International Journal of Clinical Oncology

, Volume 24, Issue 12, pp 1620–1628 | Cite as

Treatment-associated outcomes of patients with primary ocular adnexal MALT lymphoma after accurate diagnosis

  • Yuya Masuda
  • Kazuto Takeuchi
  • Toshio Kodama
  • Tomoaki Fujisaki
  • Yoshitaka Imaizumi
  • Eiichi Otsuka
  • Shuji Ozaki
  • Shinji Hasebe
  • Yoshihiro YakushijinEmail author
Original Article



Differentiation between primary ocular adnexal mucosa-associated lymphoid tissue (POA-MALT) lymphoma and reactive lymphoid hyperplasias sometimes may be difficult. We have examined the treatment-associated mortality of POA-MALT lymphoma after confirmed diagnosis and evaluated their proper treatments.

Patients and methods

From 1991 through 2016, cases of POA-MALT lymphoma were retrospectively analyzed based on their pathological and molecular/immunological diagnoses.


A total of 78 cases with POA-MALT lymphoma with a median age of 66 years were analyzed over median/mean observations of 6.4/7.1 years. Forty-four patients (56%) were diagnosed with IgH gene clonality and 10 patients (13%) were diagnosed with flow cytometric analysis in addition to the pathological decision. The rest (24 patients, 31%) were diagnosed employing pathological decisions of hemato-pathologists and clinical decisions. All patients, except cases of watchful waiting, achieved complete remission. After initial treatment, 68 patients (87%) presented disease-free during the observation period. As treatment, a radiotherapy-based strategy was followed with 15 patients (19%, group A). Immuno-chemotherapy was administered to 24 patients (31%, B). Surgical extraction only was selected for 36 patients (46%, C). Watchful waiting was selected with three patients (4%). Recurrence after the initial treatment was found in one patient (7%) out of A, in three patients (13%) out of B, and in six patients (17%) out of C, respectively. Progression-free survivals at 5 and 10 years were 100 and 100% in A, 95 and 75% in B, and 88 and 81% in C, respectively. The recurrence rates between the patients who were diagnosed with only pathological decision (n = 24) and the patients who were diagnosed with molecular and immunological procedures (n = 54) did not show any statistical differences.


Our results indicate that radiotherapy-based treatment strategies for patients with POA-MALT lymphoma show a low rate of recurrence and may improve their prognosis even after the accurate diagnosis. However, contamination of the cases with reactive (polyclonal) lymphoid hyperplasia into those with MALT lymphoma should be carefully removed to avoid unnecessary treatment for malignancies that do not exist.


Monoclonality Non-Hodgkin lymphoma POA-MALT lymphoma Radiotherapy Treatment-associated mortality 



We would like to thank the staff members in the division of cell sorting and hematological analysis in Ehime University Hospital.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no competing interests.

Supplementary material

10147_2019_1481_MOESM1_ESM.docx (15 kb)
Supplementary file1 (DOCX 15 kb)


  1. 1.
    Wotherspoon AC, Ortiz-Hidalgo C, Falzon MR et al (1991) Helicobacter pylori-associated gastritis and primary B-cell gastric lymphoma. Lancet 338:1175–1176CrossRefGoogle Scholar
  2. 2.
    Lecuit M, Abachin E, Martin A et al (2004) Immunoproliferative small intestinal disease associated with Campylobacter jejuni. New Engl J Med 350:239–248CrossRefGoogle Scholar
  3. 3.
    Hyjck E, Smith WJ, Isaacon PG (1988) Primary B cell lymphoma of salivary glands and its relationship to myoepithelial sialadenitis. Hum Pathol 19:766–776CrossRefGoogle Scholar
  4. 4.
    Hyjck E, Isaacon PG (1988) Primary B cell lymphoma of the thyroid and its relationship to Hashimoto’s thyroiditis. Hum Pathol 19:1315–1326CrossRefGoogle Scholar
  5. 5.
    Ferreri AJ, Guidoboni M, Ponzoni M et al (2004) Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. J Natl Cancer Inst 96:586–594CrossRefGoogle Scholar
  6. 6.
    Yakushijin Y, Kodama T, Takaoka I et al (2007) Absence of chlamydial infection in Japanese patients with ocular adnexal lymphoma of mucosa-associated lymphoid tissue. Int J Hematol 85:223–230CrossRefGoogle Scholar
  7. 7.
    Harada K, Murakami N, Kitaguchi M et al (2014) Localized ocular adnexal mucosa-associated lymphoid tissue lymphoma treated with radiation therapy: a long-term outcome in 86 patients with 104 treated eyes. Int J Radiat Oncol Biol Phys 88:650–654CrossRefGoogle Scholar
  8. 8.
    Tanimoto K, Kaneko A, Suzuki S et al (2006) Long-term follow-up results of no initial therapy for ocular adnexal MALT lymphoma. Ann Oncol 17:135–140CrossRefGoogle Scholar
  9. 9.
    Knowles DM, Jakobiec FA, McNally L et al (1990) Lymphoid hyperplasia and malignant lymphomas occurring in the ocular adnexa (orbita, conjunctiva, and eyelids): a prospective multiparametric analysis of 108 cases during 1977 to 1987. Hum Pathol 21:959–973CrossRefGoogle Scholar
  10. 10.
    The Non-Hodgkin’s Lymphoma Classification Project (1997) A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin’s lymphoma. Blood 89:3909–3918CrossRefGoogle Scholar
  11. 11.
    Coupland SE, Krause L, Delecluse HJ et al (1998) Lymphoproliferative lesions of the ocular adnexa: Analysis of 112 cases. Ophthalmology 105:1430–1441CrossRefGoogle Scholar
  12. 12.
    Jenkins C, Rose GE, Bunce C et al (2000) Histological features of ocular adnexal lymphoma (REAL classification) and their association with patient morbidity and survival. Br J Ophthalmol 84:907–913CrossRefGoogle Scholar
  13. 13.
    McKelvie PA, McNab A, Francis IC et al (2001) Ocular adnexal lymphoproliferative disease: a series of 73 cases. Clin Exp Ophthalmol 29:387–393CrossRefGoogle Scholar
  14. 14.
    Fung CY, Tarbell NJ, Lucarelli MJ et al (2003) Ocular adnexal lymphoma: clinical behavior of distinct World Health Organization classification subtypes. Int J Radiat Oncol Biol Phys 57:1382–1391CrossRefGoogle Scholar
  15. 15.
    Woolf DK, Kuhan H, Shoffren O et al (2015) Outcomes of primary lymphoma of the ocular adnexa (orbital lymphoma) treated with radiotherapy. Clin Oncol (R Coll Radiol) 27:153–159CrossRefGoogle Scholar
  16. 16.
    Platt S, Al Zahrani Y, Singh N et al (2017) Extranodal marginal zone lymphoma of ocular adnexa: outcomes following radiation therapy. Ocul Oncol Pathol 3:181–187CrossRefGoogle Scholar
  17. 17.
    Desai A, Joag MG, Lekakis L et al (2017) Long-term course of patients with primary ocular adnexal MALT lymphoma: a large single-institution cohort study. Blood 129:324–332CrossRefGoogle Scholar
  18. 18.
    Goda JS, Gospodarowicz M, Pintilie M et al (2010) Long-term outcome in localized extranodal mucosa-associated lymphoid tissue lymphomas treated with radiotherapy. Cancer 116:3815–3824CrossRefGoogle Scholar
  19. 19.
    Hashimoto N, Sasaki R, Nishimura H et al (2012) Long-term outcome and patterns of failure in primary ocular adnexal mucosa-associated lymphoid tissue lymphoma treated with radiotherapy. Int J Radiat Oncol Biol Phys 82:1509–1514CrossRefGoogle Scholar
  20. 20.
    Kaushik M, Pulido JS, Schild SE et al (2012) Risk of radiation retinopathy in patients with orbital and ocular lymphoma. Int J Radiat Oncol Biol Phys 84:1145–1150CrossRefGoogle Scholar
  21. 21.
    Cho WK, Lee SE, Paik JS et al (2013) Risk potentiality of frontline radiotherapy associated cataract in primary ocular adnexal mucosa-associated lymphoid tissue lymphoma. Korean J Ophthalmol 27:243–248CrossRefGoogle Scholar
  22. 22.
    Park HH, Lee SW, Sung SY et al (2017) Treatment outcome and risk analysis for cataract after radiotherapy of localized ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma. Radiat Oncol J 35:249–256CrossRefGoogle Scholar
  23. 23.
    Laurenti L, De Padua L, Battendieri R et al (2011) Intralesional administration of rituximab for treatment of CD20 positive orbital lymphoma: safety and efficacy evaluation. Leuk Res 35:682–684CrossRefGoogle Scholar
  24. 24.
    Savino G, Battendieri R, Balia L et al (2012) Evaluation of intraorbital injection of rituximab for treatment of primary ocular adnexal lymphoma: a pilot study. Cancer Sci 102:1565–1567CrossRefGoogle Scholar
  25. 25.
    Savino G, Battendieri R, Gari M et al (2013) Long-term outcomes of primary ocular adnexal lymphoma treatment with intraorbital rituximab injections. J Cancer Res Clin Oncol 139:1251–1255CrossRefGoogle Scholar
  26. 26.
    Streubel B, Lamprecht A, Dierlamm J et al (2003) T(14;18)(q32;q21) involving IGH and MALT1 is a frequent chromosomal aberration in MALT lymphoma. Blood 101:2335–2339CrossRefGoogle Scholar

Copyright information

© Japan Society of Clinical Oncology 2019

Authors and Affiliations

  1. 1.Department of Clinical OncologyEhime University School of MedicineEhimeJapan
  2. 2.Cancer CenterEhime University HospitalToonJapan
  3. 3.Department of OphthalmologyMatsuyama Red Cross HospitalEhimeJapan
  4. 4.Department of HematologyMatsuyama Red Cross HospitalMatsuyamaJapan
  5. 5.Department of HematologyNagasaki University HospitalNagasakiJapan
  6. 6.Department of HematologyOita Prefectural HospitalOitaJapan
  7. 7.Department of HematologyTokushima Prefectural Central HospitalTokushimaJapan
  8. 8.Department of Clinical OncologyEhime University Graduate School of MedicineShitsukawa EhimeJapan

Personalised recommendations