Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Pediatric Astrocytoma Clinical Presentation

  • Author: Tobey J MacDonald, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
 
Updated: Nov 25, 2014
 

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.
    • Leroy et al conducted a retrospective study to determine whether severe anorexia might be a harbinger of low-grade astrocytomas of the fourth ventricle in pediatric patients.[3] Their review included 34 patients (16 boys, 18 girls; mean age, 8 years) who underwent surgical treatment of low-grade astrocytoma between 1991 and 2012. Of the study cohort, 31 patients had pilocytic astrocytoma, and 3 had diffuse astrocytoma. Seven of the patients presented with notable anorexia; the average body mass index (BMI) in this group was ≤2 standard deviation (SD). Twenty-seven of the patients had no anorexia; the average BMI in this group was +1 SD. There were no significant differences in these groups with regard to hydrocephalus or tumor location. For all the children with anorexia, BMI improved markedly during the postoperative follow-up period, which lasted on average 6 years. The investigators concluded that unexplained or atypical anorexia, in association with negative etiologic assessment, shouldprompt cerebral imaging. They indicated that clinical improvement after surgical resection could suggest a possible interaction between tumor tissue and appetite-suppressing peptide secretion.[3]
  • 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.
Next

Physical

See the list below:

  • 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.
Previous
Next

Causes

See the list below:

  • 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.
Previous
 
 
Contributor Information and Disclosures
Author

Tobey J MacDonald, MD Professor, Department of Pediatrics, Emory University School of Medicine; Director, Pediatric Brain Tumor Program, Aflac Chair for Neuro-Oncology, Aflac Cancer and Blood Disorders Center, Children's Healthcare of Atlanta

Tobey J MacDonald, MD is a member of the following medical societies: American Association for Cancer Research, Society for Neuro-Oncology, International Society of Paediatric 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, Society for Neuro-Oncology, Pediatric Brain Tumor Consortium, Neurofibromatosis Clinical Trials Consortium

Disclosure: Nothing to disclose.

Specialty Editor Board

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, FAAP Chief, Division of Hematology/Oncology/BMT, Gordon Teter Endowed Chair in Pediatric Cancer, Nationwide Children's Hospital; Professor of Pediatrics, Ohio State University College of Medicine

Timothy P Cripe, MD, PhD, FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Association for Cancer Research, American Pediatric Society, American Society of Gene and Cell Therapy, American Society of Pediatric Hematology/Oncology, Connective Tissue Oncology Society, Society for Pediatric Research, Children's Oncology Group

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

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

Disclosure: Nothing to disclose.

Acknowledgements

Samuel Gross, MD Professor Emeritus, Department of Pediatrics, University of Florida College of Medicine; Clinical Professor, Department of Pediatrics, University of North Carolina at Chapel Hill School of Medicine; Adjunct Professor, Department of Pediatrics, Duke University School of Medicine

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.

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. 2010 Aug. 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. 2012 Jan. 36(1):43-55. [Medline].

  3. Leroy HA, Baroncini M, Delestret I, Florent V, Vinchon M. Anorexia: an early sign of fourth ventricle astrocytoma in children. Childs Nerv Syst. 2014 Dec. 30(12):2089-95. [Medline].

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

  5. 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. 2006 Apr. 77(2):193-8. [Medline].

  6. 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. 2005 Oct 20. 23(30):7621-31. [Medline].

  7. 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. 2006 Jul 20. 24(21):3431-7. [Medline].

  8. 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. 2012 Jan. 28(1):111-5. [Medline].

  9. Ait Khelifa-Gallois N, Laroussinie F, Puget S, Sainte-Rose C, Dellatolas G. Long-term functional outcome of patients with cerebellar pilocytic astrocytoma surgically treated in childhood. Brain Inj. 2014 Nov 10. 1-8. [Medline].

  10. Chen L, Du C, Wang L, Yang C, Zhang JR, Li N, et al. Human positive coactivator 4 (PC4) is involved in the progression and prognosis of astrocytoma. J Neurol Sci. 2014 Sep 19. [Medline].

  11. 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.

  12. 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. 1999 Jul. 5(7):1786-92. [Medline].

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

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

  15. 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. 1995 Jan. 13(1):112-23. [Medline].

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

  17. 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. 2003 Sep 1. 9(10 Pt 1):3620-4. [Medline].

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

  19. 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. 2007 Apr. 9(2):161-8. [Medline].

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

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

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

  23. 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. 2003 Apr 15. 63(8):1865-70. [Medline].

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

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

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

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

  28. 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. 2005 Dec 15. 104(12):2862-71. [Medline].

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

  30. 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. 1998 Sep. 16(9):3037-43. [Medline].

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

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

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

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

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

  36. 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. 1997 May. 32(3):235-41. [Medline].

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

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

  39. 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. 2006 Jun 1. 24(16):2570-5. [Medline].

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

  41. Thorarinsdottir HK, Rood B, Kamani N, et al. Outcome for children 111111111111111111Pediatr Blood Cancer</i>. 2006 Feb 2. [Medline].

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

  43. 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. 1998 Jul. 89(1):52-9. [Medline].

Previous
Next
 
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.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.