Introduction
Extranodal NK/T cell nasal type lymphoma (ENKL) is an aggressive
malignancy which presents with a midline destructive lesion in the upper
respiratory tract in 60 to 90% of patients. 1-4 ENKL
is most common in Asian and Hispanic populations where it accounts for
approximately 5% of lymphoma, however it is rare in Western
populations.5,6 The characteristic features of the
disease are expression of NK cell markers (CD2, CD3, CD56), cytotoxic
molecules (granzyme B, perforin, TIA1), EBV infection of the neoplastic
cells, and angiocentric growth pattern with vascular wall
necrosis.1,7
The prognosis of ENKL is poor, with 5-year overall survival rates with
traditional anthracycline containing regimens (e.g. CHOP) ± radiotherapy
of 20-50%. 3,4 Part of the reason for the poor
prognosis is the lack of efficacy of anthracyclines due to excretion
from ENKL cells by P-glycoprotein. Chemotherapy regimens incorporating
L-asparaginase such as SMILE (steroid - dexamethasone, methotrexate,
ifosfamide, L-asparaginase, and etoposide) have shown more promise.8
Extra-nasal ENKL represents approximately one quarter of cases.4 The most common extranasal primary sites described
in the International Peripheral T-Cell Lymphoma Project were the
intestine (37%), skin (26%), testis (17%), and lung (14%).4 CNS involvement in ENKL is rare and concomitant CNS
and extranasal ENKL is particularly uncommon, with no prior reported
cases to our knowledge. Here we report a patient with concomitant CNS
and cutaneous ENKL who was refractory to multiple lines of therapy.