Limited Role of Workup Including PET-CT, Esophagogastroduodenoscopy and Bone Marrow Biopsy in Staging of Ocular Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue (MALT) Type

Background

Lymphoma of the ocular adnexa accounts for approximately 1 to 2% of non-Hodgkin lymphomas and the most common subtype is extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type (80-90%). Ocular MALT lymphoma is mostly localized disease with indolent clinical course. The workup for non-gastric MALToma is usually done similar to the workup for other non-Hodgkin lymphoma. However, as there are paucity of data in the role of PET, esophagogastroduodenoscopy and bone marrow biopsy,weaimed toinvestigate these issues in patients with ocular MALT lymphoma.

Patients and Method

One hundred thirty patients diagnosed as ocular MALT lymphoma between Jan 2001 and Dec 2015 at a single tertiary hospital were included in this retrospective analysis. We retrospectively reviewed clinical characteristics, diagnosis, natural history of disease, treatment and outcomes.

Result

The median age of all patients was 48 years (range, 20-70 years). Fifty-nine (45%) were male patients. The majority of patients (n=87, 67%) presented with stage I disease, followed by bilateral involvement, defined as stage I+I (n=21, 16%), stage IV (n=16, 12%), and stage II (n=6, 5%). Nodal involvement was present in 8% (n=10), and 12% (n=16) of patents accompanied extra-nodal involvement other than orbits at initial diagnosis. Positron emission tomography (PET) had low sensitivity in detecting primary orbit lesions (n=44/120, 37%). Moreover, only in 3% of cases (n=4), PET imaging after CT scans on neck, chest, abdomen and pelvis could provide additional clinically meaningful information that led to upstaging. Accompanying gastric MALToma was found in 2% (n=2/82) of patients by EGD. Those two patients all survived until last follow-up. Bone marrow (BM) involvement was found in 6% (n=7/120). None of them had clinically significant cytopenia. With a median follow-up period of 74 months, 5-year overall survival (OS) rate was 99% in all patients. There was no significant difference in 5-year OS rates according to BM involvement (100% with and 99% without BM involvement).

Conclusion

PET-CT, EGD and BM biopsy has limited role in staging workup of primary ocular MALT lymphoma. Those workups should be performedonly in selected cases.