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Paris staging system for primary gastrointestinal lymphomas
  1. A Ruskoné-Fourmestraux1,
  2. B Dragosics2,
  3. A Morgner3,
  4. A Wotherspoon4,
  5. D de Jong5
  1. 1Hotel Dieu, Paris, France
  2. 2Gesundheitszentrum, Vienna, Austria
  3. 3Medical Department 1, Technical University Hospital, Dresden, Germany
  4. 4Royal Marsden Hospital, London, UK
  5. 5The Netherlands Cancer Institute, Amsterdam, the Netherlands, on behalf of the European Gastro-Intestinal Lymphoma Study Group (EGILS)
  1. Correspondence to:
    Dr D De Jong, Department of Pathology, the Netherlands Cancer Institute, Amsterdam 1066 CX, the Netherlands;

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Currently, much effort is put into the development of specific therapeutical approaches that are tailored to specific lymphoma entities. As a consequence, more specific information needs to be collected to support the choice of therapy. In gastric marginal zone B cell lymphoma of MALT-type, the need for specialised documentation of tumour extent in the organ wall has been stressed by the high predictive value for failure of response after Helicobacter pylori eradication of infiltration beyond the mucosa, a predictive parameter irrespective of the presence of the t(11;18) translocation.1–,5 With new techniques such as endosonography, this infiltration depth can be assessed with improving accuracy. However, the information cannot be translated into the current staging systems. The dissemination patterns of extranodal lymphomas are also essentially different from primary nodal lymphomas.6 As tumour stage is one of the most important guidelines in the choice of local therapy (surgery, radiotherapy) and chemotherapy, adequate documentation of tumour localisation in the organ related lymph nodes and beyond is essential.

The Ann Arbor staging system, developed for and routinely used in nodal non-Hodgkin’s lymphoma, is not optimal for documentation of the specific relevant features of primary extranodal lymphoma in the gastrointestinal tract. Several modifications and alternatives have been proposed. However, neither differentiation of stage I1 (confinement of lymphoma to the mucosa and submucosa) from stage I2 (tumour extension beyond the submucosa according to Radaszkiewicz and colleagues7) nor discrimination of stage II1 (involvement of regional lymph nodes) from stage II2 (node involvement beyond the regional area, as assessed in the Musshoff modification8) is sufficiently serving the demand for documenting all features of lymphoma. To meet these shortcomings, the Lugano classification was constructed by Rohatiner and colleagues9 introducing stage IIE for “serosa penetration” without lymph node involvement into the Ann Arbor system. This represents a change in meaning of stage II that originally indicated lymph node involvement. Therefore, the Lugano system is causing more confusion than benefit.

TNM staging for tumours of epithelial origin has also been proposed as an alternative in gastrointestinal lymphoma to describe localised disease. The “T” part of this system pertains to the anatomical structure of the organs and sufficiently fulfills the requirements for staging of local extent of the disease.

The European Gastro-Intestinal Lymphoma Study Group (EGILS) is a multidisciplinary group of investigators, including clinical investigators such as gastroenterologists, medical oncologists, radiotherapists, and pathologists, as well as basic researchers such as cellular and molecular biologists. Several groups from the UK, France, Germany, the Netherlands, Spain, and Austria now take part and have come together regularly since 1999 to discuss and study subjects in epidemiology and molecular and cell biology of gastrointestinal lymphoma. Clinical protocols and trials have been developed and performed as a collaborative effort. As a result of discussions on staging protocols and reporting systems over the past years, we would like to propose a modified TNM staging system, named after the first venue of the group in Paris. The staging system adequately records: (1) depth of tumour infiltration; (2) extent of nodal involvement; as well as (3) specific lymphoma spreading (table 1). It is adjusted to the gastrointestinal origin of the lymphoma, considering histopathological characteristics of extranodal B and T cell lymphomas. The use of this system in future studies will permit accurate comparison of the reported cohorts and should allow rapid accumulation of good data for proper stratification of patients for risk assessment and treatment options.

Table 1

Paris staging system for primary gastrointestinal lymphomas*†