Medulloblastomas with extensive posttherapy neuronal maturation

Report of two cases

Dan X. Cai Departments of Pathology and Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri; Department of Pathology, Hospital S. Jose, Lisbon, Portugal; and Department of Pathology/Laboratory Medicine, Mayo Clinic, Rochester, Minnesota

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Manuela Mafra Departments of Pathology and Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri; Department of Pathology, Hospital S. Jose, Lisbon, Portugal; and Department of Pathology/Laboratory Medicine, Mayo Clinic, Rochester, Minnesota

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Robert E. Schmidt Departments of Pathology and Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri; Department of Pathology, Hospital S. Jose, Lisbon, Portugal; and Department of Pathology/Laboratory Medicine, Mayo Clinic, Rochester, Minnesota

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Bernd W. Scheithauer Departments of Pathology and Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri; Department of Pathology, Hospital S. Jose, Lisbon, Portugal; and Department of Pathology/Laboratory Medicine, Mayo Clinic, Rochester, Minnesota

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Tae Sung Park Departments of Pathology and Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri; Department of Pathology, Hospital S. Jose, Lisbon, Portugal; and Department of Pathology/Laboratory Medicine, Mayo Clinic, Rochester, Minnesota

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Arie Perry Departments of Pathology and Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri; Department of Pathology, Hospital S. Jose, Lisbon, Portugal; and Department of Pathology/Laboratory Medicine, Mayo Clinic, Rochester, Minnesota

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✓ The authors report on two patients with classic medulloblastoma, each of whom underwent extensive therapy-associated neuronal maturation. The first patient presented at 3 months of age with hydrocephalus caused by a 5-cm tumor in the cerebellar vermis. He underwent a gross-total resection of a desmoplastic medulloblastoma. No mature elements were identified. Despite adjuvant chemotherapy, a 1.5-cm recurrent tumor developed 6 months later. Sections from the subtotally resected tumor demonstrated exclusively mature neuronal elements, ranging from neurocytes to ganglion cells. Four months later, a second recurrent tumor was resected. The specimen collected this time demonstrated classic medulloblastoma morphological characteristics. The patient was subsequently treated with radiation therapy, which seemed to have an effect; however, the tumor eventually progressed and the patient died. The second patient presented at 3 years of age with a midline medulloblastoma and was treated with subtotal resection, radiation therapy, and chemotherapy. Although the tumor remained stable on radiographic imaging, a second resection was performed 8 years later to alleviate hydrocephalus. Histological examination revealed predominantly small mature neurons with scattered ganglion cells and extensive calcification. No adjuvant therapy was given and the patient is alive and well as of his last follow-up examination.

The mature neuronal neoplasms resected in both patients demonstrated negligible proliferative indices and stained appropriately with neuronal immunohistochemical markers. The smaller neuronal population resembled those of a central neurocytoma and medullocytoma/cerebellar neurocytoma. Analogous to neuroblastoma, our cases suggest that adjuvant therapy can induce extensive or complete neuronal maturation in medulloblastoma. Additional cases must be studied to determine the prognostic significance of this rare phenomenon.

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  • 1.

    Bernier PJ, & Parent A: Bcl-2 protein as a marker of neuronal immaturity in postnatal primate brain. J Neurosci 18:24862497, 1998 Bernier PJ, Parent A: Bcl-2 protein as a marker of neuronal immaturity in postnatal primate brain. J Neurosci 18:2486–2497, 1998

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2.

    Brandis A, , Heyer R, & Hori A, et al: Cerebellar neurocytoma in an infant: an important differential diagnosis from cerebellar neuroblastoma and medulloblastoma? Neuropediatrics 28:235238, 1997 Brandis A, Heyer R, Hori A, et al: Cerebellar neurocytoma in an infant: an important differential diagnosis from cerebellar neuroblastoma and medulloblastoma? Neuropediatrics 28:235–238, 1997

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 3.

    Bruggers CS, , Fults D, & Perkins SL, et al: Coexpression of genes involved in apoptosis in central nervous system neoplasms. J Pediatr Hematol Oncol 21:1925, 1999 Bruggers CS, Fults D, Perkins SL, et al: Coexpression of genes involved in apoptosis in central nervous system neoplasms. J Pediatr Hematol Oncol 21:19–25, 1999

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 4.

    Burger PC, , Scheithauer BW, & Vogel FS: Surgical Pathology of the Nervous System and its Coverings, ed 3. New York:Churchill Livingstone, 1991, p 88 Burger PC, Scheithauer BW, Vogel FS: Surgical Pathology of the Nervous System and its Coverings, ed 3. New York: Churchill Livingstone, 1991, p 88

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5.

    De Chadarévian JP, , Montes JL, & O'Gorman AM, et al: Maturation of cerebellar neuroblastoma into ganglioneuroma with melanosis. A histologic, immunocytochemical, and ultrastructural study. Cancer 59:6976, 1987 De Chadarévian JP, Montes JL, O'Gorman AM, et al: Maturation of cerebellar neuroblastoma into ganglioneuroma with melanosis. A histologic, immunocytochemical, and ultrastructural study. Cancer 59:69–76, 1987

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6.

    Geyer JR, , Schofield D, & Berger M, et al: Differentiation of a primitive neuroectodermal tumor into a benign ganglioglioma. J Neurooncol 14:237241, 1992 Geyer JR, Schofield D, Berger M, et al: Differentiation of a primitive neuroectodermal tumor into a benign ganglioglioma. J Neurooncol 14:237–241, 1992

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7.

    Giangaspero F, , Cenacchi G, & Roncaroli F, et al: Medullocytoma (lipidized medulloblastoma). A cerebellar neoplasm of adults with favorable prognosis. Am J Surg Pathol 20:656664, 1996 Giangaspero F, Cenacchi G, Roncaroli F, et al: Medullocytoma (lipidized medulloblastoma). A cerebellar neoplasm of adults with favorable prognosis. Am J Surg Pathol 20:656–664, 1996

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 8.

    Giangaspero F, , Rigobello L, & Badiali M, et al: Large-cell medulloblastomas. A distinct variant with highly aggressive behavior. Am J Surg Pathol 16:687693, 1992 Giangaspero F, Rigobello L, Badiali M, et al: Large-cell medulloblastomas. A distinct variant with highly aggressive behavior. Am J Surg Pathol 16:687–693, 1992

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9.

    Hara A, , Hirose Y, & Yoshimi N, et al: Expression of Bax and bcl-2 proteins, regulators of programmed cell death, in human brain tumors. Neurol Res 19:623628, 1997 Hara A, Hirose Y, Yoshimi N, et al: Expression of Bax and bcl-2 proteins, regulators of programmed cell death, in human brain tumors. Neurol Res 19:623–628, 1997

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10.

    Kane W, & Aronson SM: Gangliogliomatous maturation in cerebellar medulloblastoma. Acta Neuropathol 9:273279, 1967 Kane W, Aronson SM: Gangliogliomatous maturation in cerebellar medulloblastoma. Acta Neuropathol 9:273–279, 1967

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11.

    Merry DE, , Veis DJ, & Hickey WF, et al: bcl-2 protein expression is widespread in the developing nervous system and retained in the adult PNS. Development 120:301311, 1994 Merry DE, Veis DJ, Hickey WF, et al: bcl-2 protein expression is widespread in the developing nervous system and retained in the adult PNS. Development 120:301–311, 1994

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12.

    Middleton G, , Piñón LGP, & Wyatt S, et al: Bcl-2 accelerates the maturation of early sensory neurons. J Neurosci 18:33443350, 1998 Middleton G, Piñón LGP, Wyatt S, et al: Bcl-2 accelerates the maturation of early sensory neurons. J Neurosci 18:3344–3350, 1998

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13.

    Novack DV, & Korsmeyer SJ: Bcl-2 protein expression during murine development. Am J Pathol 145:6173, 1994 Novack DV, Korsmeyer SJ: Bcl-2 protein expression during murine development. Am J Pathol 145:61–73, 1994

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14.

    Oka H, , Satoh Y, & Kawano N, et al: Expression of BCL-2 gene product in embryonal tumors of the central nervous system. Surg Neurol 45:230235, 1996 Oka H, Satoh Y, Kawano N, et al: Expression of BCL-2 gene product in embryonal tumors of the central nervous system. Surg Neurol 45:230–235, 1996

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15.

    Packer RJ, , Cogen P, & Vezina G, et al: Medulloblastoma: clinical and biologic aspects. Neurooncology 1:232250, 1999 Packer RJ, Cogen P, Vezina G, et al: Medulloblastoma: clinical and biologic aspects. Neurooncology 1:232–250, 1999

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 16.

    Paulino AC, & Melian E: Medulloblastoma and supratentorial primitive neuroectodermal tumors. An institutional experience. Cancer 86:142148, 1999 Paulino AC, Melian E: Medulloblastoma and supratentorial primitive neuroectodermal tumors. An institutional experience. Cancer 86:142–148, 1999

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17.

    Soylemezoglu F, , Soffer D, & Onol B, et al: Lipomatous medulloblastoma in adults. A distinct clinicopathological entity. Am J Surg Pathol 20:413418, 1996 Soylemezoglu F, Soffer D, Onol B, et al: Lipomatous medulloblastoma in adults. A distinct clinicopathological entity. Am J Surg Pathol 20:413–418, 1996

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18.

    Yachnis AT, , Giovanini MA, & Eskin TA, et al: Developmental patterns of BCL-2 and BCL-X polypeptide expression in the human spinal cord. Exp Neurol 150:8297, 1998 Yachnis AT, Giovanini MA, Eskin TA, et al: Developmental patterns of BCL-2 and BCL-X polypeptide expression in the human spinal cord. Exp Neurol 150:82–97, 1998

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 19.

    Yachnis AT, , Powell SZ, & Olmsted JJ, et al: Distinct neurodevelopmental patterns of Bcl-2 and Bcl-x expression are altered in glioneuronal hamartias of the human temporal lobe. J Neuropathol Exp Neurol 56:186198, 1997 Yachnis AT, Powell SZ, Olmsted JJ, et al: Distinct neurodevelopmental patterns of Bcl-2 and Bcl-x expression are altered in glioneuronal hamartias of the human temporal lobe. J Neuropathol Exp Neurol 56:186–198, 1997

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation
  • 20.

    Zhang K, , Westberg JA, & Hölttä E, et al: BCL2 regulates neural differentiation. Proc Natl Acad Sci USA 93:45044508, 1996 Zhang K, Westberg JA, Hölttä E, et al: BCL2 regulates neural differentiation. Proc Natl Acad Sci USA 93:4504–4508, 1996

    • Crossref
    • PubMed
    • Search Google Scholar
    • Export Citation

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