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Pediatric Medulloblastoma Workup

  • Author: Tobey J MacDonald, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
 
Updated: Nov 26, 2014
 

Laboratory Studies

The routine pretreatment laboratory evaluation for medulloblastoma includes CBC count, electrolytes, liver, and renal function tests.

Baseline thyroid function studies and viral titers are also recommended.

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Imaging Studies

CT scanning

A CT scan of the head with and without contrast has more than 95% sensitivity for the detection of brain tumors.

On CT scans, prominent hydrocephalus and a solid, homogeneous, isodense to hyperdense, contrast-enhancing, midline cerebellar mass are characteristic of (although not diagnostic of) medulloblastoma.

MRI

Head and spinal MRI with and without gadolinium should be performed in all patients with CT or clinical findings consistent with medulloblastoma.

Other midline posterior fossa tumors, such as cerebellar astrocytoma and ependymoma, may have a similar appearance on CT.

MRI can be useful in such instances by better demonstrating the anatomic origin and extent of tumor (see the image below).

MRI showing a medulloblastoma of the cerebellum. MRI showing a medulloblastoma of the cerebellum.

Preoperative and postoperative MRI is required for detection and measurement of residual disease following surgical resection. Postoperative MRI evaluation should be performed within 72 hours of surgery to delineate residual tumor from the postsurgical inflammatory changes that are visualized on MRI at this time.

Spinal MRI is the most sensitive method available for detection of spinal cord metastasis.

Bone scan

Because medulloblastoma can metastasize outside the CNS, especially to bone, a bone scan with plain film correlation may be useful in symptomatic patients.

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Other Tests

A baseline hearing test (audiography or brainstem auditory-evoked response [BAER]) is recommended because of the potential toxicity from radiation and chemotherapy.

Some investigational treatment protocols may require additional tests, such as echocardiography, pulmonary function tests, or other more specific tests, for the purposes of monitoring treatment-related toxicity.

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Procedures

Lumbar puncture

CSF cytologic examination is useful for the detection of microscopic leptomeningeal tumor dissemination. However, neither clinical symptoms nor negative CSF cytologic findings can be relied on to indicate the presence of nodular spinal cord disease. As many as 50% of patients with positive spine MRI studies are asymptomatic and have negative cytologic results.

Funduscopic examination (or CT or MRI) must be performed before lumbar puncture (LP) to rule out the presence of hydrocephaly.

In known cases of medulloblastoma, LP generally is deferred until 2 weeks postoperation to avoid the presence of tumor cells that have disseminated as a result of surgery.

Bone marrow aspirate and biopsy

Medulloblastoma rarely metastasizes to bone marrow.

These tests should be reserved for patients who demonstrate abnormal peripheral blood findings that have no clear etiology.

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Histologic Findings

Medulloblastomas are undifferentiated embryonal neuroepithelial tumors of the cerebellum. They are highly cellular, soft, and friable tumors composed of cells with deeply basophilic nuclei of variable size and shape, little discernible cytoplasm, and often abundant mitoses (see the image below).

This section displays a typical medulloblastoma, c This section displays a typical medulloblastoma, composed of undifferentiated cells with deeply basophilic nuclei of variable size and shape and little discernible cytoplasm.

These characteristics give the microscopic appearance of a small, round, blue cell tumor. Morphologically identical tumors arising in the pineal region are termed pineoblastomas, and those arising in other CNS locations are called primitive neuroectodermal tumors (PNETs).

Homer-Wright rosettes (ringlike accumulations of tumor cell nuclei around a neuropil-containing or fibrillary core) and pseudorosettes are variably present (see the image below).

Section displaying Homer-Wright rosettes and pseud Section displaying Homer-Wright rosettes and pseudorosettes of a medulloblastoma.

These tumors express neuronal and neuroendocrine markers, including synaptophysin and neurofilament proteins.

Various degrees of glial or neuroblastic differentiation are noted, suggesting that the primitive cell of origin possesses the capacity for bipotential differentiation. A histologic variant with abundant stromal component, desmoplastic medulloblastoma, occurs dominantly in the lateral cerebellar areas of adolescents and adults. Another more recently described variant is characterized by marked features of anaplasia that is associated with MYCC oncogene amplification.

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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.

Steven K Bergstrom, MD Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, Children's Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology

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.

Additional Contributors

Kathleen M Sakamoto, MD, PhD Shelagh Galligan Professor, Division of Hematology/Oncology, Department of Pediatrics, Stanford University School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

References
  1. Grammel D, Warmuth-Metz M, von Bueren AO, Kool M, Pietsch T, Kretzschmar HA, et al. Sonic hedgehog-associated medulloblastoma arising from the cochlear nuclei of the brainstem. Acta Neuropathol. 2012 Feb 21. [Medline].

  2. Kieffer-Renaux V, Bulteau C, Grill J, et al. Patterns of neuropsychological deficits in children with medulloblastoma according to craniospatial irradiation doses. Dev Med Child Neurol. 2000 Nov. 42(11):741-5. [Medline].

  3. Deorah S, Lynch CF, Sibenaller ZA, Ryken TC. Trends in brain cancer incidence and survival in the United States: Surveillance, Epidemiology, and End Results Program, 1973 to 2001. Neurosurg Focus. 2006 Apr 15. 20(4):E1. [Medline].

  4. Massimino M, Cefalo G, Riva D, Biassoni V, Spreafico F, Pecori E, et al. Long-term results of combined preradiation chemotherapy and age-tailored radiotherapy doses for childhood medulloblastoma. J Neurooncol. 2012 Feb 16. [Medline].

  5. Tait DM, Thornton-Jones H, Bloom HJ, et al. Adjuvant chemotherapy for medulloblastoma: the first multi-centre control trial of the International Society of Paediatric Oncology (SIOP I). Eur J Cancer. 1990 Apr. 26(4):464-9. [Medline].

  6. Robertson PL, Muraszko KM, Holmes EJ, Sposto R, Packer RJ, Gajjar A, et al. Incidence and severity of postoperative cerebellar mutism syndrome in children with medulloblastoma: a prospective study by the Children's Oncology Group. J Neurosurg. 2006 Dec. 105(6 Suppl):444-51. [Medline].

  7. Verma J, Mazloom A, Teh BS, South M, Butler EB, Paulino AC. Comparison of supine and prone craniospinal irradiation in children with medulloblastoma. Pract Radiat Oncol. 2014 Oct 2. [Medline].

  8. Min C, Paganetti H, Winey BA, Adams J, MacDonald SM, Tarbell NJ, et al. Evaluation of permanent alopecia in pediatric medulloblastoma patients treated with proton radiation. Radiat Oncol. 2014 Nov 18. 9(1):220. [Medline].

  9. Brown HG, Kepner JL, Perlman EJ, et al. "Large cell/anaplastic" medulloblastomas: a Pediatric Oncology Group Study. J Neuropathol Exp Neurol. 2000 Oct. 59(10):857-65. [Medline].

  10. Holland AA, Hughes CW, Stavinoha PL. School Competence and Fluent Academic Performance: Informing Assessment of Educational Outcomes in Survivors of Pediatric Medulloblastoma. Appl Neuropsychol Child. 2014 Nov 14. 1-8. [Medline].

  11. Dufour C, Beaugrand A, Pizer B, Micheli J, Aubelle MS, Fourcade A, et al. Metastatic Medulloblastoma in Childhood: Chang's Classification Revisited. Int J Surg Oncol. 2012. 2012:245385. [Medline]. [Full Text].

  12. Cohen BH, Zeltzer PM, Boyett JM, et al. Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: a Childrens Cancer Group randomized trial. J Clin Oncol. 1995 Jul. 13(7):1687-96. [Medline].

  13. 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. 1996 Feb. 38(2):265-71. [Medline].

  14. Boon K, Eberhart CG, Riggins GJ. Genomic amplification of orthodenticle homologue 2 in medulloblastomas. Cancer Res. 2005 Feb 1. 65(3):703-7. [Medline].

  15. Duffner PK, Horowitz ME, Krischer JP, et al. Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors [see comments]. N Engl J Med. 1993 Jun 17. 328(24):1725-31. [Medline].

  16. Dunkel IJ, Finlay JL. High dose chemotherapy with autologous stem cell rescue for patients with medulloblastoma. J Neurooncol. 1996 Jul. 29(1):69-74. [Medline].

  17. Dupuis-Girod S, Hartmann O, Benhamou E, et al. Will high dose chemotherapy followed by autologous bone marrow transplantation supplant cranio-spinal irradiation in young children treated for medulloblastoma?. J Neurooncol. 1996 Jan. 27(1):87-98. [Medline].

  18. Eberhart CG, Burger PC. Anaplasia and grading in medulloblastomas. Brain Pathol. 2003 Jul. 13(3):376-85. [Medline].

  19. Gajjar A, Chintagumpala M, Ashley D, et al. Risk-adapted craniospinal radiotherapy followed by high-dose chemotherapy and stem-cell rescue in children with newly diagnosed medulloblastoma (St Jude Medulloblastoma-96): long-term results from a prospective, multicentre trial. Lancet Oncol. 2006 Oct. 7(10):813-20. [Medline].

  20. Gajjar A, Fouladi M, Walter AW, et al. Comparison of lumbar and shunt cerebrospinal fluid specimens for cytologic detection of leptomeningeal disease in pediatric patients with brain tumors. J Clin Oncol. 1999 Jun. 17(6):1825-8. [Medline].

  21. 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].

  22. Grotzer MA, Hogarty MD, Janss AJ, et al. MYC messenger RNA expression predicts survival outcome in childhood primitive neuroectodermal tumor/medulloblastoma. Clin Cancer Res. 2001 Aug. 7(8):2425-33. [Medline]. [Full Text].

  23. Grotzer MA, Janss AJ, Fung K, et al. TrkC expression predicts good clinical outcome in primitive neuroectodermal brain tumors. J Clin Oncol. 2000 Mar. 18(5):1027-35. [Medline].

  24. Hallahan AR, Pritchard JI, Hansen S, et al. The SmoA1 mouse model reveals that notch signaling is critical for the growth and survival of sonic hedgehog-induced medulloblastomas. Cancer Res. 2004 Nov 1. 64(21):7794-800. [Medline]. [Full Text].

  25. Hernan R, Fasheh R, Calabrese C, et al. ERBB2 up-regulates S100A4 and several other prometastatic genes in medulloblastoma. Cancer Res. 2003 Jan 1. 63(1):140-8. [Medline].

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

  27. Kim JY, Sutton ME, Lu DJ, et al. Activation of neurotrophin-3 receptor TrkC induces apoptosis in medulloblastomas. Cancer Res. 1999 Feb 1. 59(3):711-9. [Medline]. [Full Text].

  28. Kortmann RD, Kuhl J, Timmermann B, et al. Postoperative neoadjuvant chemotherapy before radiotherapy as compared to immediate radiotherapy followed by maintenance chemotherapy in the treatment of medulloblastoma in childhood: results of the German prospective randomized trial HIT '91. Int J Radiat Oncol Biol Phys. 2000 Jan 15. 46(2):269-79. [Medline].

  29. MacDonald TJ, Brown KM, LaFleur B, et al. Expression profiling of medulloblastoma: PDGFRA and the RAS/MAPK pathway as therapeutic targets for metastatic disease. Nat Genet. 2001 Oct. 29(2):143-52. [Medline].

  30. Merchant TE, Kun LE, Krasin MJ, et al. Multi-institution prospective trial of reduced-dose craniospinal irradiation (23.4 Gy) followed by conformal posterior fossa (36 Gy) and primary site irradiation (55.8 Gy) and dose-intensive chemotherapy for average-risk medulloblastoma. Int J Radiat Oncol Biol Phys. 2008 Mar 1. 70(3):782-7. [Medline].

  31. Oliver TG, Grasfeder LL, Carroll AL, et al. Transcriptional profiling of the Sonic hedgehog response: a critical role for N-myc in proliferation of neuronal precursors. Proc Natl Acad Sci U S A. 2003 Jun 10. 100(12):7331-6. [Medline]. [Full Text].

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

  33. Packer RJ, Finlay JL. Medulloblastoma: presentation, diagnosis and management. Oncology (Huntingt). 1988 Sep. 2(9):35-45, 48-9. [Medline].

  34. Packer RJ, Gajjar A, Vezina G, et al. Phase III study of craniospinal radiation therapy followed by adjuvant chemotherapy for newly diagnosed average-risk medulloblastoma. J Clin Oncol. 2006 Sep 1. 24(25):4202-8. [Medline].

  35. Packer RJ, Goldwein J, Nicholson HS, et al. Treatment of children with medulloblastomas with reduced-dose craniospinal radiation therapy and adjuvant chemotherapy: A Children's Cancer Group Study. J Clin Oncol. 1999 Jul. 17(7):2127-36. [Medline].

  36. Packer RJ, Sutton LN, Elterman R, et al. Outcome for children with medulloblastoma treated with radiation and cisplatin, CCNU, and vincristine chemotherapy. J Neurosurg. 1994 Nov. 81(5):690-8. [Medline].

  37. Pizzo PA, Poplack DG. Tumors of the central nervous system. Principles and Practice of Pediatric Oncology. 3rd ed. 633-97.

  38. Pomeroy SL, Tamayo P, Gaasenbeek M, et al. Prediction of central nervous system embryonal tumour outcome based on gene expression. Nature. 2002. 415(6870):436-42.

  39. Reddy AT, Packer RJ. Pediatric central nervous system tumors. Curr Opin Oncol. 1998 May. 10(3):186-93. [Medline].

  40. Romer JT, Kimura H, Magdaleno S, et al. Suppression of the Shh pathway using a small molecule inhibitor eliminates medulloblastoma in Ptc1(+/-)p53(-/-) mice. Cancer Cell. 2004 Sep. 6(3):229-40. [Medline].

  41. Rutkowski S, Bode U, Deinlein F, et al. Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med. 2005 Mar 10. 352(10):978-86. [Medline].

  42. Siu IM, Lal A, Blankenship JR, et al. c-Myc promoter activation in medulloblastoma. Cancer Res. 2003 Aug 15. 63(16):4773-6. [Medline]. [Full Text].

  43. Stearns D, Chaudhry A, Abel TW, et al. c-myc overexpression causes anaplasia in medulloblastoma. Cancer Res. 2006 Jan 15. 66(2):673-81. [Medline].

  44. Strother D, Ashley D, Kellie SJ, et al. Feasibility of four consecutive high-dose chemotherapy cycles with stem-cell rescue for patients with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor after craniospinal radiotherapy: results of a collaborative study. J Clin Oncol. 2001 May 15. 19(10):2696-704. [Medline].

  45. Thomas PR, Deutsch M, Kepner JL, et al. Low-stage medulloblastoma: final analysis of trial comparing standard- dose with reduced-dose neuraxis irradiation. J Clin Oncol. 2000 Aug. 18(16):3004-11. [Medline].

  46. Thorarinsdottir HK, Rood B, Kamani N, et al. Outcome for children 111111111111111111Pediatr Blood Cancer</i>. 2007 Mar. 48(3):278-84. [Medline].

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

  48. Yachnis AT. Neuropathology of pediatric brain tumors. Semin Pediatr Neurol. 1997 Dec. 4(4):282-91. [Medline].

  49. Zeltzer PM, Boyett JM, Finlay JL, 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. 1999 Mar. 17(3):832-45. [Medline].

  50. Zerbini C, Gelber RD, Weinberg D, et al. Prognostic factors in medulloblastoma, including DNA ploidy. J Clin Oncol. 1993 Apr. 11(4):616-22. [Medline].

 
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MRI showing a medulloblastoma of the cerebellum.
Section displaying Homer-Wright rosettes and pseudorosettes of a medulloblastoma.
This section displays a typical medulloblastoma, composed of undifferentiated cells with deeply basophilic nuclei of variable size and shape and little discernible cytoplasm.
 
 
 
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