EP108 – Aggressive disease course of papillary thyroid cancer arising in thyroglossal duct cyst

      Zund, Santiago1; Giannini Natalia1; Califano, Inés2; Carrizo Fernando3 1 Department of Head & Neck Surgery, Roffo Cancer Center, University of Buenos Aires, Buenos Aires, Argentina 2 Department of Endocrinology, Roffo Cancer Center, University of Buenos Aires, Buenos Aires, Argentina 3 Department of Pathology, Roffo Cancer Center, University of Buenos Aires, Buenos Aires, Argentina   Background / Purpose: Papillary thyroid cancer (PTC) occurs in 0.7-1.6% of thyroglossal duct cysts (TDC). Specific mortality is extremely rare. Our aim is to report a case with torpid course and fatal outcome. Methods: Patient data was obtained from clinical chart. Results: A 51-year old female showed an upper-neck midline mass. Both ultrasound and CT scan showed thyroid nodules and a solid, calcified 64×56 mm mass, involving the hyoid bone. Fine needle aspiration cytology was inconclusive. A tru-cut biopsy was performed, yielding PTC. The patient underwent Sistrunk procedure, total thyroidectomy and bilateral central neck dissection. Pathology report confirmed multifocal PTC in TDC and thyroid. Stage:IVa T4a N1a M0, with ki67 of 15%. She received 150 mCi of radioiodine; with post dose uptake in thyroid bed. Five months later, CT showed a cervical mass and lung metastases. Resection of the cervical lesion confirmed PTC. She received 200 mCi of radioiodine; post dose scan showed uptake only in the neck. The patient was classified as radioactive iodine (RAI) refractory. After pulmonary progression, sorafenib (800 mg/day) was given. Eight months later, lung, pleural and bone progression was noted. The patient died of respiratory insufficiency 4 years after initial diagnosis. Discussion: PTC arising in TDC is rare and usually carries an excellent prognosis. Adverse prognostic features are advanced age, tumoral size, extrathyroid extension and distant metastases. Multifocal disease (both in TDC and thyroid) is reported in 25% of the cases, and recurrence rates are 14-30%. RAI refractoriness is not specified in the literature. High expression of ki67 index reflects elevated proliferating activity, as was confirmed by the rapid clinical progression.   References:
    1. Wei S, Livolsi V, Baloch Z. Pathology of Thyroglossal Duct. An Institutional Experience. Endocr Pathol 2015;26:75-79.
    2. Zizic M, Faquin, W, Stephen, AE, Kamani D, Nehme R, et al. Upper Neck Papillary Thyroid Cancer (UPTC): A New Proposed Term for the Composite of Thyroglossal Duct Cyst-Associated Papillary Thyroid Cancer, Pyramidal Lobe Papillary Thyroid Cancer, and Delphian Node Papillary Thyroid Cancer Metastasis. Laryngoscope 2016;126(7):1709-14.
    3. Hartl D, Ghuzlan A, Chami L, Leboulleux S, Schlumberger M, et al. High Rate of Multifocality and Occult Lymph Node Metastases in Papillary Thyroid Carcinoma Arising in Thyroglossal Duct Cysts. Ann Surg Oncol 2009;16:2595-2601.
    4. Aghaghazvini L, Mazaher H, Sharifian H, Shirin Aghaghazvini S, Assadi M. Invasive thyroglossal duct cyst papillary carcinoma: a case report. J Med Case Rep. 2009 Dec 1;3:9308.
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