Primary Thyroid Marginal Zone B-cell Lymphoma of the Mucosa-Associated Lymphoid Tissue Type: Clinical Manifestation and Outcome of a Rare Disease - Consortium for Improving Survival of Lymphoma Study

Acta Haematol. 2012;127(2):100-4. doi: 10.1159/000333113. Epub 2011 Dec 15.


Purpose: Primary thyroid marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type (pTY-MZL) is an extremely uncommon form of lymphoma. Due to its rarity, the natural history and optimal treatment modality for this disease have yet to be clearly established.

Methods: A total of 27 patients with histologically confirmed pTY-MZL were retrospectively analyzed.

Results: The median age of our subjects was 53 years (range 25-82). This study involved 17 females (63.0%) and 10 males (37.0%). Twenty-four out of 27 patients (88.9%) initially presented with localized disease, defined by Ann Arbor stage I/II. Bone marrow involvement was detected in 8.3% of the patients (2 patients), and 91.7% of the patients (25 of 27) were categorized into the low or low-intermediate risk group, according to the International Prognostic Index criteria. Accompanying Hashimoto's thyroiditis was detected in 72% of the patients, whereas thyroglobulin antibody levels were elevated in 70% of the patients. Twenty-six patients were treated with surgery, radiotherapy or chemotherapy, and 25 patients achieved complete remission. During the follow-up period, only 2 patients evidenced progression, and no deaths occurred over the course of the study.

Conclusion: pTY-MZL tends to be an indolent disease. However, unlike other mucosa-associated lymphoid tissue site MZLs, pTY-MZL was well controlled via several treatment modalities, and the patients' responses were sustained for a prolonged period.

Publication types

  • Multicenter Study

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Combined Modality Therapy
  • Female
  • Humans
  • Lymphoma, B-Cell, Marginal Zone / pathology*
  • Lymphoma, B-Cell, Marginal Zone / therapy
  • Male
  • Middle Aged
  • Prognosis
  • Retrospective Studies
  • Thyroid Neoplasms / pathology*
  • Thyroid Neoplasms / therapy
  • Treatment Outcome