Severe Respiratory Compromise Due to Primary Thyroid Lymphoma

Primary thyroid lymphoma is a rare condition making up 5% of thyroid malignancies and 2.5% of all lymphomas. Most primary thyroid lymphomas are diffuse large B-cell lymphoma, which is the most aggressive type.

Case Description :

An 87-year-old female with a past medical history of hypertension, aortic insufficiency, congestive heart failure, chronic obstructive pulmonary disease, Hashimoto’s thyroiditis on levothyroxine, and a prior benign thyroid nodule presented to her primary care doctor with cough, congestion, and shortness of breath. She was diagnosed with pneumonia and treated with azithromycin with improvement. However, her shortness of breath continued to progressively worsen for several weeks until she was admitted. On physical exam, inspiratory stridor was noted, but no thyromegaly or thyroid nodules were palpated. CT neck showed a large thyroid mass with erosion into trachea causing significant narrowing. She was transferred to ICU for higher level of care. Her labs were unremarkable except for a TSH of 6.35 and free T4 of 1.03. A repeat CT neck done showed a large 5.2cm thyroid mass extending to the anterior portion of the larynx, consistent with thyroid cancer per radiology. A CT chest and head was negative for metastatic disease. She was started on bronchodilators and IV steroids. ENT was consulted and performed a tracheostomy with a partial thyroid resection for pathologic evaluation. The patient’s respiratory status improved afterwards. The pathology was consistent with diffuse large B-cell lymphoma, with CD45, CD20, CD10, CD3, PAX5, BCL6, P53, and K1-67 being positive. Bone marrow aspirate and biopsy showed no evidence of B-cell lymphoma.  Oncology was consulted and recommended outpatient chemotherapy with radiation. The patient was discharged to a skilled nursing facility in the meantime, but then had a hip fracture after a fall. She was readmitted for a possible orthopedic procedure but due to recurrent decompensations in respiratory status, she was deemed to not be a good surgical candidate. She was started on weekly Rituximab and IV steroids per oncology, but she continued to deteriorate to where she and her son decided to transition to hospice care. She died due to respiratory compromise contributed by the primary thyroid lymphoma.

Discussion :

This case illustrates the importance of early diagnosis of primary thyroid lymphoma. Although diffuse large B-cell lymphoma of the thyroid gland seen in this patient is rare, it is more likely to be seen in older aged females with Hashimoto’s thyroiditis or other autoimmune disorders. This case demonstrates the importance of regularly monitoring patients with Hashimoto’s thyroiditis for early detection and treatment of diffuse large B-cell lymphoma of the thyroid before significant airway compromise and clinical deterioration occur.