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Localized Follicular Lymphoma
Management of Patients With Localized Follicular Lymphoma

Released: July 29, 2015

Expiration: July 27, 2016

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Therapy for follicular lymphomas has undergone significant change in the last decade with the advent of newer chemotherapeutics, targeted, and immunomodulatory agents. These agents have improved disease-free survival outcomes and have the potential to alter the natural history of the disease. Recently approved targeted agents and immunomodulatory agents including PI3-kinase inhibitors like idelalisib and lenalidomide in combination with rituximab have also provided new options for relapsed or refractory disease. Although substantial data and personal opinion exists for the management of patients with advanced-stage disease, information about the optimal management of limited stage disease is a matter of debate and based on limited data. The following case is an example of this clinical challenge.

Case Presentation
A 57-year-old man presents with worsening back pain for the last 3 months. History and physical examination were unremarkable and imaging with a thoracic spine MRI revealed a 5 x 4 cm mass in the paravertebral region at the T10 level. Needle biopsy was suspicious for a lymphoma, and an excisional biopsy revealed fibrous tissue infiltrated by an atypical lymphoid infiltrate. The lymphoid cells were composed of small to medium sized cells with irregular folded nuclei, inconspicuous nucleoli, condensed chromatin, and scant cytoplasm. Scattered larger cells were present with cytoplasmic retraction artifact and vesicular chromatin; however, no sheets or aggregates of large cells were seen. Most of the cells were B cells coexpressing CD20 and CD10 but not CD5, BCL1, or BCL2. The Ki67 proliferation index was approximately 20% to 30%. Flow cytometry analysis found a population of lambda-predominant B cells expressing CD19, CD20, and CD10 without CD5 coexpression. FISH was positive for t(14;18). Bone marrow biopsy and staging PET/CT scan showed no evidence of any other disease sites or FDG uptake and confirmed the diagnosis of localized, low-grade stage I follicular lymphoma.

Management Strategies
Three main modalities are used for the management of patients with limited-stage disease. This includes observation, radiation and chemoimmunotherapy, or immunotherapy alone. Observation is used in patients who are likely to experience significant morbidity with the use of radiation or systemic therapy, including patients with comorbid conditions or patients with disease involving the face and neck. Involved field radiation therapy is a very effective therapeutic option that serves as definitive curative therapy in a subset of patients with limited-stage disease. Chemotherapy with or without immunotherapy is rarely used for localized follicular lymphoma. However, an argument can be made for patients with grade 3 histology and/or bulky disease who may benefit more from these regimens and may experience longer progression-free intervals. Prognosis of patients with localized follicular lymphoma is excellent, and more patients can potentially be cured by definitive radiation therapy. A discussion of pros and cons of each therapeutic approach is essential with the patient and can help formulate a comprehensive management plan that will suit the individual need. In my practice, the patient in the clinical case detailed above was treated with definitive radiation that resulted in rapid symptomatic relief and complete response without major adverse effects.

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