Peripheral Nerve Lymphomatosis

Abstract

Lymphoma involvement of peripheral nerves is rare and it may mimic benign neurogenic tumors or neuropraxic injury. This study presents three patterns of presentations in four patients with neurolymphomatous involvement of their peripheral nerves. We reviewed the clinical records of four patients who underwent exploratory brachial plexus surgery (n = 1), pronator tunnel decompression (n = 1) and peripheral nerve exploration (n = 2) and subsequently found to have neurolymphomatosis (NL). Histological diagnoses were diffuse large B-cell lymphoma (n = 3) and NK/T-cell lymphoma (n = 1). NL lacks pathognomonic clinical and imaging features that aid clinicians in diagnosis. Apart from a history of lymphoma, and high clinical index of suspicion, PET-CT scans appear to be a helpful adjunct in detecting high metabolic lesions occuring in situ or systemically. Intra-operative frozen section is helpful to detect round blue cells, before final cytological diagnosis.

Primary Extranodal NK/T-Cell Lymphoma Presenting as Neurolymphomatosis Involving Multiple Cranial Nerves: A Case Report

“…Our patient is currently in unconfirmed complete remission, at over 2 years after diagnosis and required 2 lines of salvage chemotherapy and aggressive craniospinal radiation to the entire neuro axis after an early (60 days) leptomeningeal relapse. This prolonged survival is atypical, as underscored by the other reported cases: 5 patients died of their disease within 1 year of NL diagnosis, one died of disease with time interval unspecified, and 3 were alive at the time of publication; however, one had limited disease with isolated involvement of a single peripheral nerve; and for the two, time interval is unspecified [4,[13][14][15][17][18][19][20][21].…”

“…English language literature review (PubMed search; keywords: NK/T-cell Lymphoma AND Neurolymphomatosis) reveals 4 cases of NK/T-lineage-related NL, with 5 additional cases identified as cited literature (shown in Table 1) [4,[13][14][15][17][18][19][20][21]. NL was restricted to periph- eral nerves in 6 cases; one showed involvement of multiple peripheral nerves and unilateral involvement of the right third facial nerve; one showed right peroneal nerve involvement, with subsequent optic nerve involvement; and one presented with isolated involvement of the cauda equina.…”

“…DOI: 10.1159/000518797 Although Epstein-Barr virus (EBV) infection is typically restricted to B lymphocytes, the virus is invariably present in ENKTL [12]. Review of the English language literature reveals 10 cases of NL associated with NK/T-lineage lymphoma, including our case: 6 as initial manifestation of disease and 4 in the context of disease progression or relapse [4,[13][14][15][16][17][18][19][20][21]. Of these cases, ours is the only one with multiple nerve involvement and documented prolonged survival [4,[13][14][15][16][17][18][19][20][21].…”

“…Review of the English language literature reveals 10 cases of NL associated with NK/T-lineage lymphoma, including our case: 6 as initial manifestation of disease and 4 in the context of disease progression or relapse [4,[13][14][15][16][17][18][19][20][21]. Of these cases, ours is the only one with multiple nerve involvement and documented prolonged survival [4,[13][14][15][16][17][18][19][20][21]. To the best of our knowledge, this is the first case of ENKTL presenting with exclusive involvement of CN and cauda equina nerve roots.…”

Tumors of the Brachial Plexus

“…One example is the Pancoast tumor, which was first described by Hare in 1838 as “a tumor affecting certain nerves.” 4 , 5 The last etiology is metastatic—though this is rare, these tumors can arise from almost any primary tumor site. A variant of metastatic tumors, neurolymphomatosis, where the peripheral nerves are involved in patients with advanced lymphoma, can also arise within the brachial plexus 6 .…”