Mantle Cell Lymphoma in the Thyroid: A Rare Presentation

Abstract

Background While 2% of all extranodal Non-Hodgkin Lymphomas present in the thyroid, there exists insufficient data to describe the incidence of mantle cell lymphoma in the thyroid. A case series of 1400 patients revealed that <1% of thyroid lymphomas may be MCL; hence better understanding of the disease course is essential. Patient Findings A 65-year-old female was referred for a multinodular goiter. Multiple fine needle aspirations from the dominant right nodule were consistent with Hashimoto's thyroiditis and flow cytometry was negative. Due to progressing dysphagia, she underwent total thyroidectomy. Summary Pathology revealed MCL with mantle zone growth pattern in the right thyroid. Flow cytometry showed monoclonal B cells comprising 9% of total cells. The Ki-67 index was 10%. She was diagnosed as having stage IIE MCL and offered conservative management by medical oncology, given that she had no B symptoms. Conclusion Though chemotherapy is the treatment of choice in MCL, a subset of patients with low-grade disease may be observed. As in our patient, mantle zone growth pattern and a Ki-67 index < 10% suggest a favorable prognosis. A diagnosis of primary MCL in the thyroid remains rare and staging modalities as well as treatment options continue to evolve.

Flow cytometric, gene rearrangement, and karyotypic analyses of 110 cases of primary thyroid lymphoma: a single-institutional experience in Japan

“…Such analyses for primary nodal lymphomas have been well documented [15][16][17][18]. For PTL, however, a few sporadic reports have been published [19][20][21][22][23].…”

Mantle cell lymphoma involving the thyroid gland

“…8 Notably, only two cases of primary thyroid MCL have been described in the literature, both of which had underlying Hashimoto thyroiditis (HT) (Table 1). 3,5 Interestingly, the most common risk factor associated with PTL is HT, which can increase the risk of developing PTL by 40-80 times. 9 In a recent meta-analysis assessing the prevalence of HT in PTL, 79% of patients with PTL had evidence of HT and interestingly, the prevalence of HT was higher for MZL than in DLBCL.…”

“…The major sites of extranodal manifestations are blood, bone marrow, gastrointestinal tract, and lungs 2 . Notably, thyroid gland involvement is uncommonly reported, with only four case reports in the literature 3‐6 . Herein, we present two MCL patients with thyroid gland involvement on presentation.…”

“…2 Notably, thyroid gland involvement is uncommonly reported, with only four case reports in the literature. [3][4][5][6] Herein, we present two MCL patients with thyroid gland involvement on presentation.…”

Mantle Cell Lymphoma of Mucosa‐Associated Lymphoid Tissue: A European Mantle Cell Lymphoma Network Study

“…Involvement of more than 2 extranodal sites occurs in 30% to 50% of patients. However, isolated extranodal disease (ED) is rarely detected in cMCL at diagnosis and only a few reports are available: therefore, in everyday clinical practice it is not possible to define the optimal treatment strategy 11,12 …”