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Annals of Clinical & Laboratory Science 39:295-302 (2009)
© 2009 Association of Clinical Scientists

Case Report

Molecular Genetic Analysis of a Primitive Neuroectodermal Tumor Arising after Intracranial Radiation and Chemotherapy for Leukemia

Mahlon D. Johnson1,5, Paul L. Moots3, Zhengping Zhuang4 and Robert J. Weil2,6
1 Departments of Pathology, 2 Neurosurgery, and 3 Neurology, Vanderbilt University Medical Center, Nashville, Tennessee; 4 Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland; 5 Department of Pathology, University of Rochester Medical Center, Rochester, New York; 6 Brain Tumor & Neuro-Oncology Center, Cleveland Clinic Foundation, Cleveland, Ohio

Address correspondence to Robert J. Weil, M.D., Brain Tumor & Neuro-Oncology Center, Cleveland Clinic Foundation, ND4-40 Lerner Research Institute, 9500 Euclid Ave, Cleveland, OH 44195, USA; tel 216 444 2007; fax 216 444 2683; e-mail weilr{at}ccf.org; or Mahlon D, Johnson, M.D., Ph.D., Dept. of Pathology & Laboratory Medicine, Univ. of Rochester Medical Center, 601 Elmwood Ave, Box 626, Rochester, NY 14623, USA; tel 585 276 3087; fax 585 273 1027; e-mail mahlon_johnson{at}urmc.rochester.edu.

Primitive neuroectodermal tumors are aggressive tumors of the central nervous system (CNS), yet their etiology remains unclear. We report a case of a primitive neuroectodermal tumor (PNET) arising in the cerebellum and pons 7 yr after intracranial radiation and chemotherapy for leukemia involving the CNS. This case suggests a possible link between radiation, chemotherapy, and the formation of these tumors, with a potential new pathogenetic role for somatic inactivation of the protooncogene RET.

Keywords: chemotherapy, primitive neuroectodermal tumor, leukemia, loss of heterozygosity, allelic imbalance, radiation-induced tumors, secondary neoplasms, microdissection, RET protooncogene




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[Abstract] [PDF]


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