Thyroid Lymphoma: Recent Advances in Diagnosis and Optimal Management Strategies

Learning Objectives Explain the diagnostic modalities used to diagnose primary thyroid lymphoma. Describe the role of the endocrine surgeon in the diagnosis and treatment of thyroid lymphoma. Cite the recent advances in the treatment of primary thyroid lymphoma. Primary thyroid lymphoma is rare, composing approximately 5% of all thyroid malignancies and less than 3% of all extranodal lymphomas. It typically presents as a rapidly enlarging goiter with associated compressive symptoms. Thyroid ultrasound and fine needle aspiration cytology, using flow cytometry and immunohistochemistry, remain the main modalities used to confirm the presence of lymphoma. The increasing use of an ultrasound‐guided core biopsy to achieve an accurate diagnosis has further limited the role of surgery. An open surgical biopsy may still be required not only for definitive diagnosis but also to confirm the subtype of lymphoma. There are limited numbers of randomized or prospective trials to guide management, and controversy remains over optimal treatment. Treatment and prognosis of this disease can be dichotomized into two separate groups: pure mucosa‐associated lymphoid tissue (MALT) lymphoma and diffuse large B‐cell lymphoma (DLBCL) or mixed subtypes. Early stage (stage IE) intrathyroidal MALT lymphomas typically have an indolent course and may be treated with single‐modality surgery, radiotherapy, or a combination of both. DLBCLs are more aggressive, and survival outcomes are highest with multimodal therapy incorporating monoclonal antibodies, chemotherapy, and radiotherapy. The prognosis is generally excellent but can be varied because of the heterogeneous nature of thyroid lymphomas. The aim of this paper is to discuss the changes in diagnostic modalities and to focus on the recent alterations in the management of this rare disease, including targeted therapies as well as the more limited role of the endocrine surgeon. 摘要 原发性甲状腺淋巴瘤很罕见，大约占所有甲状腺恶性肿瘤的5%，所有结外淋巴瘤的3%。其典型表现为迅速增大的甲状腺肿块和相应的压迫症状。甲状腺超声和细针穿刺活检是确认存在淋巴瘤的主要方式。超声引导下的空心针活检可获得准确的诊断，该方法日益增加的应用进一步局限了手术的作用。但明确诊断和明确淋巴瘤的亚型仍然可能需要开放手术活检。由于指导处理方法的随机或前瞻性研究数量有限，目前在最佳治疗上仍然存在争议。疾病治疗和预后可以分为两个独立小组：纯粘膜相关淋巴组织（MALT）淋巴瘤和弥漫大B细胞淋巴瘤（DLBCL）或混合亚型。早期（IE期）甲状腺内MALT淋巴瘤具有典型的惰性病程并能单用手术、放疗或两者结合的方式治疗。DLBCL更具侵袭性，联合单克隆抗体、化疗和放疗的多重模式治疗的生存结局最佳。该病预后通常良好，但由于甲状腺淋巴瘤的异质性，预后是多变的。本文的目的是讨论诊断方式的改变并关注这一罕见疾病近期处理的改变，包括靶向治疗和作用越来越有限的内分泌外科治疗。The Oncologist 2013;18:994‐1003 Primary thyroid lymphoma is rare, and there are limited numbers of randomized or prospective trials to guid