Relationship between focal cortical dysplasia and epilepsy-associated low-grade tumors: an immunohistochemical study

Marucci G, Martinoni M, Giulioni M. Relationship between focal cortical dysplasia and epilepsy-associated low-grade tumors: an immunohistochemical study. APMIS 2013; 121: 22-9. We retrospectively analyzed 29 seizure-associated temporal lobe low-grade tumors to evaluate the utility of CD34 and bcl-2 expression in clarifying the relationship of these tumors with different classes of focal cortical dysplasia (FCD). CD34 immunostained 75% of gangliogliomas (GG) and 60% of pleomorphic xanthoastrocytomas. FCD type IIIb [i.e. abnormal cortical layering associated with a glioneuronal tumor, according to the new International League Against Epilepsy (ILAE) classification] presented CD34-immunopositive cells in 2/9 (22.2%) cases, whereas FCD type II in 6/7 (85.7%) cases, a difference statistically significant (p = 0.0117). Bcl-2 immunostained 9/12 (75%) gangliogliomas and 2/3 (66.6%) gangliocytomas. The cases of FCD type IIIb resulted negative for Bcl-2, whereas 4/7 cases (57.1%) of FCD type II showed immunopositive cells. These differences in Bcl-2 expression between FCD type IIIb and FCD type II resulted statistically significant (p = 0.0088). Abnormal cortical layering, overall, represents the kind of FCD more commonly associated with seizure-related low-grade tumors, whereas FCD type II is more frequently associated with GG. The profile of CD34 and Bcl-2 expression exhibited by GG is more similar to that observed in FCD type II. Such immunoprofile suggests the existence of a common pathogenesis linking glioneuronal tumors and FCD type II.