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. 2021 Apr 9;13(8):1803.
doi: 10.3390/cancers13081803.

Treatments and Outcomes in Stage I Extranodal Marginal Zone Lymphoma in the United States

Juan Pablo Alderuccio  1 , Jorge A Florindez  2 , Isildinha M Reis  3   4 , Wei Zhao  4 , Izidore S Lossos  1   5
Affiliations

Treatments and Outcomes in Stage I Extranodal Marginal Zone Lymphoma in the United States

Juan Pablo Alderuccio et al. Cancers (Basel). .

Abstract

A considerable number of patients with extranodal marginal zone lymphoma (EMZL) are diagnosed with stage I disease. Information on treatments and survival by primary location remains limited. We extracted data from the Surveillance, Epidemiology, and End Results (SEER) database to assess treatment, primary location, and survival of patients with stage I EMZL. Results show that 7961 patients met inclusion criteria. Observation (no treatment) was the most common approach (31%) followed by radiation therapy (RT, 23%). The median overall survival (OS) was 17.3 years (95%CI 16.3 to 18.3). Shorter survival was observed in patients with stage I EMZL compared to expected survival in a cohort derived from the general U.S. population matched by sex, age, and calendar year at diagnosis. However, similar survival was observed in RT-treated patients. We identified age ≥ 60 years (SHR = 4.00, 95%CI 3.10-5.15; p < 0.001), higher grade transformation (SHR = 4.63, 95%CI 3.29-6.52; p < 0.001), and primary lung EMZL (SHR = 1.44, 95%CI 1.05-1.96; p = 0.022) as factors associated with shorter lymphoma-specific survival (LSS). Conversely, primary skin location (SHR = 0.50, 95%CI 0.33-0.77; p = 0.002) was associated with longer LSS. Our results support the use of RT as the preferred approach in localized EMZL.

Keywords: SEER database; extranodal marginal zone lymphoma; stage I; survival; treatment.

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Conflict of interest statement

J.P.A. has received honoraria from OncLive and Oncinfo and has served on the advisory board of ADC Therapeutics. An immediate family member has served on the advisory boards of Puma Biotechnology, Inovio Pharmaceuticals, Agios Pharmaceuticals, Forma Therapeutics, and Foundation Medicine. I.S.L. has served on the advisory boards of Seattle Genetics, Janssen Scientific, and Verastem.

Figures

Figure 1
Figure 1
Consort chart of patient selection.
Figure 2
Figure 2
Overall survival in SEER stage I EMZL cohort vs. age-, sex-, and calendar-matched U.S. population cohort (A); overall survival in SEER stage I EMZL cohort by primary location (B), by treatment modality (C), and by combined treatment modality and RT only (D). Tick marks for censored observations not shown given the large number of patients.
Figure 3
Figure 3
Overall survival in patients with stage I extranodal marginal zone lymphoma treated with radiation only (n = 1835) versus age-, sex-, and calendar-matched U.S. population cohort (A); overall survival in patients with stage I EMZL treated with radiation only (n = 1835) by primary location (B); and cumulative incidence rate of lymphoma-specific death in stage I EMZL overall (C) and by primary location (D). Tick marks for censored observations not shown given the large number of patients.
See this image and copyright information in PMC

References

    1. Zucca E., Arcaini L., Buske C., Johnson P.W., Ponzoni M., Raderer M., Ricardi U., Salar A., Stamatopoulos K., Thieblemont C., et al. Marginal zone lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann. Oncol. 2020;31:17–29. doi: 10.1016/j.annonc.2019.10.010. - DOI - PubMed
    1. Du M.-Q. Mucosa-associated lymphoid tissue lymphoma of various sites: Common molecular mechanisms but different players. Ann. Lymphoma. 2020:4. doi: 10.21037/aol-20-15. - DOI
    1. Zucca E., Bertoni F. The spectrum of MALT lymphoma at different sites: Biological and therapeutic relevance. Blood. 2016;127:2082–2092. doi: 10.1182/blood-2015-12-624304. - DOI - PubMed
    1. Alderuccio J.P., Lossos I.S. Prognostic factors and risk of transformation in marginal zone lymphoma. Ann. Lymphoma. 2020:4. doi: 10.21037/aol-20-8. - DOI
    1. Zucca E., Conconi A., Martinelli G., Bouabdallah R., Tucci A., Vitolo U., Martelli M., Pettengell R., Salles G., Sebban C., et al. Final Results of the IELSG-19 Randomized Trial of Mucosa-Associated Lymphoid Tissue Lymphoma: Improved Event-Free and Progression-Free Survival With Rituximab Plus Chlorambucil Versus Either Chlorambucil or Rituximab Monotherapy. J. Clin. Oncol. 2017;35:1905–1912. doi: 10.1200/JCO.2016.70.6994. - DOI - PubMed

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