Primary CD56 positive lymphomas of the gastrointestinal tract

• Published in: Cancer Vol. 91; no. 3; pp. 525 - 533
• Main Authors: Chim, Chor S., Au, Wing Y., Shek, Tony W. H., Ho, Judy, Choy, Carolyn, Ma, Shiu K., Tung, Hiu M., Liang, Raymond, Kwong, Yok L.
• Format: Journal Article
• Language: English
• Published: New York John Wiley & Sons, Inc 01.02.2001; Wiley-Liss
• Subjects: Adult; Aged; Biological and medical sciences; CD56 Antigen - analysis; Female; Hematologic and hematopoietic diseases; Humans; Intestinal Neoplasms - immunology; Intestinal Neoplasms - pathology; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; literature review; Lymphoma, T-Cell - immunology; Lymphoma, T-Cell - pathology; Male; Medical sciences; Middle Aged; natural killer cell lymphoma; natural killer‐like T‐cell lymphoma; primary gastrointestinal lymphoma; Human; Immunophenotype; Biological marker; Male; Malignant hemopathy; Gastrointestinal; Non Hodgkin lymphoma; Natural killer cell; Case study; Lymphoproliferative syndrome; T-Lymphocyte; Digestive diseases; Intestinal disease; Adult; Female; Elderly; Bibliographic review; Gastric disease
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/1097-0142(20010201)91:3<525::AID-CNCR1030>3.0.CO;2-U

BACKGROUND Primary CD56 positive lymphoma of the gastrointestinal (GI) tract is rare. Genotypically, these tumors can be classified into natural killer (NK)‐like T‐cell lymphoma or NK cell lymphoma by the presence or absence of T‐cell receptor (TCR) gene rearrangement. However, they have a considerable degree of morphologic and immunophenotypic overlap, making a definitive diagnosis difficult. METHODS The clinicopathologic features of three patients with primary CD56 positive lymphoma of the small and large bowel are presented. This is followed by a review of the English literature from 1966 to the present. RESULTS All patients had CD56 posistive/CD3ϵ positive disease on paraffin section. Two patients were positive for Epstein–Barr virus‐encoded early nuclear RNAs (EBER) according to in situ histochemistry results and were negative for TCR gene rearrangement, consistent with primary NK lymphoma of the GI tract. The other patient was EBER negative with rearranged TCR, consistent with NK‐like T‐cell lymphoma. There was no clinical or histologic evidence of enteropathy in any of the patients. The major presenting symptoms included fever, weight loss, and intestinal perforation. All patients died between 1 week and 6 months after diagnosis despite undergoing surgery and intensive chemotherapy. CONCLUSIONS These results, together with a literature review, suggest that primary NK cell lymphoma of the GI tract may be considered a distinct clinicopathologic entity. Both primary NK and NK‐like T‐cell lymphoma pursue an aggressive clinical course. EBER and TCR gene rearrangement are useful in distinguishing NK cell lymphoma from NK‐like T‐cell lymphoma, particularly when frozen tissue is not available for immunophenotyping. Cancer 2001;91:525–33. © 2001 American Cancer Society. Primary CD56 positive lymphoma of the gastrointestinal tract is an aggressive disease in which patients frequently present with gut perforation.