With IgG4-RD, having MALT lymphoma near my eye wasn’t entirely surprising

I have lived with immunoglobulin G4-related disease (IgG4-RD) affecting my orbits, or eye sockets, for more than 25 years. It began with recurring eyelid swelling and eventually progressed to double vision. While the eyelid swelling has largely subsided over time, I haven’t seen straight since the 1990s.

Around 2018, I noticed a small lump near the tear duct under my eye, about the size of a pea. I consulted two highly respected ophthalmologists, and both thought it was probably a blocked tear duct.

In early 2025, I noticed the lump growing. The change was subtle at first, but over time it increased to roughly the size of a pearl. Concerned by its growth, I insisted on having it removed. I was advised to seek care from an orbital and oculoplastic surgeon and ultimately selected a well-respected oculofacial plastic surgeon.

Between finding the right specialist, completing consultations and testing, obtaining insurance authorization, and waiting for an opening on the surgeon’s schedule, the procedure was finally performed in December 2025. While the removed mass didn’t appear concerning, the pathology report told a different story.

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The mass was diagnosed as mucosa-associated lymphoid tissue (MALT) lymphoma, a slow-growing type of non-Hodgkin lymphoma that develops from B-cells in the marginal zones of lymphoid tissues. MALT lymphoma is rare and is typically classified as either:

• Gastric (relating to the stomach)
• Nongastric, most commonly affecting the salivary glands, thyroid, lungs, skin, gut, or tissues around the eye

It typically develops in people older than 60. Given the known association between IgG4-RD and B-cells, chronic inflammation, and tumor formation, the diagnosis was not entirely surprising.

My oncologist told me that if any cancer were to develop, this was “the best one” to get. He explained that MALT lymphoma typically originates outside the lymph nodes, so it remains localized, responds well to treatment, and has a very high treatment success rate. He believed we had caught it early.

According to a 2018 article published in Virchows Archiv: European Journal of Pathology, although lymphomas are uncommon in patients with IgG4-RD, “most reported cases … are MALT lymphomas occurring in orbital IgG4-RD.”

I also found a study of 121 patients with orbital MALT lymphoma treated at the West China Hospital of Sichuan University between 2012 and 2017. The researchers found that the “positive rates of IgG and IgG4 in the cases of orbital MALT lymphoma were 90.91% and 61.98%, respectively.”

Following my diagnosis, I underwent a bone marrow biopsy to determine whether the cancer had spread elsewhere. The biopsy involved collecting a sample from my hip, similar in concept to a spinal tap that I had undergone decades earlier. Although the in-office procedure was uncomfortable, it was not nearly as painful as I had feared despite the size of the needle. I was incredibly relieved when the biopsy came back negative.

Because IgG4-RD is a systemic autoimmune disease characterized by inflammation and the formation of tumor-like masses, it is important to remain vigilant for changes in or on your body. Looking back, I should have been more insistent about having the lump looked into. But I am grateful that I trusted my instincts and continued to advocate for further evaluation when something did not seem right.

Note: IgG4-RD News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of IgG4-RD News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to IgG4-RD.