Multiple lymphomatous polyposis of the gastrointestinal tract: Prospective clinicopathologic study of 31 cases

A. Ruskone-Fourmestraux, A. Delmer, A. Lavergne, T. Molina, N. Brousse, J. Audouin, J. C. Rambaud, C. Matuchansky, B. Messing, Y. Bouhnik, A. Bitoun, J. Cosnes, F. Carbonnel, J. P. Gendre, R. Jian, M. Lemann, R. Modigliani, P. Pescatore, B. Rio, R. ZittounJ. D. Grange, P. Opolon, C. Buffet, F. Prat, G. Pelletier, C. Eugene, A. Boruchowichz, J. F. Colombel, H. Louvet, L. Legoux, L. Le Bodic, J. P. Galmiche, R. Lemaire

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Abstract

Background and Aims: Multiple lymphomatous polyposis (MLP) is an uncommon type of gastrointestinal lymphoma. The aim of this study was to analyze the clinicopathologic features and outcome of a large series of patients. Methods: From 1984 to 1995, 31 patients were enrolled prospectively. Exhaustive staging and immunohistochemical analysis were performed. The first 10 patients were treated with cyclophosphamide- vincristine-prednisolone (COP) and the subsequent 21 with doxorubicin- teniposide-cyclophosphamide-prednisolone (AVmCP). Eleven patients were treated with high-dose radiochemotherapy and stem cell autotransplantation because of partial response or relapse. Results: Advanced age and multiple polyps involving several gastrointestinal segments characterized initial clinical presentation. The typical tumor cell phenotype (pan-B+, CD5+, and CD10-) along with Bcl-1 rearrangement indicated that MLP is the gastrointestinal counterpart of mantle cell lymphoma. After COP treatment, only 3 patients achieved partial remissions, whereas 80% of patients after AVmCP treatment achieved complete or good partial remission. High-dose radiochemotherapy changed partial into complete remission in 80% of cases. None of the initial 10 patients survived for 3 years, but the expected 5- year survival rate was 59% in patients receiving the anthracycline-containing regimen (P < 0.0001). Conclusions: MLP is a distinct entity among gastrointestinal lymphomas. An anthracycline-containing multidrug regimen and high-dose radiochemotherapy supported by autotransplantation seem to improve its poor prognosis.

Original languageEnglish
Pages (from-to)7-16
Number of pages10
JournalGastroenterology
Volume112
Issue number1
DOIs
StatePublished - 1997
Externally publishedYes

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