Endodermal cyst in the cerebellopontine angle with immunohistochemical reactivity for CA19-9

We present a rare case of the endodermal cyst in the cerebellopontine angle with positive immunohistochemical reactivity for CA19-9. The patient was a 67-year-old woman with an endodermal cyst in the cerebellopontine angle who presented with progressive gait disturbance. A magnetic resonance image showed a cerebellopontine angle which was low in signal on T1-weighted, and high on T2-weighted image without gadolinium enhancement. The preoperative diagnosis was an epidermoid or arachnoid cyst. The patient underwent surgery. Since the intraoperative histological diagnosis by frozen section was endodermal cyst, total removal of the cyst wall was achieved for preventing recurrence. The postoperative definitive histological diagnosis was endodermal cyst, type A by Wilkins-Odom classification, lined by mucinous epithelium with an immunohistochemical reactivity for CA 19-9 protein and cytokeratin. This is the first reported case of a huge endodermal cyst in the cerebellopontine angle of an elderly woman which exclusively showed a differentiation toward the midgut epithelium. The histological diagnosis during surgery by frozen section should be performed for proper treatment.