Workup for a Patient with MALT Lymphoma of the Lacrimal Gland with Worsening Symptoms
For a patient with history of MALT lymphoma of the right lacrimal gland who has completed excision and radiation therapy but now presents with worsening symptoms, a comprehensive diagnostic workup should include contrast-enhanced MRI of the orbits, PET/CT scan, complete blood counts, comprehensive metabolic panel, LDH, beta-2-microglobulin, and multiple biopsies of any suspicious lesions. 1, 2
Initial Evaluation
Imaging Studies
- MRI of the orbits with contrast: Essential to evaluate the right lacrimal gland region for recurrence or progression, given the previous finding of "redemonstration of a right lacrimal gland lesion" on the 10/7/24 MRI 1
- PET/CT scan of chest, abdomen, and pelvis: To assess for systemic disease, despite the previous negative PET scan from 5/6/2025 1
- Special attention to the left iliac chain lymph nodes that previously showed hypermetabolism
- Evaluate cervical lymph nodes that showed uptake of unclear etiology on the most recent PET
Laboratory Studies
- Complete blood count with differential
- Comprehensive metabolic panel
- Serum lactate dehydrogenase (LDH) 1
- Beta-2-microglobulin 1
- Hepatitis B testing (HBsAg and HBcAb) - especially important if rituximab therapy is being considered 1
- Serum protein electrophoresis and immunofixation - to evaluate for monoclonal proteins 2
Tissue Diagnosis
Biopsy Considerations
- Biopsy of the right lacrimal gland lesion: Given the "redemonstration" on MRI and worsening symptoms, a repeat biopsy is essential to confirm recurrence 1, 2
- Biopsy of any new suspicious lesions: Particularly any enlarged or hypermetabolic lymph nodes identified on imaging 1
Pathological Analysis
- Histopathology with immunohistochemistry panel including:
- FISH analysis or PCR for detection of specific translocations if applicable 1, 2
Additional Considerations
Specialized Testing
- Ophthalmologic evaluation: Given the orbital location and worsening symptoms, a complete ophthalmologic examination is essential to assess visual function and ocular complications
- Bone marrow biopsy: Consider if systemic disease is suspected based on imaging or laboratory findings 1
Pitfalls to Avoid
- Inadequate sampling: Ensure sufficient tissue is obtained during biopsy to allow for comprehensive pathological analysis 2
- Overlooking systemic involvement: MALT lymphomas can involve multiple extranodal sites, so thorough evaluation is necessary even with initially localized disease 1
- Delayed processing of samples: Ensure proper handling of biopsy specimens for accurate FISH or PCR analysis 2
Management Planning Based on Workup Results
If recurrence is confirmed, treatment options will depend on the extent of disease:
- Localized recurrence: Consider local therapy such as radiation (if not at maximum dose from previous treatment) 1
- Systemic disease: Consider systemic therapy with rituximab alone or in combination with chemotherapy 3
The workup should be completed promptly to guide appropriate management decisions, as early intervention may improve outcomes in terms of morbidity, mortality, and quality of life.