NK-cell lineage predicts poor survival in primary intestinal NK-cell and T-cell lymphomas

• Published in: The American journal of surgical pathology Vol. 33; no. 8; p. 1230
• Main Authors: Chuang, Shih-Sung, Chang, Sheng-Tsung, Chuang, Wen-Yu, Huang, Wan-Ting, Hsieh, Pin-Pen, Tsou, Mei-Hua, Liao, Yung-Liang, Lin, Shu-Hui, Hsieh, Yen-Chuan, Lu, Chin-Li, Sheu, Ming-Jen, Liu, Hongxiang
• Format: Journal Article
• Language: English
• Published: United States 01.08.2009
• Subjects: Adult; Aged; Aged, 80 and over; Antineoplastic Agents - therapeutic use; Biomarkers, Tumor - analysis; Combined Modality Therapy; Digestive System Surgical Procedures; Female; Herpesviridae Infections - complications; Humans; Immunohistochemistry; In Situ Hybridization; Intestinal Neoplasms - mortality; Intestinal Neoplasms - pathology; Intestinal Neoplasms - virology; Kaplan-Meier Estimate; Killer Cells, Natural - metabolism; Killer Cells, Natural - pathology; Lymphoma, T-Cell - mortality; Lymphoma, T-Cell - pathology; Lymphoma, T-Cell - virology; Male; Middle Aged; Neoplasm Staging; Prognosis
• ISSN: 1532-0979
• DOI: 10.1097/PAS.0b013e3181a95c63

Most primary intestinal natural killer (NK)-cell and T-cell lymphomas (PINKTL) in the Northern Europe are enteropathy-associated T-cell lymphomas, a complication of celiac disease, which is rare in the East. Primary intestinal NK-cell lymphoma is extremely rare and is poorly characterized. We investigated 30 cases of PINKTL from Taiwan with male: female at 2:1, median age at 55.5, 80% with jejunal/ileal involvement, 77% with perforation, 27% with multicentric tumors, and 67% at stage IE. All 7 cases tested for serum IgA anti-tissue transglutaminase were negative. Only 3 (10%) tumors showed enteropathy. Six (20%) were NK-cell lymphoma and 24 (80%) were T-cell lymphoma. The tumor cells in 21/30 (70%) cases were small to medium sized, which correlated with the coexpression of both CD8 and CD56. All tumors expressed at least 1 cytotoxic marker. All 6 NK-cell lymphomas were negative for betaF1, diffusely positive for Epstein-Barr virus-encoded mRNA (EBER), and polyclonal for T-cell receptor gene rearrangement. Five (22%) of the 24 T-cell tumors expressed betaF1, 8 (35%) of the 23 tumors were positive for EBER, and 20 (95%) of the 21 tumors were clonal for T-cell receptor. The overall 1-year survival was 36%. Univariate regression analysis showed that NK-cell lineage, multicentricity, and perforation were associated with poor prognosis. NK-cell lineage (P=0.037) was a poor prognostic factor by multivariate Cox proportional hazard regression analysis. PINKTL in Taiwan is predominantly not enteropathic with a high frequency of perforation, small to medium tumor cell size and cytotoxic phenotype. Primary intestinal NK-cell lymphoma carries a very poor prognosis, and is probably a distinct entity.