CD8+, CD56+ (natural killer-like) T-cell lymphoma involving the small intestine with no evidence of enteropathy: Clinicopathology and molecular study of five Japanese patients

• Published in: Pathology international 2008-10, Vol.58 (10), p.626-634
• Main Authors: Akiyama, Takashi, Okino, Takeshi, Konishi, Hiroshi, Wani, Yoji, Notohara, Kenji, Tsukayama, Choutatsu, Tsunoda, Tsukasa, Tasaka, Taizo, Masaki, Yuji, Sugihara, Takashi, Sadahira, Yoshito
• Format: Article
• Language: English
• Subjects: Adult; Aged; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biomarkers, Tumor - analysis; CD56; CD56 Antigen - immunology; CD8-Positive T-Lymphocytes - immunology; Clone Cells; Combined Modality Therapy; Cyclophosphamide - therapeutic use; cytotoxic; Doxorubicin - therapeutic use; Female; Gene Deletion; Gene Rearrangement, T-Lymphocyte - genetics; Humans; Immunoenzyme Techniques; In Situ Hybridization; Intestinal Neoplasms - immunology; Intestinal Neoplasms - mortality; Intestinal Neoplasms - pathology; Intestinal Neoplasms - therapy; intestine; Intestine, Small - pathology; Killer Cells, Natural - immunology; Killer Cells, Natural - pathology; lymphoma; Lymphoma, T-Cell - immunology; Lymphoma, T-Cell - mortality; Lymphoma, T-Cell - pathology; Lymphoma, T-Cell - therapy; Male; Middle Aged; Neoplasm Staging; Prednisolone - therapeutic use; RNA, Viral - analysis; Survival Rate; T cell; Vincristine - therapeutic use
• ISSN: 1320-5463; 1440-1827
• DOI: 10.1111/j.1440-1827.2008.02281.x

The present study reports five CD8+, CD56+ (natural killer (NK)‐like) T‐cell lymphomas involving the small intestine without evidence of enteropathy, from Japan. Three were intestinal T‐cell lymphoma. The site of origin of the other two was not definitive. Four of five patients underwent emergency operation because of intestinal perforation. The small intestines of these patients had multiple ulcerative lesions with or without demarcated tumors. Histologically, the lymphoma cells were monomorphic or slightly pleomorphic and displayed epitheliotropism of varying degrees. Lymphoma cells of all patients shared the common phenotype: CD3+, CD4−, CD5−, CD8+, CD56+, CD57−, T‐cell intracellular antigen‐1+, granzyme B+. In contrast to nasal/nasal type NK‐cell lymphomas, they had clonal rearrangement of T‐cell receptor(TCR) genes and were negative for EBV‐encoded RNA. Immunohistochemistry and genetics suggested that three cases were of αβT‐cell origin and two cases were of γδT‐cell origin. There was no evidence of enteropathy in any patient. The cases followed a clinically aggressive course with a frequent involvement of lung. According to the classification based on the recent genetic studies of European enteropathy‐type intestinal T‐cell lymphoma (ETL), the present cases could be classified as type 2 ETL.