Journal of Clinical Oncology, Vol 12, 1375-1382, Copyright © 1994 by American Society of Clinical Oncology
Primary small-intestinal lymphomas in Taiwan: immunoproliferative small- intestinal disease and nonimmunoproliferative small-intestinal disease
LY Shih, SJ Liaw, P Dunn and TT Kuo
Department of Internal Medicine, Chang Gung Memorial Hospital, Chang Gung Medical College, Taipei, Taiwan, Republic of China.
PURPOSE: The clinicopathologic findings in 45 adult Chinese patients with
primary small-intestinal lymphoma (PSIL) are described and compared with
those in Western countries and in underdeveloped nations. The efficacy of
combination chemotherapy is also assessed. PATIENTS AND METHOD: Six
patients had immunoproliferative small-intestinal disease (IPSID) indicated
by the presence of alpha-heavy chain protein (alpha- CP) in body fluids or
tumor tissues. Thirty-nine patients had non- IPSID, including one with
postrenal transplant lymphoma. Thirty-three non-IPSID patients received a
minimum of four cycles of combination chemotherapy with cyclophosphamide,
doxorubicin, vincristine, and prednisolone (CHOP). RESULTS: All IPSID
patients presented with the clinical and laboratory features of severe
intestinal malabsorption, and all had diffuse lymphoplasmacytic
infiltration in the mucosa of the small bowel. Lymphomas were localized
mainly in the jejunum and mesenteric nodes. The histologic subtypes were
diffuse large cell in two, immunoblastic in three, and diffuse mixed in
one. All patients responded poorly to chemotherapy, with a median survival
duration of 10.5 months. The common presenting symptoms of the 39 non-IPSID
patients included abdominal pain (90%), weight loss (31%), abdominal mass
(26%), obstruction (26%), and perforation (23%). Diffuse large- cell and
immunoblastic lymphomas constituted 82% of cases. Four patients had stage
IE, 19 stage II 1E, and 16 stage 112E disease according to the Musshoff's
criteria; 22 had bulky tumors and 19 had multiple tumors. The tumors were
completely resected in 14 patients. Of 33 patients treated with combination
chemotherapy, 73% achieved a complete remission. With a median follow-up
duration of 90 months, there have been four relapses, with only one at the
primary tumor site. The overall 5-year survival and disease-free survival
rates for non- IPSID patients who were treated with chemotherapy were 59%
and 54%, respectively. CONCLUSION: Intensive chemotherapy produces
long-term disease-free survival in locally advanced non-IPSID PSIL.
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