Introduction and importancePrimary Mucosa-associated lymphoid tissue (MALT) lymphoma of the thyroid is a rare tumor. Presentation of caseA previously well male in his 50s presented to our institution with difficulty in breathing and sleep apnea. He was diagnosed with a large retrosternal multinodular goiter with level 2 unilateral cervical lymphadenopathy. Fine needle aspiration cytology of the thyroid revealed chronic thyroiditis and the enlarged lymph node cytology was inconclusive. He underwent total thyroidectomy and level VI bilateral cervical lymph node clearance. The histology revealed an extra-nodal marginal zone lymphoma of MALT. A whole-body CT scan did not demonstrate any other primary site. The patient received 4 cycles of local radiotherapy. Subsequently, he was diagnosed with a brain tumor not amenable to surgical interventions following persistent headaches. He died shortly after due to complications of probable cerebral metastasis. Case discussionMALT lymphomas of the thyroid carry a good prognosis; however, no universal guidance exists regarding the optimal therapy and follow-up. ConclusionThis case report highlights the importance of early diagnosis, identification of poor prognostic factors, and patient-tailored therapy and follow-up.

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