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Presentation of Medulloblastomas in Children

This page was last updated on April 8th, 2024

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Symptoms and Signs

Obstructive hydrocephalus

  • Elevated ICP: Patients with a medulloblastoma most commonly present with signs and symptoms of elevated ICP from obstructive hydrocephalus, especially headaches and vomiting.
  • Nonspecific signs in infants and young children: Younger patients may present with irritability alone, sometimes associated with macrocephaly and/or full fontanelle, or loss of developmental milestones.  Infants may also present with torticollis (15).

Less common signs and symptoms

  • Cerebellar signs: Signs of cerebellar dysfunction such as truncal or appendicular ataxia and gait abnormalities may be present.
  • Cranial neuropathies: Cranial neuropathies, especially sixth nerve palsy, may also be present.

Time for evolution

  • Rapid evolution typical: Symptoms typically evolve rapidly in patients with malignant neoplasms such as medulloblastoma (18).

Evaluation at Presentation

Intervention

Stabilization

  • Admission to intensive care unit: If any signs or symptoms of obstructive hydrocephalus or of rapidly worsening condition on neurological examination are present, the patient should be admitted immediately to an ICU.
  • Dexamethasone: Dexamethasone should be started on admission (0.5–1 mg/kg IV loading dose, up to 10 mg, then 0.25–0.5 mg/kg/day IV or PO divided every 6 hours).
  • Antacid: An antacid should be started for gastrointestinal protection when dexamethasone is started.

Preparation for definitive intervention, nonemergent

  • Type and cross-match packed red blood cells in preparation for surgery:  Malignant tumors may be quite vascular, and young children may have extensive venous sinuses in the posterior fossa dura.
  • MRI of the brain and total spine: Preoperative MRI with and without gadolinium contrast should be obtained.

Hydrocephalus management

  • Dexamethasone Dexamethasone is administered (0.5–1 mg/kg IV loading dose, up to 10 mg, then 0.25–0.5 mg/kg/day IV or PO divided every 6 hours) accompanied by an antacid for gastrointestinal protection, admission to an intensive care unit, and timely tumor resection.
  • Ventriculostomy placement: A ventriculostomy may be placed when the patient exhibits lethargy, bradycardia, or neurological deterioration.  Avoid rapid decompression of the ventricles, and set no lower than 15 cm H2O above the external auditory meatus to minimize the risk of upward herniation.
  • ETV before tumor resection: This is an area of current controversy.  It may reduce the incidence of postoperative hydrocephalus (22).  However, only a minority of children require CSF diversion after tumor resection (6).
  • Shunt placement: Shunt placement is not currently recommended, as only a few patients require permanent CSF diversion, and it exposes patients to a lifetime of shunt dependency and shunt-related complications.

Admission Orders

  • HOB, positioning and activity: Bed rest with head of bed at 30 degrees is indicated for patients presenting with symptomatic hydrocephalus.
  • VS: Hourly VS with neurological checks are indicated for any patient with mental status changes, vomiting, or signs of elevated ICP.
  • BP parameters: BP is monitored hourly, but no specific parameters are typically ordered pre-operatively
  • IVF and rate: Isotonic fluid such as normal saline is administered with the rate determined on the basis of the patient’s weight.
  • CSF drainage parameters: Most patients do not require ventriculostomy placement pre-operatively. If a ventriculostomy is required, care must be taken to avoid overdrainage. Initially, the drain is placed at a height of 20 cm H2O above the external auditory meatus, a maximum of 10 ml per hour is drained, and ICP is monitored.
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References

  1. Albright AL, Wisoff JH, Zeltzer PM, et al: Effects of medulloblastoma resections on outcome in children: a report from the Children's Cancer Group. Neurosurgery 38:265-271, 1996
  2. 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 25:166-178, 1995
  3. Chatty EM, Earle KM: Medulloblastoma. A report of 201 cases with emphasis on the relationship of histologic variants to survival. Cancer 28:977-983, 1971
  4. Dunkel IJ, Boyett JM, Yates A, 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 16:222-228, 1998
  5. Evans DG, Farndon PA, Burnell LD, et al: The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma. Br J Cancer 64:959-961, 1991
  6. Fritsch MJ, Doerner L, Kienke S, et al: Hydrocephalus in children with posterior fossa tumors: role of endoscopic third ventriculostomy. J Neurosurg 103:40-42, 2005.
  7. Halberg FE, Wara WM, Fippin LF, et al: Low-dose craniospinal radiation therapy for medulloblastoma. Int J Radiat Oncol Biol Phys 20:651-654, 1991
  8. Hershatter BW, Halperin EC, Cox EB: Medulloblastoma: the Duke University Medical Center experience. Int J Radiat Oncol Biol Phys 12:1771-1777, 1986
  9. Johnson DL, McCabe MA, Nicholson HS, et al: Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80:1004-1010, 1994
  10. Kimmings E, Kleinlugtebeld AT, Casey A, et al: Medulloblastoma: factors influencing the educational potential of surviving children. Br J Neurosurg 9:611-617, 1995
  11. Lee M, Wisoff JH, Abbott R, et al: Management of hydrocephalus in children with medulloblastoma: prognostic factors for shunting. Pediatr Neurosurg 20:240-247, 1994
  12. Louis DN, Ohgaki H, Wiestler OD, et al (eds): WHO Classification of Tumours of the Central Nervous System. Lyon: IARC Press, 2007, pp 132-136
  13. Maher CO, Friedman JA, Raffel C: Posterior fossa tumors in children, in Batjer HH, Loftus CM (eds): Textook of Neurological Surgery:  Principles and Practice. Philadelphia: Lippincott Williams & Wilkins, 2003, p 985
  14. Meyers SP, Kemp SS, Tarr RW: MR imaging features of medulloblastomas. AJR Am J Roentgenol 158:859-865, 1992
  15. Muraszko KM, Brahma B, Orringer D: Medulloblastomas, in Albright AL, Pollack IF, Adelson PD (eds): Principles and Practice of Pediatric Neurosurgery. New York: Thieme Medical Publishers, Inc., 2008, pp 605-607
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  19. Roberts RO, Lynch CF, Jones MP, et al: Medulloblastoma: a population-based study of 532 cases. J Neuropathol Exp Neurol 50:134-144, 1991
  20. Rutka JT, Kuo JS: Pediatric surgical neuro-oncology: current best care practices and strategies. J Neurooncol 69:139-150, 2004
  21. Rutkowski S, Bode U, Deinlein F, et al: Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med 352:978-986, 2005
  22. Sainte-Rose C, Cinalli G, Roux FE, et al: Management of hydrocephalus in pediatric patients with posterior fossa tumors: the role of endoscopic third ventriculostomy. J Neurosurg 95:791-797, 2001
  23. Evans AE, Jenkin RD, Sposto R, et al: The treatment of medulloblastoma: results of a prospective randomized trial of radiation therapy with and without CCNU, vincristine, and prednisone. J Neurosurg 72:573-582, 1990
  24. Steinbok P, Cochrane DD, Perrin R, et al: Mutism after posterior fossa tumour resection in children: incomplete recovery on long-term follow-up. Pediatr Neurosurg 39:179-183, 2003
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