Pietro Fiaschi1, Filippo Badaloni2, Bernarda Cagetti2, Luca Bruzzone2, Gianluca Marucci3, Anna Dellachà4, Marco Pavanello5, Giuseppe Ganci6, Riccardo Padolecchia6, Valtero Valsania2. 1. Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health, University of Genoa, Genoa, Italy. Electronic address: pietro.fiaschi@alice.it. 2. Department of Neurosurgery, Santa Corona Hospital, Pietra Ligure, Italy. 3. Neuropathology Unit, IRCCS Foundation Carlo Besta Neurological Institute, Milan, Italy. 4. Department of Pathology, Ospedale Santa Corona, Pietra Ligure, Italy. 5. Department of Neurosurgery, Istituto Giannina Gaslini, Genova, Italy. 6. Department of Neuroradiology, Ospedale Santa Corona, Pietra Ligure, Italy.
Abstract
BACKGROUND: Diffuse leptomeningeal glioneuronal tumor (DLGNT) was recently added to the World Health Organization classification of central nervous system tumors. DLGNT is a rare entity that occurs more commonly in pediatric patients, but occasional cases have been reported in adults. This tumor has been recognized as a distinct pathologic entity; however, its biologic behavior remains unclear. It is considered an indolent neoplasm, although considerable morbidity has been reported. For this reason, further characterization and collection of evidence are crucial. METHODS: In this article, we reported a case of a 36-year-old woman with a DLGNT characterized by rapid, aggressive behavior. We also performed a review of the literature for reported cases of low-grade and high-grade forms involving adults and children. RESULTS: DLGNTs should no longer be considered only as low-grade tumors affecting pediatric patients. The spectrum of presentations also includes aggressive tumors affecting adults. CONCLUSIONS: Further clinical and pathologic data supported by cytogenetic and molecular investigations are mandatory to better characterize DLGNTs.
BACKGROUND: Diffuse leptomeningeal glioneuronal tumor (DLGNT) was recently added to the World Health Organization classification of central nervous system tumors. DLGNT is a rare entity that occurs more commonly in pediatric patients, but occasional cases have been reported in adults. This tumor has been recognized as a distinct pathologic entity; however, its biologic behavior remains unclear. It is considered an indolent neoplasm, although considerable morbidity has been reported. For this reason, further characterization and collection of evidence are crucial. METHODS: In this article, we reported a case of a 36-year-old woman with a DLGNT characterized by rapid, aggressive behavior. We also performed a review of the literature for reported cases of low-grade and high-grade forms involving adults and children. RESULTS: DLGNTs should no longer be considered only as low-grade tumors affecting pediatric patients. The spectrum of presentations also includes aggressive tumors affecting adults. CONCLUSIONS: Further clinical and pathologic data supported by cytogenetic and molecular investigations are mandatory to better characterize DLGNTs.
Authors: Karol Wiśniewski; Michael G Brandel; David D Gonda; John R Crawford; Michael L Levy Journal: Childs Nerv Syst Date: 2022-07-22 Impact factor: 1.532