Pediatric Astrocytoma Clinical Presentation

  • Author: Tobey MacDonald, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Feb 29, 2012
 

History

Patients often report a history of illness for more than 3 months prior to diagnosis.

  • Increased intracranial pressure
    • Initial symptoms are usually nonspecific, nonlocalizing, and related to increased intracranial pressure (ICP). These signs occur in as many as 75% of patients regardless of tumor location.
    • The classic triad of a raised ICP consists of morning headaches, vomiting, and lethargy. The headache is characterized by pain upon arising that is relieved by vomiting and lessens during the day.
    • School-aged children more commonly report vague intermittent headaches and fatigue. They may have a declining academic performance and may exhibit personality changes.
    • Infants may present with irritability, anorexia, developmental delay, or regression.
  • Seizures: Seizures are present at diagnosis in at least 25% of patients with supratentorial astrocytomas. They may precede diagnosis by several months to 1-2 years.
  • Signs related to tumor location
    • Focal motor deficits occur in as many as 60% of patients with hemispheric and central diencephalic tumors. They are more common in individuals with high-grade gliomas.
    • Seizures occur in 30-50% of children, may be focal, and are a more common presenting finding in low-grade gliomas.
    • Hypothalamic tumors may be associated with neuroendocrine abnormalities, growth hormone deficiency, diabetes insipidus, and precocious pubertal development. These tumors may also impinge on the optic chiasm, resulting in optic atrophy and visual deficits.
    • Patients with diencephalic tumors may present with the classic diencephalic syndrome (ie, emesis, emaciation, unusual euphoria), but the syndrome is rare in children older than 3 years.
    • Patients with astrocytomas of the cerebellum may present with weakness, dysmetria, tremor, and ataxia.
    • Astrocytomas of the brain stem are characterized by the presence of isolated cranial nerve deficits and contralateral hemiparesis.
    • Astrocytomas of the visual pathways may be brought to medical attention because of strabismus, proptosis, nystagmus, or developmental delay. Young children rarely report the slow and progressive visual loss characteristic of these tumors. Infants frequently display head tilt, head bobbing, and nystagmus. Astrocytomas in children with neurofibromatosis type 1 (NF1) may be asymptomatic at the time of diagnosis and may be detected on screening studies.
    • Patients with astrocytomas of the spinal cord most frequently present with pain (70% of patients have pain localized to the vertebral segments adjacent to the tumor), weakness, gait disturbance, and sphincter dysfunction. Paresthesias and loss of sensation occur later in the disease course.
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Physical

  • Increased intracranial pressure
    • A funduscopic examination reveals papilledema. Infants may have only optic pallor.
    • Palsy of cranial nerve VI is common and results in the inability to abduct one or both eyes.
    • Infants may demonstrate the setting sun sign, observed as an impaired upgaze and a forced downward deviation of both eyes. Measurement of head circumference in infants with open sutures may reveal macrocephaly.
  • Other signs
    • Strength and motor testing may reveal weakness and monoplegia or hemiplegia.
    • Localized deficits in truncal steadiness, upper extremity coordination, and gait may be observed with tumors of the posterior fossa and basal ganglia.
    • Multiple and bilateral cranial nerve deficits, especially VI and VII; long tract signs; and ataxia are associated with brainstem tumors.
    • Visual acuity is frequently reduced to less than 20/200 with optic gliomas. The pattern of visual loss in those patients with intraorbital tumors is most commonly a decrease in central vision, whereas bitemporal hemianopsia is most often noted in those patients with chiasmatic tumors. The involved eye generally shows optic pallor and nystagmus. Mild proptosis is usually present with primary intraorbital tumors.
    • Spinal astrocytomas often cause weaknesses of a variable extent and severity, ranging from monoparesis to quadriparesis. Pain along the involved vertebral segment may occur when the patient sneezes or coughs. Papilledema and hydrocephaly are present in 15% of patients and are attributed to increased cerebrospinal fluid (CSF) viscosity from an elevated protein content.
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Causes

  • Epidemiologic studies investigating parental occupational exposure, environmental exposure, and maternal nutritional intake failed to identify linkages with any of the childhood brain tumors.
  • An association with NF1 is present in 50-80% of patients with isolated optic nerve astrocytomas and in as many as 20% of those with chiasmal or deeper optic tract tumors. NF1 and tuberous sclerosis are also associated with other low-grade astrocytomas. Twenty percent of children with NF1 have low-grade gliomas, especially visual pathway tumors.
  • Astrocytoma is the most frequent CNS tumor in people with the Li-Fraumeni syndrome (germline mutation of the p53 tumor suppressor gene on the short arm of chromosome 17).
  • Ionizing radiation to the head for prior malignancies causes secondary supratentorial malignant astrocytomas in a small number of patients.
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Contributor Information and Disclosures
Author

Tobey MacDonald, MD  Clinical Director of Neuro-Oncology, Children's Hospital National Medical Center; Associate Professor, Department of Pediatric Hematology-Oncology, George Washington University

Tobey MacDonald, MD is a member of the following medical societies: American Association for Cancer Research, Children's Oncology Group, Pediatric Brain Tumor Consortium, and Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Coauthor(s)

Roger J Packer, MD  Senior Vice President, Neuroscience and Behavioral Medicine, Director, Brain Tumor Institute, Children's National Medical CenterProfessor of Neurology and Pediatrics, The George Washington University

Roger J Packer, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Pediatric Society, Child Neurology Society, Children's Oncology Group, Neurofibromatosis Clinical Trials Consortium, Pediatric Brain Tumor Consortium, and Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Specialty Editor Board

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Timothy P Cripe, MD, PhD  Professor of Pediatrics, Division of Hematology/Oncology, Cincinnati Children's Hospital Medical Center; Clinical Director, Musculoskeletal Tumor Program, Co-Medical Director, Office for Clinical and Translational Research, Cincinnati Children's Hospital Medical Center; Director of Pilot and Collaborative Clinical and Translational Studies Core, Center for Clinical and Translational Science and Training, University of Cincinnati College of Medicine

Timothy P Cripe, MD, PhD is a member of the following medical societies: American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

David Pallares, MD  Clinical Assistant Professor, Department of Pediatrics, Division of Allergy and Immunology, University of Louisville School of Medicine

David Pallares, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References

  1. Hales RK, Shokek O, Burger PC, Paynter NP, Chaichana KL, Quiñones-Hinojosa A, et al. Prognostic factors in pediatric high-grade astrocytoma: the importance of accurate pathologic diagnosis. J Neurooncol. Aug 2010;99(1):65-71. [Medline].

  2. Tihan T, Ersen A, Qaddoumi I, Sughayer MA, Tolunay S, Al-Hussaini M, et al. Pathologic characteristics of pediatric intracranial pilocytic astrocytomas and their impact on outcome in 3 countries: a multi-institutional study. Am J Surg Pathol. Jan 2012;36(1):43-55. [Medline].

  3. Belirgen M, Berrak SG, Ozdag H, Bozkurt SU, Eksioglu-Demiralp E, Ozek MM. Biologic tumor behavior in pilocytic astrocytomas. Childs Nerv Syst. Mar 2012;28(3):375-89. [Medline].

  4. Chintagumpala MM, Friedman HS, Stewart CF, et al. A phase II window trial of procarbazine and topotecan in children with high-grade glioma: a report from the children's oncology group. J Neurooncol. Apr 2006;77(2):193-8. [Medline].

  5. Geyer JR, Sposto R, Jennings M, et al. Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children's Cancer Group. J Clin Oncol. Oct 20 2005;23(30):7621-31. [Medline].

  6. Pollack IF, Hamilton RL, Sobol RW, et al. O6-methylguanine-DNA methyltransferase expression strongly correlates with outcome in childhood malignant gliomas: results from the CCG-945 Cohort. J Clin Oncol. Jul 20 2006;24(21):3431-7. [Medline].

  7. Akyüz C, Demir HA, Varan A, Yalçin B, Kutluk T, Büyükpamukçu M. Temozolomide in relapsed pediatric brain tumors: 14 cases from a single center. Childs Nerv Syst. Jan 2012;28(1):111-5. [Medline].

  8. Bouffet E, Jakacki R, Goldman S, et al. Phase II Study of weekly vinblastine in recurrent/refractory pediatric low-grade gliomas. Neuro-Oncology. 2008;10(3):450.

  9. Bredel M, Pollack IF, Hamilton RL, James CD. Epidermal growth factor receptor expression and gene amplification in high-grade non-brainstem gliomas of childhood. Clin Cancer Res. Jul 1999;5(7):1786-92. [Medline].

  10. Cokgor I, Friedman AH, Friedman HS. Gliomas. Eur J Cancer. Nov 1998;34(12):1910-5; discussion 1916-8. [Medline].

  11. Fernandez C, Figarella-Branger D, Girard N, et al. Pilocytic astrocytomas in children: prognostic factors--a retrospective study of 80 cases. Neurosurgery. Sep 2003;53(3):544-53; discussion 554-5. [Medline].

  12. Finlay JL, Boyett JM, Yates AJ, et al. Randomized phase III trial in childhood high-grade astrocytoma comparing vincristine, lomustine, and prednisone with the eight-drugs-in-1-day regimen. Childrens Cancer Group. J Clin Oncol. Jan 1995;13(1):112-23. [Medline].

  13. Finlay JL, Wisoff JH. The impact of extent of resection in the management of malignant gliomas of childhood. Childs Nerv Syst. Nov 1999;15(11-12):786-8. [Medline].

  14. Gilbertson RJ, Hill DA, Hernan R, et al. ERBB1 is amplified and overexpressed in high-grade diffusely infiltrative pediatric brain stem glioma. Clin Cancer Res. Sep 1 2003;9(10 Pt 1):3620-4. [Medline].

  15. Grill J, Couanet D, Cappelli C, et al. Radiation-induced cerebral vasculopathy in children with neurofibromatosis and optic pathway glioma. Ann Neurol. Mar 1999;45(3):393-6. [Medline].

  16. Gururangan S, Fisher MJ, Allen JC, Herndon JE 2nd, Quinn JA, Reardon DA, et al. Temozolomide in children with progressive low-grade glioma. Neuro Oncol. Apr 2007;9(2):161-8. [Medline].

  17. Guthrie BL, Laws ER Jr. Supratentorial low-grade gliomas. Neurosurg Clin N Am. Jan 1990;1(1):37-48. [Medline].

  18. Huncharek M, Wheeler L, McGarry R, Geschwind JF. Chemotherapy response rates in recurrent/progressive pediatric glioma; results of a systematic review. ALYSIS. Jul-Aug 1999;19(4C):3569-74. [Medline].

  19. Jacobson DM. Gliomas of the anterior visual pathways. Neurosurg Clin N Am. Oct 1999;10(4):683-98, ix. [Medline].

  20. Khatua S, Peterson KM, Brown KM, et al. Overexpression of the EGFR/FKBP12/HIF-2alpha pathway identified in childhood astrocytomas by angiogenesis gene profiling. Cancer Res. Apr 15 2003;63(8):1865-70. [Medline].

  21. Khaw SL, Coleman LT, Downie PA, Heath JA, Ashley DM. Temozolomide in pediatric low-grade glioma. Pediatr Blood Cancer. Nov 2007;49(6):808-11. [Medline].

  22. Komotar RJ, Mocco J, Carson BS, et al. Pilomyxoid astrocytoma: a review. MedGenMed. 2004;6(4):42. [Medline].

  23. Kuo DJ, Weiner HL, Wisoff J, et al. Temozolomide is active in childhood, progressive, unresectable, low-grade gliomas. J Pediatr Hematol Oncol. May 2003;25(5):372-8. [Medline].

  24. Lafay-Cousin L, Holm S, Qaddoumi I, et al. Weekly vinblastine in pediatric low-grade glioma patients with carboplatin allergic reaction. Cancer. Jun 15 2005;103(12):2636-42. [Medline].

  25. MacDonald TJ, Arenson EB, Ater J, et al. Phase II study of high-dose chemotherapy before radiation in children with newly diagnosed high-grade astrocytoma: final analysis of Children's Cancer Group Study 9933. Cancer. Dec 15 2005;104(12):2862-71. [Medline].

  26. Nadkarni TD, Rekate HL. Pediatric intramedullary spinal cord tumors. Critical review of the literature. Childs Nerv Syst. Jan 1999;15(1):17-28. [Medline].

  27. Nicholson HS, Krailo M, Ames MM, et al. Phase I study of temozolomide in children and adolescents with recurrent solid tumors: a report from the Children's Cancer Group. J Clin Oncol. Sep 1998;16(9):3037-43. [Medline].

  28. Packer RJ. Brain tumors in children. Arch Neurol. Apr 1999;56(4):421-5. [Medline].

  29. Pencalet P, Maixner W, Sainte-Rose C, et al. Benign cerebellar astrocytomas in children. J Neurosurg. Feb 1999;90(2):265-73. [Medline].

  30. Pollack IF. The role of surgery in pediatric gliomas. J Neurooncol. May 1999;42(3):271-88. [Medline].

  31. Pollack IF, Boyett JM, Finlay JL. Chemotherapy for high-grade gliomas of childhood. Childs Nerv Syst. Oct 1999;15(10):529-44. [Medline].

  32. Pollack IF, Finkelstein SD, Woods J, et al. Expression of p53 and prognosis in children with malignant gliomas. N Engl J Med. Feb 7 2002;346(6):420-7. [Medline].

  33. Prados MD, Edwards MS, Rabbitt J, Lamborn K, Davis RL, Levin VA. Treatment of pediatric low-grade gliomas with a nitrosourea-based multiagent chemotherapy regimen. J Neurooncol. May 1997;32(3):235-41. [Medline].

  34. Reddy AT, Packer RJ. Chemotherapy for low-grade gliomas. Childs Nerv Syst. Oct 1999;15(10):506-13. [Medline].

  35. Rubin G, Michowitz S, Horev G, et al. Pediatric brain stem gliomas: an update. Childs Nerv Syst. Apr-May 1998;14(4-5):167-73. [Medline].

  36. Sharif S, Ferner R, Birch JM, et al. Second primary tumors in neurofibromatosis 1 patients treated for optic glioma: substantial risks after radiotherapy. J Clin Oncol. Jun 1 2006;24(16):2570-5. [Medline].

  37. Stupp R, Mason WP, van den Bent MJ, et al. Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma. N Engl J Med. Mar 10 2005;352(10):987-96. [Medline].

  38. Thorarinsdottir HK, Rood B, Kamani N, et al. Outcome for children < 4 years of age with malignant central nervous system tumors treated with high-dose chemotherapy and autologous stem cell rescue. Pediatr Blood Cancer. Feb 2 2006;[Medline].

  39. Vredenburgh JJ, Desjardins A, Herndon JE 2nd, et al. Bevacizumab plus irinotecan in recurrent glioblastoma multiforme. J Clin Oncol. Oct 20 2007;25(30):4722-9. [Medline].

  40. Wisoff JH, Boyett JM, Berger MS, et al. Current neurosurgical management and the impact of the extent of resection in the treatment of malignant gliomas of childhood: a report of the Children's Cancer Group trial no. CCG-945. J Neurosurg. Jul 1998;89(1):52-9. [Medline].

 
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This MRI shows a juvenile pilocytic astrocytoma of the cerebellum.
This MRI shows a supratentorial glioblastoma multiforme.
This section displays the typical biphasic pattern of a juvenile pilocytic astrocytoma, consisting of dense, relatively anuclear, fibrillar areas alternating with looser cystic fields.
This section displays the high cellularity, mitosis, and nuclear atypia characteristic of an anaplastic astrocytoma (grade III).
This section displays a typical field of a glioblastoma multiforme (grade IV) with pseudopalisading neovascularity, nuclear atypia, numerous mitoses, and areas of hemorrhage.
 
 
 
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