Angiocentric Glioma: A Clinicopathologic Review of Five Tumors with Identification of Associated Cortical Dysplasia
TB Marburger, RA Prayson. Cleveland Clinic Foundation, Cleveland, OH
Background: Angiocentric glioma is a rare, epilepsy-associated, low grade neoplasm with a characteristic perivascular growth pattern which has been recently codified in the 2007 WHO classification of CNS tumors. The purpose of this study is to describe the clinicopathologic features of five angiocentric gliomas and to evaluate for coexistent malformation of cortical development (MCD)/cortical dysplasia.
Design: Retrospective review of the clinicopathologic features of 5 angiocentric gliomas (3 females and 2 males; median age at surgery 10 yrs, range 3-19 yrs). Adjacent MCD was classified according to criteria outlined by Palmini et al.
Results: Seizures were the most common presenting symptom (n=4); one patient presented with headaches. Four of the tumors were located in the parieto-occipital, parietal, and temporal cortex and one case arose in the thalamus. All tumors consisted of an angiocentric growth pattern of bipolar spindle cells with mild pleomorphism. Three tumors also demonstrated a focal solid growth pattern. Two cases exhibited a population of cells with rounded nuclei resembling oligodendroglial cells. Evidence of adjacent MCD (focal cortical dysplasia) was observed in 3/4 cases with sufficient tissue for evaluation; all were Palmini et al Type I lesions (Type IA n=2; Type IB n=1). Calcifications (n=2) and microcystic elements (n=1) were observed in a minority of cases. Mitotic activity, necrosis, vascular proliferation, and meningeal extension were not observed. Ki-67 labeling indices were <4% in all cases. Ultrastructural evaluation (n=4) exhibited evidence of ependymal differentiation in one case. All patients are living (median follow-up 3 years postoperatively, range <1-10 years). No tumor recurrence following gross total resection (n=3) has been reported. Post-surgical follow up revealed near complete cessation of seizure symptoms (Engel Class 1) for 3/3 patients who underwent gross total resection and only mild improvement in seizure symptoms (Engel Class 4) in the single case with subtotal resection.
Conclusions: The thalamic location of one tumor represents an undescribed location for this typically superficial cortical tumor. Rare cases may exhibit a population of cells resembling oligodendroglial cells. A subset of angiocentric gliomas, similar to other low grade chronic epilepsy-related tumors of childhood, may be associated with coexistent MCD, suggesting a developmental basis to their origin.
Monday, March 22, 2010 9:30 AM
Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 221, Monday Morning