Neurolymphomatosis: diagnosis, management, and outcomes in patients treated with rituximab

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

“…Although the total number of reported NL cases diagnosed by FDG-PET/CT is still small, positive findings are highly suggestive of a diagnosis of NL, particularly in patients with a known history of hematologic malignancy [1,6]. Furthermore, FDG-PET/CT can often diagnose NL when other diagnostic modalities have failed [7], and can allow disease extent to be visualized and therapeutic response to be determined [8].…”

Neurolymphomatosis: A Rare Cause of Multiple Mononeuropathy

“…Peripheral nervous system (PNS) involvement is usually secondary to spread from systemic and only rarely it is primary as seen in our patient [2]. The relative incidence of NL is estimated to be about 3% of newly diagnosed NHL cases [3]. NL usually presents in four ways: 1) painful polyneuropathy or polyradiculopathy (most common; usually lumbosacral or cauda equina roots, rarely cervical or thoracic; usually subacute to chronic, rarely acute like Guillain-Barre syndrome), 2) cranial neuropathy (20%; most common facial nerve followed by abducens, oculomotor and trigeminal nerve involvement), 3) painless neuropathy (sensory before motor) and 4) peripheral mononeuropathy (usually sciatic, rarely median, radial or intercostal nerve involvement).…”

“…The clinical course is either progressive or relapsing remitting. Whatsoever the initial site, NL ultimately diffusely infiltrates peripheral neural structures as well as CSF and the substance of brain and spinal cord [3].…”

Neurolymphomatosis exhibiting repeated exacerbation and remission in both the peripheral and central nervous systems

“…Neurolymphomatosis occurs in 8.5-29% of non-Hodgkin's lymphoma cases [1]. Although some cases have been reported to have recurrence with neurolymphomatosis after the complete remission of a diffuse large B-cell lymphoma (DLBCL) with treatment [2], no case of neurolymphomatosis exhibiting repeated exacerbation and spontaneous remission in the central (CNS) as well as peripheral nervous systems (PNS) has been reported. Here, we report the first case of neurolymphomatosis that exhibited repeated exacerbation and spontaneous remission in both the PNS and CNS over the long term.…”

“…It was reported that neurolymphomatosis may develop after a complete remission of primary lesion of lymphoma with treatment of rituximab [2]. While, large B-cell neurolymphomatosis may be confined to the PNS without CNS lesions throughout clinical course [5].…”