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 in a Dog With B-Cell Lymphoma

“…1,3,7,8 Most human neurolymphomatosis cases are diffuse large B-cell lymphomas when classified by the Revised EuropeanAmerican Lymphoma or World Health Organization system. 3,4 Lymphoma and leukemia are well recognized in veterinary medicine, and lymphoma is one of the most common neoplasias of cats and dogs.…”

“…[1][2][3] In humans, there are 4 basic clinical presentations: painful polyneuropathy or polyradiculopathy, cranial neuropathy, painless polyneuropathy, and peripheral mononeuropathy. 1 The syndrome may occur as the initial manifestation of neoplastic disease, present concurrently with systemic neoplasia, or represent progression of disease in patients diagnosed and previously treated for lymphoma or leukemia. 1,3,7,8 Most human neurolymphomatosis cases are diffuse large B-cell lymphomas when classified by the Revised EuropeanAmerican Lymphoma or World Health Organization system.…”

Extranodal Lymphoma with Peripheral Nervous System Involvement in a Dog

“…On the other hand, involvement of peripheral nerves (neurolymphomatosis) is rarely reported in veterinary species. Neurolymphomatosis is a rare condition defined as infiltration of peripheral nerves or nerve roots by neurotropic lymphoma or leukemia [1,2,3]. In humans, there are 4 basic clinical presentations: 1) painful polyneuropathy or polyradiculopathy, 2) cranial neuropathy, 3) painless polyneuropathy and 4) peripheral mononeuropathy [1].…”

“…Neurolymphomatosis is a rare condition defined as infiltration of peripheral nerves or nerve roots by neurotropic lymphoma or leukemia [1,2,3]. In humans, there are 4 basic clinical presentations: 1) painful polyneuropathy or polyradiculopathy, 2) cranial neuropathy, 3) painless polyneuropathy and 4) peripheral mononeuropathy [1]. Most human neurolymphomatosis cases are diffuse large B-cell lymphomas when classified by the Revised European- American Lymphoma or World Health Organization (WHO) system [3, 4].…”

Neurolymphomatosis mimicking neurosarcoidosis: a case report

“…Despite the infrequent clinical presentation of neurological complications, autopsy studies indicate common involvement of the peripheral nervous system [9]. In our patient, serum LDH was consistently normal and CSF cytology was always unremarkable - but in fact, only a minority of cases has positive CSF cytology [10]. His low lymphocyte count may have represented a clue, but a bone marrow biopsy (even at the time his skin nodules had developed) was normal, as was FDG-PET scanning - again, not unusual in some instances of lymphoma [11].…”