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Natural history of a medulloblastoma: 30months of wait and see in a child with a cerebellar incidentaloma

Zeilhofer, Ulrike ; Scheer, Ianina ; Warmuth-Metz, Monika ; Rushing, Elisabeth ; Pietsch, Torsten ; Boltshauser, Eugen ; Grotzer, Michael ; Gerber, Nicolas

In: Child's Nervous System, 2013, vol. 29, no. 7, p. 1207-1210

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    Title
    • Natural history of a medulloblastoma: 30months of wait and see in a child with a cerebellar incidentaloma
    Author
    • Zeilhofer, Ulrike. Department of Oncology, University Children's Hospital, 8032, Zurich, Switzerland
    • Scheer, Ianina. Department of Diagnostic Imaging, University Children's Hospital, 8032, Zurich, Switzerland
    • Warmuth-Metz, Monika. Department of Neuroradiology, University of Wuerzburg, 97080, Wuerzburg, Germany
    • Rushing, Elisabeth. Institute of Neuropathology, University Hospital Zurich, 8091, Zurich, Switzerland
    • Pietsch, Torsten. Institute of Neuropathology, University of Bonn, 53105, Bonn, Germany
    • Boltshauser, Eugen. Department of Neurology, University Children's Hospital, 8032, Zurich, Switzerland
    • Grotzer, Michael. Department of Oncology, University Children's Hospital, 8032, Zurich, Switzerland
    • Gerber, Nicolas. Department of Oncology, University Children's Hospital, 8032, Zurich, Switzerland
    Document Type
    • Postprint
    OAI-PMH Identifier
    • oai:doc.rero.ch:318224
    Summary
    Introduction: With the increasing use of neuroimaging studies, the discovery of incidental neoplastic lesions is becoming more frequent. However, standard procedures are lacking, and little is known about their optimal management. Case Report: We here present the case of a boy with a cerebellar mass incidentally discovered on a CT scan performed after head trauma. In another scan performed after another incident of head trauma 14months earlier, the lesion could be seen after retrospective examination. In view of the asymptomatic clinical and stable radiological status and the presumed diagnosis of a low-grade glioma, a watch-and-wait strategy was elected. After clinical and radiological progression was observed, the tumour was resected, 2½ years after the initial imaging study. Histological evaluation revealed a WNT pathway-activated classical medulloblastoma. Discussion: To our knowledge, this is the first description of such a long natural history and pre-symptomatic period of a medulloblastoma. The long period of stability followed by a period of accelerated tumour growth is compatible with increasing biological aggressiveness, possibly related to the stepwise accumulation of genetic changes