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  • • Often history of hypothyroidism, specifically Hashimoto thyroiditis

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Epidemiology

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  • • 2.2-2.6% of all cases of non-Hodgkin lymphoma

    • Incidence of 0.5-1/100,000

    • Approximately 2% of all thyroid malignancies

    • Strong female prevalence

    • Median age of onset is late seventh decade

    • Hashimoto thyroiditis is frequent precursor

    • Vast majority are B-cell type

    • 42-60% of patients present with stage IE disease

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Symptoms and Signs

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  • • Rapidly enlarging neck mass

    • Hoarseness

    • Stridor

    • Dysphagia, odynophagia

    • Thyroid is firm and fixed

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Rule Out

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  • • Anaplastic thyroid carcinoma

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  • • History and physical exam

    • Needle biopsy or open biopsy

    • Full staging with neck, chest, abdomen, and pelvis CT

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  • • Little role for surgical therapy except for incisional biopsy

    • Multiple agent chemotherapy and radiation therapy

    • Radiation therapy can abrogate airway compromise

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Surgery

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Indications

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  • • Tracheostomy if airway compromise

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Medications

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  • • Multiple agent chemotherapy

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Prognosis

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  • • Poor; predicted by dysphagia, hoarseness, initial tumor size > 10 cm, and stage at presentation beyond IE

    • 5-year survival for stage IE is 80%

    • 5-year survival for stage IIE is 50%

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Practice Guidelines

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