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.