A 61 year old white woman with a history of type 2 diabetes, hypertension, Hashimoto’s associated hypothyroidism and diverticulosis presented with a right buttock mass. Excisional biopsy showed extranodal NK/T-cell lymphoma (CD56+, negative for CD3, CD4, CD5, CD8, CD30, ALK-1, CD34, CD117, myeloperoxidase and TdT). EBV latent membrane protein (LMP) immunostain was negative. This was consistent with extranodal NK/T cell lymphoma, nasal type. Staging with CT and PET scans and bone marrow biopsy revealed only the left gluteal mass, consistent with stage IAE extranasal NK/T cell lymphoma. She was asymptomatic, specifically with no complaints referable to nasal passages or aerodigestive tract. Physical exam was remarkable only for well healed left buttock biopsy site. The patient received 3 cycles of CHOP followed by radiation to the left buttock. However, a week after completion of radiation, nodular lesions arose on the buttocks and lower left extremity, and right buttock, all outside the original radiation field. Biopsy of the leg showed recurrent NK/T lymphoma in the dermis and subcutis. During local radiation to the right buttock she developed sinus congestion. CT scan of the maxillofacial region showed soft tissue thickening of the nasal fossa. Biopsy of the nasal mass revealed diffuse, predominantly small cell NK/T-cell lymphoma, CD3+, CD56+ and CD5−. Flow cytometry showed cytoplasmic CD3 dim, CD56 dim, CD25+, and CD16+. EBV early RNA (EBER) was positive by in situ hybridization (ISH). Radiation to the right nasal fossa was initiated, along with prednisone. Treatment with denileukin diftitox was discontinued secondary to capillary leak syndrome with pulmonary edema, with no response. She then developed multiple progressive ulcerated, raised erythematous circumferential lesions on both lower extremities from below the knee to the ankle. CT staging did not show any other disease. Pegaspargase, a pegylated form of L asparaginase, 2500mg/m2 IV every 2 weeks led to a complete response (CR) after 4 doses. Treatment was discontinued after an anaphylactic reaction during the fifth dose. The response lasted 6 months from the end of therapy, at which time cutaneous, but not nasal, relapse occurred. After local radiation, she then proceeded to autologous stem cell transplant with a cyclophosphamide/TBI preparative regimen, but presented 7 months later with leucopenia. Bone marrow biopsy for the first time showed involvement by NK/T lymphoma. She died of progressive disease.
A 47 year old Chinese man noted fullness in the left nasal cavity and difficulty breathing on that side. CT scan revealed a soft tissue opacity filling the left maxillary frontal and ethmoid sinuses. MRI was confirmatory, with no intracranial disease. Biopsy revealed a diffuse lymphohistiocytic infiltrate with large areas of necrosis. Immunophenotyping revealed CD56+, CD3+, CD2+, CD8+, TIA-1+, granzyme+, EBER (by ISH) +, CD30+ (focal), consistent with extranodal NK/T cell lymphoma, nasal type. He denied fevers, chills or night sweats, but had 7–8 pound weight loss over 2 months. His physical exam was remarkable only for erythema and swelling in the inferior turbinate. Bone marrow biopsy and CT scan showed no other evidence of lymphoma. He was diagnosed with Stage IE extranodal NK/T lymphoma, nasal type. He received involved field radiation to 5040 cGy, followed by CHOP for 4 cycles. Two months later, CT scan revealed 4 liver lesions, the largest 2.5 × 2.5 cm. Liver biopsy confirmed NK/T cell lymphoma nasal type, CD56 positive, CD3+, TIA-1+. Pegaspargase was initiated at 2500 units/m2 IV every 21 days, and then reduced to 2000units/m2 secondary to elevated transaminases. CT scan after 7 doses of pegaspargase showed complete resolution of liver lesions. Treatment was stopped after 11 total doses. He is currently being observed in ongoing complete remission assessed by CT scan.