Neurolymphomatosis Caused by Nasal-type Extranodal Natural Killer/T-cell Lymphoma

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

“…These biopsies were diagnostic in 6 cases and nondiagnostic in one, presumptively due to prior treatment with steroids [15]. Marrow involvement was positive in 2 cases and negative in 3; 1 case showed few atypical lymphocytes, without flow cytometric abnormalities [4]. CSF was positive in 1 case, 2 cases showed nonspecific mild increases in protein, and in our case, it demonstrated lymphocytic pleocytosis and was positive for EBV by PCR (36,700 IU/mL).…”

“…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.…”

“…Neurolymphomatosis (NL) is a condition characterized by the infiltration of cranial nerves (CN), peripheral nerves, or nerve roots by lymphocytic malignancy [1][2][3]. The associated lymphomas are usually of B lineage, most commonly diffuse large B-cell lymphoma, with only rare cases of T-or NK-cell lineage [1][2][3][4]. NL frequently presents as a painful polyneuropathy or polyradiculopathy or alternatively may manifest as a cranial neuropathy, painless polyneuropathy, or peripheral mononeuropathy [5,6].…”