From the Guidelines
Endoscopy is not routinely required to exclude extranodal involvement in nodal marginal zone lymphoma (NMZL). For most patients with NMZL, a comprehensive staging workup including physical examination, laboratory tests, CT or PET/CT imaging, and bone marrow biopsy is sufficient to assess disease extent, as outlined in the ESMO clinical practice guidelines for diagnosis, treatment, and follow-up of marginal zone lymphomas 1.
Key Considerations
- NMZL usually presents with disseminated lymphadenopathy, with or without bone marrow and blood involvement at diagnosis, and initial staging aims to discriminate localized from advanced-stage disease 1.
- The disease is often advanced at presentation, and around one-third of cases may represent nodal dissemination of an extranodal marginal zone lymphoma (EMZL), emphasizing the need to rule out primary EMZL 1.
- Endoscopy should be performed only when there are specific clinical indications, such as gastrointestinal symptoms, abnormal imaging findings suggesting GI tract involvement, or elevated risk factors for extranodal disease.
Clinical Approach
- NMZL is primarily a nodal disease, with lymph node involvement being the predominant feature, unlike extranodal marginal zone lymphoma (MALT lymphoma) which primarily affects extranodal sites 1.
- If clinical assessment raises suspicion for GI tract involvement, then targeted endoscopic evaluation with biopsies would be appropriate, balancing the need for accurate staging while avoiding unnecessary invasive procedures in patients without clinical indications of extranodal disease.
- A comprehensive staging workup, as recommended by the ESMO guidelines 1, is crucial for determining the extent of disease and guiding treatment decisions, and endoscopy is not a routine part of this workup unless clinically indicated.
From the Research
Nodal Marginal Zone Lymphoma Diagnosis
- The diagnosis of nodal marginal zone lymphoma (NMZL) can be challenging, and a thorough work-up is necessary to exclude occult extranodal involvement 2.
- A multidisciplinary approach, including endoscopy, imaging, and small bowel resection or dissection of hypermetabolic lymph nodes, can be useful in diagnosing small intestinal marginal zone lymphoma 3.
- Endoscopy may not always be necessary to exclude extranodal involvement, as the diagnosis can be established by other means, such as surgery or imaging 3, 2.
Extranodal Involvement
- Patients with NMZL typically present with nodal disease, usually at advanced stages, and a thorough work-up is necessary to exclude occult extranodal involvement 2.
- The clinical presentation of NMZL can be variable, and extranodal involvement can occur, making it essential to perform a thorough diagnostic work-up 3, 4.
- The use of 2-18F-FDG-PET/CT can be helpful in detecting hypermetabolic lymph nodes and guiding further diagnostic procedures 3.
Diagnostic Approaches
- A scoping review of the literature revealed that diagnosis of marginal zone lymphoma with small intestinal manifestation was mainly established by surgery (73%) 3.
- Endoscopic findings, such as mucosal erosions and ulcerations, can be non-specific, and intestinal biopsies are frequently negative 3.
- The use of flow cytometry, imaging, and surgical procedures can be helpful in establishing a diagnosis of NMZL 3, 2.