Morphologic

characterization and identification of cell surface proteins in DIPG

Clinicopathologic evaluation of gastrointestinal manifestations of rare diseases

Martha Quezado, MD

DIPG Evaluation
Pediatric malignant glioma is an understudied disease, and appears to be biologically distinct from adult counterparts. DIPGs carry a very poor prognosis due to their impossible surgical resection and minimal to no response to chemoradiation. We are studying these heterogenous tumors with the purpose of 1. define their morphologic spectrum and 2. identify cell surface targets using genomic and expression analyses (proteomics and immunohistochemistry)
Autopsy material received at NIH from all over the country as per our protocol; normal and tumor areas selected for potential cell line development (JHU) and proteomics (CH). Whole mount cross sections are generated. HE evaluation/ diagnosis (report to families if requested) We select areas for CGH and apply IHC (available; as identified by proteomics evaluation)

Papers

Genomic aberrations in pediatric diffuse intrinsic pontine gliomas. Warren KE, Killian K, Suuriniemi M, Wang Y, Quezado M, Meltzer PS. Neuro Oncol. 2012 Mar;14(3):326-32.

Research Implications
In order to make this research viable, NIH, JHU, and Childrens hospital created the Mid-Atlantic DIPG consortium
Laboratory of Pathology crucial to receive/collect tissues for this research project The collaboration with Dr. Miettinen, via the Immunohistochemistry Developmental Laboratory is an essential part in this endeavor Our evaluation is necessary for identification of potential therapeutic targets

Future Direction
We expect to identify cell surface antigens expressed on DIPGs and additional pediatric tumors and therefore be able to identify potential targets for treatment. Challenges: Lack of material and develop interactions with additional researchers

Collaborators
Miettinen M Wang Z Warren K JHU Childrens Hospital, Washington DC