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Annals of Oncology 14:131-139, 2003
© 2003 European Society for Medical Oncology

Original Paper

Primary extranodal non-Hodgkin’s lymphoma (NHL): the impact of alternative definitions tested in the Comprehensive Cancer Centre West population-based NHL registry

A. D. G. Krol1,+, S. le Cessie2, S. Snijder3, J. C. Kluin-Nelemans4, P. M. Kluin5 and E. M. Noordijk1

Departments of 1 Clinical Oncology and 2 Medical Statistics, Leiden University Medical Centre, Leiden; 3 The Comprehensive Cancer Centre West, Leiden; Departments of 4 Hematology and 5 Pathology, University Hospital Groningen, Groningen, The Netherlands

Received 12 July 2002; accepted 23 July 2002

Background:

The definition of primary extranodal non-Hodgkin’s lymphoma (NHL) is a controversial issue, especially in patients where both nodal and extranodal sites are involved.

Patients and methods:

The impact of different definitions of primary extranodal NHL on incidence and prognosis is explored using data from a population-based NHL registry.

Results:

Using liberal criteria, 389 (34%) cases were classified as primary extranodal NHL. Overall survival (OS) rates of nodal and extranodal NHL patients defined this way were comparable; however, extranodal NHL patients had a better disease-free survival (DFS). When strict criteria were applied, 231 cases (20%) were classified as primary extranodal NHL. OS and DFS rates of extranodal NHL patients defined this way were superior to nodal NHL patients; however, the difference in OS was reversed after correction for differences in International Prognostic Index and malignancy grade.

Conclusion:

This study illustrates the selection bias that is introduced when a strict definition of primary extranodal NHL, that excludes cases with disseminated disease, is used. Patients with primary extranodal NHL were found to have a superior DFS, irrespective of which definition of primary extranodal NHL was used.

Key words: extranodal, nodal, non-Hodgkin’s lymphoma, prognosis


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