Managing Extranodal MZL

Ariela Noy, MD:A 64-year-old woman with Sjögren’s syndrome develops masses near her right carotid. She has a workup that shows that she has bilateral carotid masses, some small lymph nodes, and a lesion in the left lung. Serial biopsies demonstrate that the carotid mass is marginal zone lymphoma and that there is a concordant B-cell infiltrate in the lung lesion.

At first, she is observed. However, the carotid lesion becomes more symptomatic. A decision is made to treat her with rituximab. After 4 weeks of therapy, she has resolution of her symptoms and serial imaging demonstrates that she has a near complete response. However, at 20 months, she develops a contralateral lung lesion, which also demonstrates the same B-cell infiltrate.

At 20 months, when she has progression of her disease, the physician decides to retreat her with rituximab. Unfortunately, she does not have a treatment response and a decision is made to start ibrutinib.

Marginal zone lymphoma is a relatively common type of indolent lymphoma, but it is not as common as follicular lymphoma. More physicians are comfortable with treating follicular lymphoma and sometimes miss the nuances between the 2 diseases.

Despite the fact that most patients with disseminated disease may feel anxious about their prognosis, their prognosis is actually quite excellent. We currently anticipate that patients like this will have a normal life expectancy. Some of them will either be treated initially or in follow up. Some will never be treated. Regardless, we expect them to live for decades.

Transcript edited for clarity.

A 64-Year-Old Woman With Advanced Extranodal MZL

January 2016

• PH: At age 64, the patient presented with a fever of unknown origin, weight loss, and fatigue
  • PE: revealed 2 masses near left ear
  • PMH: Sjogren’s syndrome, symptoms managed on cevimeline
• CT revealed bilateral involvement in parotid glands and a 3.0-cm. mass in the left lung
• Biopsies confirmed presence of MALT lymphoma in salivary gland and lung with nodules of diffuse heterogeneous B-cell infiltrate
• IHC: B cell phenotype CD20
• HBC, HBV, and other infections ruled out

Treatment History

• After 6-month period of active monitoring/observation, salivary masses began to cause patient distress; she also developed a persistent cough
  • CT revealed an additional new mass in left lung
• Decision was made to start patient on a course of rituximab
• Follow-up imaging at 6 months and 9 months showed near complete remission

March 2018

• Imaging at 20 months showed disease progression in the lung  
• Patient started on treatment with rituximab monotherapy

June 2018

• Imaging at 3 months showed no response to therapy
• The patient was started on treatment with ibrutinib