Primary NK-/T-cell lymphoma of the gastrointestinal tract: clinical characteristics and endoscopic findings

Background and study aims: Primary NK-/T-cell lymphoma of the gastrointestinal tract is a very rare disease with a poor prognosis. The aim of this study was to determine the clinical and endoscopic characteristics of patients with primary gastrointestinal NK-/T-cell lymphoma.

Patients and methods: The clinical features of 14 patients with primary gastrointestinal NK-/T-cell lymphoma and the endoscopic findings in 11 of these patients were reviewed. Their median age was 42 years (range 23 - 78) at the time of diagnosis.

Results: The initial presenting symptoms of primary gastrointestinal NK-/T-cell lymphoma were gastrointestinal bleeding (n = 6, 42 %), abdominal pain (n = 4, 29 %), and epigastric soreness (n = 4, 29 %). The disease was at an advanced stage at the time of diagnosis: stage II in 5 patients (36 %); stage III in 4 (28 %); and stage IV in 5 (36 %). Initial treatment was with chemotherapy (n = 8, 57 %) or surgical resection (n =5, 36 %). The median survival for all patients was 9 months. On endoscopy in 11 patients, the anatomic location of the primary lesion was found to be: stomach, n = 3 (27 %); esophagus, n =2 (18 %); duodenum, n = 1 (9 %); and the ileocolonic area, n = 5 (46 %). These lesions were ulceroinfiltrative in 4 cases (36 %), ulcerative in 3 cases (27 %), superficial/erosive in 3 cases (27 %), and infiltrative in 1 case (9 %). No prominent fungating mass was seen in any patient.

Conclusions: Primary gastrointestinal NK-/T-cell lymphoma was endoscopically characterized by superficial/erosive, ulcerative, or ulceroinfiltrative lesions without fungating mass. The most common presenting symptom was gastrointestinal bleeding. Despite aggressive treatments, the prognosis was very poor.

References

• 1 Jaffe E S, Harris N L, Stein H. et al .World Health Organization classification of tumours. Pathology and genetics of tumours of the hematopoietic and lymphoid tissues. Lyon, France; IARC Press 2001
• 2 Shih L Y, Liang D C. Non-Hodgkin's lymphomas in Asia.  Hematol Oncol Clin North Am. 1991;  5 983-1001
• 3 Ko Y H, Kim C W, Park C S. et al . REAL classification of malignant lymphomas in the Republic of Korea: incidence of recently recognized entities and changes in clinicopathologic features. Hematolymphoreticular Study Group of the Korean Society of Pathologists. Revised European-American lymphoma.  Cancer. 1998;  83 806-812
• 4 Ko Y H, Cho E Y, Kim J E. et al . NK and NK-like T-cell lymphoma in extranasal sites: a comparative clinicopathological study according to site and EBV status.  Histopathology. 2004;  44 480-489
• 5 Chim C S, Au W Y, Shek T W. et al . Primary CD56 positive lymphomas of the gastrointestinal tract.  Cancer. 2001;  91 525-533
• 6 Chan J K, Sin V C, Wong K F. et al . Nonnasal lymphoma expressing the natural killer cell marker CD56: a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm.  Blood. 1997;  89 4501-4513
• 7 Lee J, Park Y H, Kim W S. et al . Extranodal nasal type NK-/T-cell lymphoma: elucidating clinical prognostic factors for risk-based stratification of therapy.  Eur J Cancer. 2005;  41 1402-1408
• 8 Chim C S, Ma S Y, Au W Y. et al . Primary nasal natural killer cell lymphoma: long-term treatment outcome and relationship with the International Prognostic Index.  Blood. 2004;  103 216-221
• 9 Cheung M M, Chan J K, Lau W H. et al . Early stage nasal NK-/T-cell lymphoma: clinical outcome, prognostic factors, and the effect of treatment modality.  Int J Radiat Oncol Biol Phys. 2002;  54 182-190
• 10 Dawson I M, Cornes J S, Morson B C. Primary malignant lymphoid tumours of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis.  Br J Surg. 1961;  49 80-89
• 11 Carbone P P, Kaplan H S, Musshoff K. et al . Report of the Committee on Hodgkin's Disease Staging Classification.  Cancer Res. 1971;  31 1860-1861
• 12 Lee J, Kim W S, Park Y H. et al . Nasal-type NK-/T cell lymphoma: clinical features and treatment outcome.  Br J Cancer. 2005;  92 1226-1230
• 13 Jaffe E S, Chan J K, Su I J. et al . Report of the Workshop on Nasal and Related Extranodal Angiocentric T/Natural Killer Cell Lymphomas. Definitions, differential diagnosis, and epidemiology.  Am J Surg Pathol. 1996;  20 103-111
• 14 Glick D D, Soule E H. Primary malignant lymphoma of colon or appendix. Report of 27 cases.  Arch Surg. 1966;  92 144-151
• 15 Jinnai D, Iwasa Z, Watanuki T. Malignant lymphoma of the large intestine-operative results in Japan.  Jpn J Surg. 1983;  13 331-336
• 16 Chiu K W, Changchien C S, Chuah S K. et al . Endoscopic and image features in primary gastro-intestinal lymphoma: a 7-year experience.  Hepatogastroenterology. 1995;  42 367-370
• 17 O’Connell D J, Thompson A J. Lymphoma of the colon: the spectrum of radiologic changes.  Gastrointest Radiol. 1978;  2 377-385
• 18 Barakat M H. Endoscopic features of primary small bowel lymphoma: a proposed endoscopic classification.  Gut. 1982;  23 36-41
• 19 Taal B G, den Hartog Jager F C, Burgers J M. et al . Primary non-Hodgkin's lymphoma of the stomach: changing aspects and therapeutic choices.  Eur J Cancer Clin Oncol. 1989;  25 439-450
• 20 Seifert E, Schulte F, Weismuller J. et al . Endoscopic and bioptic diagnosis of malignant non-Hodgkin"s lymphoma of the stomach.  Endoscopy. 1993;  25 497-501
• 21 Kelessis N G, Vassilopoulos P P, Tsamakidis K G. et al . Is gastroscopy still a valid diagnostic tool in detecting gastric MALT lymphomas? A dilemma beyond the eye. Mucosa-associated lymphoid tissue.  Surg Endosc. 2003;  17 469-474
• 22 Wang M H, Wong J M, Lien H C. et al . Colonoscopic manifestations of primary colorectal lymphoma.  Endoscopy. 2001;  33 605-609
• 23 Myung S J, Joo K R, Yang S K. et al . Clinicopathologic features of ileocolonic malignant lymphoma: analysis according to colonoscopic classification.  Gastrointest Endosc. 2003;  57 343-347
• 24 Kim Y-H, Lee J H, Yang S K. et al . Primary Colon Lymphoma in Korea: A KASID (Korean Association for the Study of Intestinal Diseases) Study.  Dig Dis Sci. 2005;  50 2243-2247