Dennis Lee1,2, Robert A Riestenberg1,3, Aden Haskell-Mendoza1,4, Orin Bloch5. 1. Department of Neurological Surgery, University of California Davis, Sacramento, CA, USA. 2. Chicago Medical School, Rosalind Franklin University of Medicine and Science, North Chicago, IL, USA. 3. Northwestern University, Feinberg School of Medicine, Chicago, IL, USA. 4. Duke University School of Medicine, Durham, NC, USA. 5. Department of Neurological Surgery, University of California Davis, Sacramento, CA, USA. obloch@ucdavis.edu.
Abstract
OBJECTIVE: In 2018, cIMPACT-NOW update 3 concluded that WHO grade II/III IDH-wildtype diffuse astrocytomas that contain TERT promoter mutations, chromosome 7 gain/10 loss, and/or EGFR amplification, correspond to a WHO grade IV diagnosis and should be classified as Diffuse astrocytic glioma, IDH-wildtype, with molecular features of glioblastoma, WHO grade IV (DAG-G). We present a single-institution series of patients with DAG-G and IDH-mutant astrocytomas and compare their clinical, molecular, and radiographic characteristics. METHODS: Patient data was retrospectively extracted from the EMR for all patients undergoing surgical biopsy/resection of a diffuse astrocytoma at our institution from 2018 to 2020. Clinical presentation, molecular alterations, radiographic appearance, surgery, and survival were reviewed for each patient. RESULTS: Six DAG-G patients were identified in our cohort. All patients had diffuse disease, and presented with expansile, T2 hyperintense lesions with minimal enhancement. Compared to patients with classic IDH-mutant astrocytomas, mean age for DAG-G patients was older (68 vs 33 years, p < 0.0001), tumors were more diffuse (p = 0.02), with patients more likely to present with focal deficits and receive a biopsy only (p = 0.005). Overall survival was significantly shorter for DAG-G patients (p = 0.03). CONCLUSION: Patients with DAG-G are more likely to be older than typical IDH-mutant diffuse astrocytoma patients. They are more likely to present with tumors in a diffuse pattern with focal deficits. When such patients are encountered, prompt biopsy/resection to confirm the diagnosis and immediate initiation of adjuvant therapy is recommended, as the disease progression and overall prognosis is similar to glioblastoma.
OBJECTIVE: In 2018, cIMPACT-NOW update 3 concluded that WHO grade II/III IDH-wildtype diffuse astrocytomas that contain TERT promoter mutations, chromosome 7 gain/10 loss, and/or EGFR amplification, correspond to a WHO grade IV diagnosis and should be classified as Diffuse astrocytic glioma, IDH-wildtype, with molecular features of glioblastoma, WHO grade IV (DAG-G). We present a single-institution series of patients with DAG-G and IDH-mutant astrocytomas and compare their clinical, molecular, and radiographic characteristics. METHODS:Patient data was retrospectively extracted from the EMR for all patients undergoing surgical biopsy/resection of a diffuse astrocytoma at our institution from 2018 to 2020. Clinical presentation, molecular alterations, radiographic appearance, surgery, and survival were reviewed for each patient. RESULTS: Six DAG-G patients were identified in our cohort. All patients had diffuse disease, and presented with expansile, T2 hyperintense lesions with minimal enhancement. Compared to patients with classic IDH-mutant astrocytomas, mean age for DAG-G patients was older (68 vs 33 years, p < 0.0001), tumors were more diffuse (p = 0.02), with patients more likely to present with focal deficits and receive a biopsy only (p = 0.005). Overall survival was significantly shorter for DAG-G patients (p = 0.03). CONCLUSION:Patients with DAG-G are more likely to be older than typical IDH-mutant diffuse astrocytomapatients. They are more likely to present with tumors in a diffuse pattern with focal deficits. When such patients are encountered, prompt biopsy/resection to confirm the diagnosis and immediate initiation of adjuvant therapy is recommended, as the disease progression and overall prognosis is similar to glioblastoma.
Authors: Andres Ramos-Fresnedo; Michael W Pullen; Carlos Perez-Vega; Ricardo A Domingo; Oluwaseun O Akinduro; Joao P Almeida; Paola Suarez-Meade; Lina Marenco-Hillembrand; Mark E Jentoft; Bernard R Bendok; Daniel M Trifiletti; Kaisorn L Chaichana; Alyx B Porter; Alfredo Quiñones-Hinojosa; Terence C Burns; Sani H Kizilbash; Erik H Middlebrooks; Wendy J Sherman Journal: J Neurooncol Date: 2022-02-17 Impact factor: 4.506