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A Not-So-Simple Thyroid Nodule

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 50-year-old woman presented with a 2-month history of a neck mass that was associated with hoarseness of voice and globus sensation. Physical examination showed thyromegaly (right greater than left) with an approximately 5-cm palpable nodule in the right lobe. There was no tenderness or cervical lymphadenopathy. There was no prior radiation exposure to the head and neck area. Thyroid function test results were normal, and she did not take thyroid medications. A relative had received a diagnosis of papillary thyroid cancer at age 50 years. There was no other pertinent family history, including a history of multiple endocrine neoplasia. Ultrasonography results showed a multinodular goiter with a dominant 4.6-cm right thyroid nodule, 1.6-cm left-sided thyroid nodule, and suspect left level IV lymph node. Ultrasonography-guided fine-needle aspiration biopsy results showed papillary thyroid carcinoma (PTC) (Bethesda VI) with a BRAFV600E variation in the left nodule and lymph node, and atypia of undetermined significance (Bethesda III) with a suspicious Afirma genomic sequence classifier in the right nodule. She underwent total thyroidectomy with modified left lateral neck dissection. Gross examination of the specimen showed a 2.9-cm pale-tan, partially circumscribed, soft nodule in the right thyroid lobe; a 1.4-cm pale-tan, fibrotic, centrally brown nodule in the left lobe; and a 1.8-cm pale-tan, rubbery, partially circumscribed nodule in the isthmus. Histologic examination of the right thyroid lesion was performed (Figure), with ancillary testing.

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D. Thyroid paraganglioma

Because of their rarity and overlapping features with other common thyroid entities, thyroid paragangliomas (TPGLs) are rarely suspected on fine-needle aspiration cytology. Although histopathologic assessment can render a definite diagnosis in most cases, immunohistochemistry may be helpful, especially in cytology specimens. In this patient, distinct from the PTCs in the left lobe, isthmus, and lymph node, the right-sided nodule showed an organoid zellballen pattern. The tumor cells showed amphophilic granular cytoplasm, round to oval nuclei, and salt-and-pepper chromatin and were positive for synaptophysin but negative for AE1/AE3, thyroid transcription factor–1 (TTF-1), thyroglobulin, paired-box gene 8, monoclonal carcinoembryonic antigen, and calcitonin. Intact succinate dehydrogenase (SDH) B expression was detected by immunohistochemistry. Focal sustentacular cells were highlighted by an S100 immunostain. These findings confirmed the diagnosis of TPGL.

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Article Information

Corresponding Author: Liwei Jia, MD, PhD, Department of Pathology, UT Southwestern Medical Center, 6201 Harry Hines Blvd, Room UH04.233, Dallas, TX 75390-9234 (liwei.jia@utsouthwestern.edu).

Published Online: September 2, 2021. doi:10.1001/jamaoto.2021.2155

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information as well as Justin Bishop, MD, University of Texas Southwestern Medical Center, who originally worked up and signed out the pathologic diagnosis on this case.

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