Neurolymphomatosis: A Case Series of Clinical Manifestations, Treatments, and Outcomes (P04.187)

Abstract

Objective: Our goal was to ascertain common factors to render earlier diagnosis, and prognostic factors to predict response to therapy. Background Neurolymphomatosis involves infiltration of malignant lymphocytes into the peripheral nervous system, specifically cranial nerves, roots, plexuses, and/or peripheral nerves. It is distinct from more common neurological complications related to chemotherapy, irradiation, paraneoplastic disorders, and leptomeningeal carcinomatosis. Design/Methods: We review five patients who developed neurolymphomatosis at M.D. Anderson Cancer Center between 2010 and 2011. Clinical, radiologic, and laboratory data were extracted from their medical records. Results: The average age at onset was 48 (range 26 - 76) with primary diagnosis being non-Hodgkin9s lymphoma in three (Burkitt9s, large B-cell) and leukemia in two (AML, CML). Initial symptoms included weakness (5/5), pain (3/5), and sensory change (2/5) and localized to lower extremities (3/5), upper extremities (2/5), unilateral (4/5), bilateral (1/5), and cranial nerve (1/5). Physical exam findings included weakness (5/5), sensory disturbance (5/5), atrophy (4/5), hypotonia (2/5), and hyporeflexia (5/5). Three patients demonstrated MRI abnormalities with clinical correlation, affecting neural foramina (1/5), paraspinal (2/5), or subcutaneous (1/5) regions; no patients had imaging evidence of parenchymal, spinal cord, or leptomeningeal pathology. All patients had positive EMG/NCS and diagnoses included mononeuritis multiplex, multifocal plexopathy, demyelinating polyneuropathy, and chronic axonal polyradiculoneuropathy. One patient had positive CSF cytology; PET was performed and positive in four patients and no nerve biopsies or autopsies were performed. Therapies included systemic (5/5) and intrathecal chemotherapy (4/5), immunomodulators (2/5), and plasma exchange (1/5); no patients underwent radiotherapy. Mortality was 40% with average survival from neurologic presentation 6 months (range 1–12 months); the other patients progressed despite treatment. Neither age, gender, malignancy type, recurrence, positive CSF/imaging/EMG, nor intervention correlated with worse prognosis. Conclusions: Further studies are warranted to define risk for developing neurolymphomatosis and identify factors affecting outcome. Disclosure: Dr. Gildersleeve has nothing to disclose. Dr. Hormozdi has nothing to disclose. Dr. Kamiya Matsuoka has nothing to disclose. Dr. Woodman has nothing to disclose.