Neurolymphomatosis

Abstract

The term "neurolymphomatosis" (NL) has included infiltration of the peripheral nervous system by lymphoma and nontumor lymphocytes. We describe NL as a lymphoma entity that affects cranial and peripheral nerves and roots. We reviewed the medical records of patients at the Massachusetts General Hospital (MGH) who registered between 1972 and 2000, as well as cases published in the English-language literature. Inclusion criteria were (A) histopathologic demonstration of lymphoma within peripheral nerve, nerve root/plexus, or cranial nerve or (B) CT/MRI or intraoperative evidence of nerve enlargement and/or enhancement beyond the dural sleeve in the setting of prior or concurrent lymphoma in systemic or CNS sites. We identified 25 patients with NL in addition to 47 reported by others. Four clinical presentations were (1) painful involvement of nerves or roots, (2) cranial neuropathy with or without pain, (3) painless involvement of peripheral nerves, (4) painful or painless involvement of a single peripheral nerve. Twenty of our patients and 44 of those reported had histopathologic confirmation of lymphoma infiltrating root or nerve. In the remainder, diagnosis was based upon clinical presentation, nodular nerve enlargement or enhancement, and lymphoma cells in spinal fluid or extraneural sites. For antemortem diagnosis, imaging studies were of greatest utility, followed by biopsy. Thirty-three patients of the combined series were not correctly diagnosed until postmortem examination. Systemic chemotherapy was used to address the multiple potential sites of involvement. When properly treated, NL carries a prognosis similar to primary CNS lymphoma in the modern era.

Neurolymphomatosis on F-18 FDG PET/CT and MRI Findings: A Case Report

“…Treatment with intravenous high-dose methotrexate was initiated, but the Discussion NL is a rare but well-described entity, in which malignant lymphocytes infiltrate the peripheral nervous system (PNS) [4]. Although NL would appear to be the least common neurologic manifestation of lymphoma, Baehring et al [2] estimated that NL represents 10% of primary lymphomas of the nervous system (0.2% of all NHLs). Furthermore, between 8.5 and 29% of NHLs metastasize to the nervous system, and an estimated 10% of these involve the PNS [2].…”

“…Although NL would appear to be the least common neurologic manifestation of lymphoma, Baehring et al [2] estimated that NL represents 10% of primary lymphomas of the nervous system (0.2% of all NHLs). Furthermore, between 8.5 and 29% of NHLs metastasize to the nervous system, and an estimated 10% of these involve the PNS [2].…”

“…The typical manifestations of NL are progressive sensorimotor peripheral neuropathy, plexopathy, or cranial neuropathy. The diagnosis of NL is very difficult because these symptoms are also manifested by other diseases, such as mononeuropathy, polyradiculopathies, cauda equina syndrome, Guillain-Barre syndrome, and chronic inflammatory demyelinating polyneuroradiculopathy [2]. The classic "gold standard" used to confirm NL is a nerve biopsy, but recent imaging modality developments have improved resolutions to the extent that affected neural structures can be detected.…”

High frequency of neurolymphomatosis as a relapse disease of intravascular large B‐cell lymphoma

“…Neurolymphomatosis (NL) also is a rare manifestation of non-Hodgkin lymphoma in which lymphoma cells infiltrate the peripheral nerves. 12,13 NL is characterized clinically by a painful polyneuropathy or polyradiculopathy, cranial neuropathy, painless polyneuropathy, and peripheral mononeuropathy. Unfortunately, the diagnosis of NL often is difficult and requires histologic confirmation of the affected nerve, which may not be feasible and often is nondiagnostic.…”

“…Unfortunately, the diagnosis of NL often is difficult and requires histologic confirmation of the affected nerve, which may not be feasible and often is nondiagnostic. 12,14 Enhanced magnetic resonance imaging (MRI) is useful in diagnosing NL before histopathologic confirmation by revealing the enlargement and enhancement of the affected nerve, although it may not always provide optimal visualization of lymphomatous involvement. 12,15 Several recent studies have demonstrated the advantages of FDG-PET/CT in the diagnosis of NL.…”