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Case Report Thyroid Disorders A Case of a Rare Association Between Right Thyroid Hemiagenesis, Lingual Thyroid, and Papillary Thyroid Cancer Umal Azmat, 1 John E Phay, 2 Theodoros Teknos, 2 James Bekeny, 3 and Fadi Nabhan 2 1. Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, Pakistan; 2. Division of Endocrinology, Diabetes, and Metabolism, Department of Surgery, Division of Surgical Oncology, Department of Otolaryngology, Ohio State University and Arthur G James Cancer Center, Columbus Ohio, US; 3. Florida Hospital Medical Group, Maitland, Florida, US L ingual thyroid and thyroid hemiagenesis are rare thyroid developmental abnormalities. These conditions can be present with other thyroid diseases, whether functional ones involving abnormal thyroid hormone levels, or structural ones such as presence of thyroid nodules, however the association with thyroid cancer and lingual thyroid or thyroid hemiagenesis is rare. In addition to that, when thyroid hemiagenesis is present, it is usually in the left lobe. We describe here a pregnant patient who presented with metastatic cervical nodes from thyroid cancer who was found on imaging to have right thyroid hemiagenesis. After she underwent an initial surgery for thyroid cancer and metastatic nodal disease, she was then later discovered to have a lingual thyroid. In this case we report this extremely rare combination and also discuss the potential implications of this association on the diagnosis and management of these patients particularly initial surgery and imaging studies needed for the thyroid cancer. Keywords Case presentation Thyroid hemiagenesis, lingual thyroid, thyroid cancer, ectopic thyroid, congenital thyroid abnormality, thyroid nodules A 32-year-old woman presented to our clinic while three months pregnant, with a left-sided neck mass that she had felt about 10 months prior to presentation. She noticed that the mass had grown slightly over that period. She denied symptoms of dysphonia, dyspnea, and dysphagia. A neck ultrasound showed an absent right thyroid lobe and isthmus, along with several left thyroid nodules, with the largest being 4.3 cm and left lateral malignant appearing adenopathy (largest 3.6 cm). Fine-needle aspiration biopsy of the left lateral lymph node was consistent with papillary thyroid carcinoma. The patient had normal thyroid function tests. During her 20th week of pregnancy, the patient underwent a left hemithyroidectomy, left central neck dissection and left lateral neck dissection including levels II–IV. Pathology revealed multifocal papillary thyroid carcinoma with largest focus at 5.2 cm with extensive vascular invasion and focal extrathyroidal extension. Five out of 12 lymph nodes were malignant (one in central neck and four in left lateral neck). After delivery of a healthy baby, the patient continued to have detectable thyroglobulin (Tg) at 11.5 ng/ml (Immulite 2000 XPi Thyroglobulin assay, Siemens Inc., Deerfield, IL, which has a functional sensitivity of 0.9 ng/mL) with undetectable anti-Tg antibodies with a thyroid stimulating hormone (TSH) level of 0.156 uIU/ml and ultrasound examination noted a left level II malignant-appearing lymph node. Neck computed tomography (CT) scan confirmed that and that no other suspicious nodes were present, however, it indicated the presence of a lingual thyroid (see Figure 1). The patient did not want to have re-operation or other additional intervention at that time, and she became pregnant again. After delivery and throughout the following year, she continued to have detectable Tg levels at 89.1 ng/ml with TSH of 9.3 uIU/ml, then 16.4 ng/ml with TSH of 0.075 uIU/ml and then 72 ng/ml with TSH of 86 uIU/ml (all these Tg levels were done using the assay mentioned above). A year after she delivered, she agreed to have re-operation that included left lateral neck dissection along with removal of a benign lingual thyroid (see Figure 2). The patient did not agree to receive radioactive iodine treatment. Her most recent thyroglobulin level was done one month after this last surgery and it was at 2.3 ng/ml (this time the Tg assay was changed to Beckman Access Tg, Beckman Coulter, and was performed at Mayo Clinic Laboratories, Rochester, MN, US, with a functional sensitivity at 0.1 ng/ml) with undetectable anti-Tg antibodies with TSH of 0.09. Disclosure: Umal Azmat, John E Phay, Theodoros Teknos, James Bekeny, and Fadi Nabhan have nothing to declare in relation to this article. No funding was received in the publication of this article. Compliance with Ethics: All procedures were followed in accordance with the responsible committee on human experimentation and with the Helsinki Declaration of 1975 and subsequent revisions, and informed consent was received from the patient involved in this case study. Authorship: All named authors meet the International Committee of Medical Journal Editors (ICMJE) criteria for authorship of this manuscript, take responsibility for the integrity of the work as a whole, and have given final approval to the version to be published. Open Access: This article is published under the Creative Commons Attribution Noncommercial License, which permits any noncommercial use, distribution, adaptation, and reproduction provided the original author(s) and source are given appropriate credit. Received: October 19, 2016 Accepted: February 6, 2017 Citation: US Endocrinology, 2017;13(1):35–6 Corresponding Author: Fadi Nabhan, Ohio State University Medical Center, Division of Endocrinology, Diabetes and Metabolism, 575 McCampbell Hall, 1581 Dodd Dr, Columbus, OH 43210, US. E: firstname.lastname@example.org TOU CH MED ICA L MEDIA 35