiat Oncol Biol Phys 52:1017–1024 2002

    • Search Google Scholar
    • Export Citation
  • 25.

    Imola MJHamlar DDShao Wet al: Resorbable plate fixation in pediatric craniofacial surgery: long-term outcome. Arch Facial Plast Surg 3:79902001Imola MJ Hamlar DD Shao W et al: Resorbable plate fixation in pediatric craniofacial surgery: long-term outcome. Arch Facial Plast Surg 3:79–90 2001

    • Search Google Scholar
    • Export Citation
  • 26.

    Jackson CGPappas DG JrManolidis Set al: Pediatric neurotologic skull base surgery. Laryngoscope 106:120512091996Jackson CG Pappas DG Jr Manolidis S et al: Pediatric neurotologic skull base surgery. Laryngoscope 106:1205–1209 1996

    • Search Google Scholar
    • Export Citation
  • 27.

    Kennedy JDHaines SJ: Review of skull base surgery approaches: with special reference to pediatric patients. J Neurooncol 20:2913121994Kennedy JD Haines SJ: Review of skull base surgery approaches: with special reference to pediatric patients. J Neurooncol 20:291–312 1994

    • Search Google Scholar
    • Export Citation
  • 28.

    Kumar MFallon RJHill JSet al: Esthesioneuroblastoma in children. J Pediatr Hematol Oncol 24:4824872002Kumar M Fallon RJ Hill JS et al: Esthesioneuroblastoma in children. J Pediatr Hematol Oncol 24:482–487 2002

    • Search Google Scholar
    • Export Citation
  • 29.

    Lang DANeil-Dwyer GEvans BTet al: Craniofacial access in children. Acta Neurochir 140:33401998Lang DA Neil-Dwyer G Evans BT et al: Craniofacial access in children. Acta Neurochir 140:33–40 1998

    • Search Google Scholar
    • Export Citation
  • 30.

    Lewark TMAllen GCChowdhury Ket al: Le Fort I osteotomy and skull base tumors: a pediatric experience. Arch Otolaryngol Head Neck Surg 126:100410082000Lewark TM Allen GC Chowdhury K et al: Le Fort I osteotomy and skull base tumors: a pediatric experience. Arch Otolaryngol Head Neck Surg 126:1004–1008 2000

    • Search Google Scholar
    • Export Citation
  • 31.

    Menezes AHGantz BJTraynelis VCet al: Cranial base chordomas. Clin Neurosurg 44:4915091997Menezes AH Gantz BJ Traynelis VC et al: Cranial base chordomas. Clin Neurosurg 44:491–509 1997

    • Search Google Scholar
    • Export Citation
  • 32.

    Perez-Cruet MJBurke JMWeber Ret al: Aggressive fibromatosis involving the cranial base in children. Neurosurgery 43:109611021998Perez-Cruet MJ Burke JM Weber R et al: Aggressive fibromatosis involving the cranial base in children. Neurosurgery 43:1096–1102 1998

    • Search Google Scholar
    • Export Citation
  • 33.

    Raney RBAnderson JRBarr FGet al: Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol 23:2152202001Raney RB Anderson JR Barr FG et al: Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life: a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol 23:215–220 2001

    • Search Google Scholar
    • Export Citation
  • 34.

    Raney RBAnderson JRKollath Jet al: Late effects of therapy in 94 patients with localized rhabdomyosarcoma of the orbit: Report from the Intergroup Rhabdomyosarcoma Study (IRS)-III, 1984–1991. Med Pediatr Oncol 34:4134202000Raney RB Anderson JR Kollath J et al: Late effects of therapy in 94 patients with localized rhabdomyosarcoma of the orbit: Report from the Intergroup Rhabdomyosarcoma Study (IRS)-III 1984–1991. Med Pediatr Oncol 34:413–420 2000

    • Search Google Scholar
    • Export Citation
  • 35.

    Raney RBAsmar LVassilopoulou-Sellin Ret al: Late complications of therapy in 213 children with localized, nonorbital soft-tissue sarcoma of the head and neck: A descriptive report from the Intergroup Rhabdomyosarcoma Studies (IRS)-II and -III. IRS Group of the Children's Cancer Group and the Pediatric Oncology Group. Med Pediatr Oncol 33:3623711999Raney RB Asmar L Vassilopoulou-Sellin R et al: Late complications of therapy in 213 children with localized nonorbital soft-tissue sarcoma of the head and neck: A descriptive report from the Intergroup Rhabdomyosarcoma Studies (IRS)-II and -III. IRS Group of the Children's Cancer Group and the Pediatric Oncology Group. Med Pediatr Oncol 33:362–371 1999

    • Search Google Scholar
    • Export Citation
  • 36.

    Sgouros SNatarajan KHockley ADet al: Skull base growth in childhood. Pediatr Neurosurg 31:2592681999Sgouros S Natarajan K Hockley AD et al: Skull base growth in childhood. Pediatr Neurosurg 31:259–268 1999

    • Search Google Scholar
    • Export Citation
  • 37.

    Teo CDornhoffer JHanna Eet al: Application of skull base techniques to pediatric neurosurgery. Childs Nerv Syst 15:1031091999Teo C Dornhoffer J Hanna E et al: Application of skull base techniques to pediatric neurosurgery. Childs Nerv Syst 15:103–109 1999

    • Search Google Scholar
    • Export Citation
  • 38.

    Tsai ECSantoreneos SRutka JT: Tumors of the skull base in children: review of tumor types and management strategies. Neurosurg Focus 12(5):Article 12002Tsai EC Santoreneos S Rutka JT: Tumors of the skull base in children: review of tumor types and management strategies. Neurosurg Focus 12(5):Article 1 2002

    • Search Google Scholar
    • Export Citation
  • 39.

    Volpe NJLiebsch NJMunzenrider JEet al: Neuro-ophthalmologic findings in chordoma and chondrosarcoma of the skull base. Am J Ophthalmol 115:971041993Volpe NJ Liebsch NJ Munzenrider JE et al: Neuro-ophthalmologic findings in chordoma and chondrosarcoma of the skull base. Am J Ophthalmol 115:97–104 1993

    • Search Google Scholar
    • Export Citation
  • 40.

    Wold LELaws ER Jr: Cranial chordomas in children and young adults. J Neurosurg 59:104310471983Wold LE Laws ER Jr: Cranial chordomas in children and young adults. J Neurosurg 59:1043–1047 1983

    • Search Google Scholar
    • Export Citation
  • 41.

    Wolden SLLa THLaQuaglia MPet al: Long-term results of three-dimensional conformal radiation therapy for patients with rhabdomyosarcoma. Cancer 97:1791852003Wolden SL La TH LaQuaglia MP et al: Long-term results of three-dimensional conformal radiation therapy for patients with rhabdomyosarcoma. Cancer 97:179–185 2003

    • Search Google Scholar
    • Export Citation
TrendMD
Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 165 163 8
Full Text Views 90 63 1
PDF Downloads 138 83 1
EPUB Downloads 0 0 0
PubMed
Google Scholar