Abstract
ObjectivesFollicular lymphoma (FL) is the most common indolent B cell lymphoma in the United States and a quarter of patients present with stage I disease. The objective of this study was to examine if primary site of disease influences survival in early stage lymphoma.ResultsThe most common extranodal primary sites were the integumentary system (8%), followed by the GI tract (6.4%) and head & neck (5.6%). We stratified patients into a pre-rituximab era (1983-1998) and the rituximab era (1999-2011). In multivariable analysis, integumentary disease was associated with better overall survival (Hazard Ratio [HR], 0.77; Confidence Interval [CI], 0.66-0.9) while primary site FL of the nervous system (HR, 2.40; CI, 1.72-3.38) and the musculoskeletal system (HR, 2.14; CI, 1.44-3.18) were associated with worse overall survival when compared to primary nodal FL. Treatment in the pre-rituximab era, male gender and older age at diagnosis were associated with worse survival.MethodsWe queried the SEER database from 1983 to 2011. We included all adult patients (>18 years) with histologically confirmed stage I FL, active follow-up, and a single primary tumor. A total of 9,865 patients met eligibility criteria, with 2520 (25%) having an extranodal primary site. We classified the primary sites by organ or anatomic location into 11 sites.ConclusionPrimary site of disease is a prognostic factor for patients with early stage FL and may help identify subsets of patients that could benefit from early, aggressive treatment.
Highlights
Follicular lymphoma (FL) is the most common subtype of indolent lymphomas and accounts for 35% of all Non-Hodgkin’s Lymphomas in the United States [1,2,3]
Primary site of disease is a prognostic factor for patients with early stage FL and may help identify subsets of patients that could benefit from early, aggressive treatment
We identified 14,059 adult cases of histologically confirmed Stage I follicular lymphoma from 1983-2011, of whom 9,865 patients met eligibility criteria (Figure 1)
Summary
Follicular lymphoma (FL) is the most common subtype of indolent lymphomas and accounts for 35% of all Non-Hodgkin’s Lymphomas in the United States [1,2,3]. FL is a heterogeneous disease with variable biologic behavior and clinical course. The Follicular Lymphoma International Prognostic Index or FLIPI score is the most commonly used prognostic tool that has been revised and validated in the rituximab era (FLIPI-2)[5, 6]. Notwithstanding the prognostic classification, treatment of follicular lymphoma is guided primarily by the extent of disease involvement [7]. Advanced stage FL (stage III/IV) is considered an incurable but indolent disease for which the decision to treat is based on several well-established clinical criteria. Treatment of limited stage FL (stage I and www.impactjournals.com/oncotarget contiguous stage II) has the potential to result in long-term disease-free survival, the treatment modality of choice is not well defined [7, 8]
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