Contributed by Jesse Lee Kresak, MD, Kyle M Fargen MD, Anthony T Yachnis, MD, Spiros Blackburn, MD
Departments of Neuropathology and Neurosurgery, University of Florida, Gainesville, Florida
A 59 year old female with a history of hypertension and depression presented to the Emergency Department with a one week history of nausea, bloody vomiting, and vertigo. She was not taking any anticoagulants and had no history of bleeding disorders. She was noted to have coffee ground emesis, but after having resolution of her nausea with antiemetic medication, opted to return the following morning for a scheduled upper gastrointestinal endoscopy. That evening while at home she developed altered mental status with sudden neurologic decline and EMS was notified. She was intubated on the scene and upon arrival to the Emergency Department, was following commands in all four extremities but demonstrated anisocoria with ophthalmoplegia and left facial weakness.
Admission laboratory and clotting studies were unremarkable. Non-contrast CT of the brain demonstrated acute hemorrhage within the left cerebellopontine angle (CPA), cerebellum, and 4th ventricle, with fullness involving the region of the internal acoustic meatus (IAM) (Figure 1). CT angiography demonstrated normal intracranial vasculature without evidence of aneurysm, arteriovenous malformation or fistula. She underwent emergent placement of a right frontal ventriculostomy for cerebrospinal fluid diversion.
MRI of the brain was obtained. This study demonstrated a 2.2 x 2.2 x 2 cm left CPA mass with extension into the IAM with surrounding hemorrhage (Figure 2). In total, the lesion with surrounding hemorrhage was approximately 3.5 x 3.7 x 3.4 cm in size. The working diagnosis based on the intracanalicular tumor extension was hemorrhagic vestibular schwannoma. Due to the overall size of the lesion and the associated hemorrhage, urgent surgical intervention was undertaken.
She was placed in the three-quarter prone position and underwent left retrosigmoid craniotomy. The CPA hematoma was evacuated, revealing a soft, hemorrhagic and easily suckable CPA mass near the IAM. Subtotal resection of the mass was performed. The patient emerged from surgery in good condition but required tracheostomy due to lower cranial neuropathies. After a full discussion with the patient and her family regarding her diagnosis, the patient elected to be discharged to home for hospice care on hospital day 9.
This neoplasm was composed of highly pleomorphic and mitotically active spindle cells. Focal individual cells had hypereosinophilic cytoplasm. (Figure 3). The tumor was focally immunoreactive for S-100 protein (Figure 4) as well as desmin (Figure 5) and myogenin (Figure 6), while being negative for EMA, cytokeratin (AE1/3), and neurofilament protein. What is your diagnosis?