Diffuse intrinsic pontine glioma (DIPG) is an aggressive tumor of the brainstem. It almost always affects the pediatric population, with approximately 200 to 300 new annual cases in Europe and as many in the United States. Most patients survive less than two years. These patients are primarily treated with radiation therapy, as surgical resection is not a viable option for them.
The effect of radiation therapy is temporary, and no chemotherapeutic agent has been effective in treating this pathology. Several clinical trials of new agents and
novel therapeutic approaches have emerged over the past decades to improve the
outcome of patients with DIPGs, yet no real success has been obtained.
The formation of this tumor may be linked to particular cells that exist in very high concentrations during the cerebral tissue development. This explains why this tumor rarely occurs in adults and almost always occurs in mid-childhood (ages 5 to 10), a period in which the cerebral tissue is very active in development.
The model has identified the neural precursor-like cell population in the normal human ventral pons that is linked both anatomically and temporally to the incidence of DIPG. The frequency of this cell type during mid-childhood mirrors the bell-shaped incidence curve of DIPG. These cells are notably absent in the midbrain, where DIPGs are virtually nonexistent.
On the molecular level, recent advances in the biological understanding of the disease have identified the H3K27M mutation in nearly 80% of DIPGs, leading to the 2021 World Health Organization (WHO) classification of diffuse midline glioma H3K27-altered, a grade 4 tumor. Brainstem gliomas affect approximately 600 children in the world each year and are the major cause of death in children with brain tumors.
Prof. Antonio Scilimati – Professor of Medicinal Chemistry of the Department of Pharmacy-Pharmaceutical Sciences of the University of Bari Aldo Moro
Prof. Grazia Paola Nicchia – ProRector of the University of Bari Aldo Moro
Prof. Francesco Leonetti – Head of the Department of Pharmacy-Pharmaceutical Sciences of the University of Bari Aldo Moro
Prof. Maria Laura Bolognesi – President of the Italian Medicinal Chemistry Division
Dr. Teresa Perillo – Pediatric Honco-hematology, Ospedale Policlinico di Bari (Italy)
Italian DIPG Foundations – Prof. Giovanni Neri as Representative of Fondazione Onlus MiaNeri (Lazio, Italy), Fondazione GAIA (Marche, Italy), Fondazione Matibellula OdV (Piemonte, Italy) and La Bottega del sorriso (Puglia, Italy), Associazione Il Coraggio dei Bambini (Campania, Italy), HEAL Fondazione (Lazio, Italy)
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