Medulloblastoma Follow-up

  • Author: George I Jallo, MD; Chief Editor: Amy Kao, MD   more...
 
Updated: Mar 9, 2012
 

Further Inpatient Care

  • Admit patients in whom further surgical intervention is being contemplated.
  • Chemotherapy usually is administered on an inpatient basis.
Next

Further Outpatient Care

  • Imaging is the primary mode of monitoring residual disease, efficacy of continuing medical treatment, and recurrence or metastasis. Because medulloblastoma is aggressive, frequent monitoring is essential. MRI should be repeated every 3 months the first year; every 4 months the second year; every 6 months the following 3 years; and yearly thereafter.
  • Radiation therapy is an outpatient procedure.
  • Any signs of change in mental status are indications for outpatient visits, as they may herald hydrocephalus and possible recurrence.
  • Lower cranial nerve or cerebellar signs also may signal recurrence.
  • Taper steroids and monitor adverse effects.
Previous
Next

Inpatient & Outpatient Medications

  • Taper steroid use. However, chemotherapy and radiation therapy may exacerbate edema and necessitate low-dose corticosteroids for a short period of time.
  • Antiepileptic medication usually is not necessary when the tumor is located in the posterior fossa.
  • Spread of disease to the supratentorial compartment may cause seizures and indicate antiseizure medication.
Previous
Next

Transfer

Transfer may be necessary for treatment at a center familiar with pediatric neurosurgery, pediatric oncology, or pediatric radiotherapy.

Previous
Next

Deterrence/Prevention

No known precautions currently exist to prevent the disease or its recurrence.

Previous
Next

Complications

  • Hydrocephalus (the most common complication of medulloblastoma) can cause secondary visual problems. Cerebellar dysfunction (the second most common complication of the disease) may lead to problems with coordination and gait. Cranial nerve palsy from brainstem involvement can lead to difficulties with vision, speech, and swallowing. With subarachnoid spread to the spinal cord, the unfortunate complications are radiculopathy and weakness.
  • Complications accompany the treatment of medulloblastoma. Fortunately, most of these complications are transient. The most common complication after surgery is a temporary worsening of ataxia accompanied by nystagmus. One of the most commonly cited complications is cerebellar mutism. The anatomic site of origin is thought to be the deep cerebellar nuclei. The constellation of symptoms includes apathy, minimal-to-absent speech, emotional lability, and refusal to initiate movement. Hemiparesis can accompany mutism. Lower cranial nerves are intact, but the syndrome is accompanied by a swallowing apraxia. It becomes apparent several hours after surgery and persists for several weeks, usually resolving completely. Other complications include a temporary Parinaud syndrome and pneumocephalus. A common complication of any surgery in the posterior fossa is aseptic meningitis. This can be alleviated with a short course of corticosteroids.
  • Complications of radiation therapy have been discussed previously and include lowered IQ, small stature, endocrine dysfunction, behavioral abnormalities, secondary neoplasms, and radiation necrosis of the white matter.
  • Chemotherapy also has numerous adverse effects on multiple organ systems including renal toxicity, ototoxicity, hepatotoxicity, pulmonary fibrosis, and gastrointestinal disturbance. Methotrexate, when used in combination with irradiation, can cause permanent necrotizing leukoencephalopathy.
Previous
Next

Prognosis

  • Medulloblastoma is a very aggressive tumor. Even after a good response to surgery and radiation, recurrence is common; most recurrences occur within 2 years after treatment.
    • The most common location of recurrence is at the primary tumor site in the posterior fossa.
    • With the use of adjuvant chemotherapy, incidence of recurrence in the spinal canal and the supratentorial region seems to decrease.
  • Systemic metastases, in the absence of a CSF shunting system, are also a recognized problem in 10-20% of patients. Bone is the most common site of systemic metastasis; regional lymph node sites follow.
  • The Collin law was first hypothesized for Wilms tumor and has been expanded since to cover many pediatric tumors thought to be congenital in origin.
    • The Collin law states that, if a tumor has not recurred in a period of time equal to age of patient plus 9 months, that patient can be considered to be cured.
    • The Collin law generally holds for medulloblastoma; however, several late recurrences (longer than 10 years after diagnosis) have been reported. The 5-year progression-free survival rate in that group is 70-80% for patients at low risk and 60-70% for patients at high risk.
  • Greater age at diagnosis has been associated with a better prognosis, most likely because adults more often harbor the less aggressive desmoplastic variant of medulloblastoma.
  • Why females have a longer recurrence-free interval is not understood.
Previous
Next

Patient Education

Teach patients and families about early signs of hydrocephalus, especially if the patient has a ventricular shunt in place.

Previous
 
Contributor Information and Disclosures
Author

George I Jallo, MD  Professor of Neurosurgery, Pediatrics, and Oncology, Director, Clinical Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine

George I Jallo, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, and American Society of Pediatric Neurosurgeons

Disclosure: Codman (Johnson & Johnson) Grant/research funds Consulting; Medtronic Grant/research funds Consulting

Coauthor(s)

Alvin Marcovici, MD  Consulting Staff, Southcoast Neurosurgery

Alvin Marcovici, MD is a member of the following medical societies: American Association of Neurological Surgeons, Congress of Neurological Surgeons, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Specialty Editor Board

Raj D Sheth, MD  Professor, Mayo College of Medicine; Chief, Division of Pediatric Neurology, Nemours Children's Clinic

Raj D Sheth, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Neurological Association, and Child Neurology Society

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Kenneth J Mack, MD, PhD  Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic

Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience

Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD  Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society

Disclosure: Nothing to disclose.

References

  1. Bailey P, Cushing H. A common type of midcerebellar glioma of childhood. Arch Neurol Psychiatr. 1925;14:192-224.

  2. Rorke LB. The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors. J Neuropathol Exp Neurol. Jan 1983;42(1):1-15. [Medline].

  3. Rieken S, Mohr A, Habermehl D, Welzel T, Lindel K, Witt O, et al. Outcome and prognostic factors of radiation therapy for medulloblastoma. Int J Radiat Oncol Biol Phys. Nov 1 2011;81(3):e7-e13. [Medline].

  4. Gupta T, Jalali R, Goswami S, Nair V, Moiyadi A, Epari S, et al. Early Clinical Outcomes Demonstrate Preserved Cognitive Function in Children with Average-Risk Medulloblastoma When Treated with Hyperfractionated Radiation Therapy. Int J Radiat Oncol Biol Phys. Feb 16 2012;[Medline].

  5. Haddy N, Mousannif A, Tukenova M, et al. Relationship between the brain radiation dose for the treatment of childhood cancer and the risk of long-term cerebrovascular mortality. Brain. May 2011;134:1362-72. [Medline].

  6. Albright AL, Wisoff JH, Zeltzer PM, Boyett JM, Rorke LB, Stanley P. Effects of medulloblastoma resections on outcome in children: a report from the Children's Cancer Group. Neurosurgery. Feb 1996;38(2):265-71. [Medline].

  7. Allen JC, Epstein F. Medulloblastoma and other primary malignant neuroectodermal tumors of the CNS. The effect of patients' age and extent of disease on prognosis. J Neurosurg. Oct 1982;57(4):446-51. [Medline].

  8. Ater JL, van Eys J, Woo SY, et al. MOPP chemotherapy without irradiation as primary postsurgical therapy for brain tumors in infants and young children. J Neurooncol. May 1997;32(3):243-52. [Medline].

  9. Bailey CC, Gnekow A, Wellek S, et al. Prospective randomised trial of chemotherapy given before radiotherapy in childhood medulloblastoma. International Society of Paediatric Oncology (SIOP) and the (German) Society of Paediatric Oncology (GPO): SIOP II. Med Pediatr Oncol. Sep 1995;25(3):166-78. [Medline].

  10. Balter-Seri J, Mor C, Shuper A, et al. Cure of recurrent medulloblastoma: the contribution of surgical resection at relapse. Cancer. Mar 15 1997;79(6):1241-7. [Medline].

  11. Blaser SI, Harwood-Nash DC. Neuroradiology of pediatric posterior fossa medulloblastoma. J Neurooncol. Jul 1996;29(1):23-34. [Medline].

  12. Carrie C, Muracciole X, Gomez F, et al. Conformal radiotherapy, reduced boost volume, hyperfractionated radiotherapy, and online quality control in standard-risk medulloblastoma without chemotherapy: results of the French M-SFOP 98 protocol. Int J Radiat Oncol Biol Phys. Nov 1 2005;63(3):711-6. [Medline].

  13. Chang CH, Housepian EM, Herbert C. An operative staging system and a megavoltage radiotherapeutic technic for cerebellar medulloblastomas. Radiology. Dec 1969;93(6):1351-9. [Medline].

  14. Cohen BH, Packer RJ. Chemotherapy for medulloblastomas and primitive neuroectodermal tumors. J Neurooncol. Jul 1996;29(1):55-68. [Medline].

  15. Dennis M, Spiegler BJ, Hetherington CR, et al. Neuropsychological sequelae of the treatment of children with medulloblastoma. J Neurooncol. Jul 1996;29(1):91-101. [Medline].

  16. Dunkel IJ, Boyett JM, Yates A, Rosenblum M, Garvin JH Jr, Bostrom BC, et al. High-dose carboplatin, thiotepa, and etoposide with autologous stem-cell rescue for patients with recurrent medulloblastoma. Children's Cancer Group. J Clin Oncol. Jan 1998;16(1):222-8. [Medline].

  17. Friedberg MH, David O, Adelman LS. Recurrence of medulloblastoma: violation of Collins' law after two decades. Surg Neurol. Jun 1997;47(6):571-4. [Medline].

  18. Giordana MT, Cavalla P, Dutto A, et al. Is medulloblastoma the same tumor in children and adults?. J Neurooncol. Nov 1997;35(2):169-76. [Medline].

  19. Giordana MT, Migheli A, Pavanelli E. Isochromosome 17q is a constant finding in medulloblastoma. An interphase cytogenetic study on tissue sections. Neuropathol Appl Neurobiol. Jun 1998;24(3):233-8. [Medline].

  20. Giordana MT, Schiffer P, Schiffer D. Prognostic factors in medulloblastoma. Childs Nerv Syst. Jun 1998;14(6):256-62. [Medline].

  21. Giralt J, Sánchez de Toledo J, Moraga F, et al. Improving survival of medulloblastoma: results in two groups of patients. Oncology. Jan-Feb 1996;53(1):38-42. [Medline].

  22. Jenkin D. The radiation treatment of medulloblastoma. J Neurooncol. Jul 1996;29(1):45-54. [Medline].

  23. Kiltie AE, Lashford LS, Gattamaneni HR. Survival and late effects in medulloblastoma patients treated with craniospinal irradiation under three years old. Med Pediatr Oncol. May 1997;28(5):348-54. [Medline].

  24. Miralbell R, Bieri S, Huguenin P, et al. Prognostic value of cerebrospinal fluid cytology in pediatric medulloblastoma. Swiss Pediatric Oncology Group. Ann Oncol. Feb 1999;10(2):239-41. [Medline].

  25. Pollack IF, Polinko P, Albright AL, et al. Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: incidence and pathophysiology. Neurosurgery. Nov 1995;37(5):885-93. [Medline].

  26. Rossi A, Caracciolo V, Russo G, Reiss K, Giordano A. Medulloblastoma: from molecular pathology to therapy. Clin Cancer Res. Feb 15 2008;14(4):971-6. [Medline].

  27. Rutka JT, Hoffman HJ. Medulloblastoma: a historical perspective and overview. J Neurooncol. Jul 1996;29(1):1-7. [Medline].

  28. Saran FH, Driever PH, Thilmann C, et al. Survival of very young children with medulloblastoma (primitive neuroectodermal tumor of the posterior fossa) treated with craniospinal irradiation. Int J Radiat Oncol Biol Phys. Dec 1 1998;42(5):959-67. [Medline].

  29. Schmandt S, Kuhl J. Chemotherapy as prophylaxis and treatment of meningosis in children less than 3 years of age with medulloblastoma. J Neurooncol. Jun-Jul 1998;38(2-3):187-92. [Medline].

  30. Sure U, Berghorn WJ, Bertalanffy H. Collins' law. Prediction of recurrence or cure in childhood medulloblastoma?. Clin Neurol Neurosurg. May 1997;99(2):113-6. [Medline].

  31. Sutton LN, Phillips PC, Molloy PT. Surgical management of medulloblastoma. J Neurooncol. Jul 1996;29(1):9-21. [Medline].

  32. Tomita T. Medulloblastomas. In: Youmans JR, ed. Neurolgical Surgery. Vol 4. 5th ed. Philadelphia: WB Saunders; 1996:2570-92.

  33. Weil MD, Lamborn K, Edwards MS, Wara WM. Influence of a child's sex on medulloblastoma outcome. JAMA. May 13 1998;279(18):1474-6. [Medline].

  34. Weitman DM, Cogen PH. Contemporary management of medulloblastoma. Contemporary Neurosurgery. 1998;20(2):1-8.

  35. Whelan HT, Krouwer HG, Schmidt MH, et al. Current therapy and new perspectives in the treatment of medulloblastoma. Pediatr Neurol. Feb 1998;18(2):103-15. [Medline].

  36. Yang SY, Wang KC, Cho BK, et al. Radiation-induced cerebellar glioblastoma at the site of a treated medulloblastoma: case report. J Neurosurg. May 2005;102(4 Suppl):417-22. [Medline].

  37. Zeltzer PM, Boyett JM, Finlay JL, Albright AL, Rorke LB, Milstein JM, et al. Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children's Cancer Group 921 randomized phase III study. J Clin Oncol. Mar 1999;17(3):832-45. [Medline].

 
Previous
Next
 
CT scan demonstrates a hyperdense lesion within the posterior fossa of an 8-year-old boy who presented with nausea and vomiting.
T1-weighted sagittal MRI of an 8-year-old boy who presented with nausea and vomiting reveals an enhancing tumor within the fourth ventricle. The child underwent a suboccipital craniotomy and resection of his medulloblastoma.
T1-weighted sagittal MRI of 4-year-old boy who presented with gait ataxia and precocious puberty. MRI shows a heterogenous enhancing tumor located within the fourth ventricle with marked hydrocephalus.
T1-weighted axial MRI shows heterogeneous enhancement of the medulloblastoma in a 4-year-old boy who presented with gait ataxia and precocious puberty.
Coronal MRI confirms the presence of the tumor within the fourth ventricle of a 4-year-old boy who presented with gait ataxia and precocious puberty.
High-power magnification hematoxylin and eosin (H&E) section of a typical medulloblastoma
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.