Contributed by Rebecca J. Asch-Kendrick MD, Elizabeth Burton, MD, Liam Chen, MD, PhD, David Nauen, MD, PhD
Department of Pathology, Department of Neuropathology, The Johns Hopkins Hospital, Baltimore, MD.
CLINICAL HISTORY AND IMAGING
A 74 year old man with a history of gait instability, bilateral hand numbness and hearing loss. An MRI showed a 4.4 x 2.5 x 3.4 centimeters (CC by AP by transverse) avidly enhancing heterogeneous T1 hypointense and T2 hyperintense mass with cystic components centered in the left cerebellopontine angle and widening extending through the left jugular foramen into the left carotid space. There was moderate to marked mass effect in the left middle cerebellar peduncle and the brainstem (Figs 1, 2 and 3). The patient was treated with radiation therapy and a course of dexamethasone. He returned 2 months after completing treatment with complaints of nausea, dizziness, gait imbalance and blurry vision. A repeat MRI was done showing fourth ventricle enlargement. The patient was admitted for VP shunt placement. This required multiple revisions. The patient was discharged to a rehabilitation facility. Shortly after discharge, the patient was found to be pale, diaphoretic and in respiratory distress. He progressed to asystole and expired.
At autopsy a firm, pink mass was visualized. The tumor was seen with passage through the jugular foramen and intracranial extension with compression of left cerebellum and brainstem (Figs 4, 5 and 6). Tissue sections stained with hematoxylin and eosin show spindled cells with dense chromatin (Fig 7). No mitotic figures were identified. Immunohistochemical staining of the tumor demonstrates diffuse S100 (Fig 8) and collagen IV staining (Fig 9) with a Ki-67 labeling index of 1-2%. What is your diagnosis?