ANGIOCENTRIC T-CELL LYMPHOMA PRESENTING WITH MULTIPLE CRANIAL NERVE PALSIES AND RETROBULBAR OPTIC NEUROPATHY

Angiocentric T-cell lymphoma (lymphomatoid granulomatosis) may present with prominent central nervous system (CNS) findings with variable ra diographic features. We describe a patient who presented with multiple cranial nerve palsies involving the left optic nerve, left facial ner ve, left ocular motor nerves, and bilateral acoustic nerves. Enhanceme nt of the right temporal meninges and a cavernous sinus mass were note d on magnetic resonance (MR) scan. A right temporal craniectomy and me ningeal biopsy were performed. Meningeal biopsy revealed atypical angi ocentric granulomatous lymphoid infiltrates without associated necrosi s, giant cells, or granuloma formation. Morphologic and T-cell recepto r gene rearrangement findings were diagnostic of an angiocentric T-cel l lymphoma. Retrobulbar optic neuropathy and multiple cranial nerve pa lsies may be the presenting features of angiocentric T-cell lymphoma. The neurologic and unique radiographic changes in our case expand the previously reported findings in CNS angiocentric T-cell lymphoma.