bluuebunny/crossref_metadata_2025_split huggingface dataset:
MON-497 Evaluation and Comparison of Ultrasonographic Features According to Histology of Thyroid Nodules
Authors: Ferrinho Cátia , Cunha Clara Valério , Silva Eugénia , Saraiva Catarina , Ferreira Rute Costa , Roque Catarina , Chorão Martinha , Oliveira Manuela , Duarte João Sequeira 
Published: May 2020

Abstract
Introduction: The prevalence of thyroid nodules identified by ultrasonography (US) may be up to 70%, with the majority being incidentalomas. Less than 15% of thyroid nodules will be malignant. Ultrasonography plays a key role in the diagnosis and management of thyroid diseases and US features have been described in way to predict the risk of malignancy. Aim: Evaluate and correlate US features with histological results, in our hospital, from January 2017 to December 2018. Materials and methods: Cross-sectional study. A total of 117 patients were evaluated, 77 had histology of nodular hyperplasia (benign) and 40 had histology of thyroid carcinoma (malign) after partial or total thyroidectomy. We described the size of the nodule (the largest diameter), the US features detailed in the ultrasound report (shape, margins, echogenicity, composition and microcalcifications), the EU-TIRADS score of each nodule and the histology of thyroid. The data were analysed using nonparametric statistical tests. The results are presented as median (interquartile range). The level of significance accepted was p&lt;0.05. Results: Of the 117 patients, 82.1% were female, with a median age of 59.7 (25.0) years, nodule size in benign histology group was 35.0 (14.0) mm and in malign histology group was 20.0 (28.0) mm. Of malignant nodules, 90.0% (n=36) were papillary carcinomas. The prevalence of US features: 76.9% were solid, 35.0% had microcalcifications, 25.6% had irregular margins, 17.9% were markedly hypoechoic and 6.8% were taller-than-wide. Three characteristics were significantly related to malignancy: irregular margins (p=0.001), markedly hypoechoic (p=0.001) and microcalcifications (p=0.015). The taller-than-wide feature had the highest specificity of 94.8%; the marked hypoechogenicity with a specificity of 92.2%, had the highest positive likelihood ratio (LHR+: 4.81 (95% CI: 2.02–11.44)) and the highest diagnostic odds ratio (DOR: 7.10 (95% CI: 2.48–20.30)). The EU-TIRADS 5 category had a significant association with malignancy (p=0.001) and 80.0% of malignant nodules were classified in this category. The specificity was 62.3%, LHR+ was 2.12 (95% CI: 1.53–2.94) and DOR was 6.62 (95% CI: 2.69–16.31). Conclusion: In the evaluation of the US features, marked hypoechogenicity had the highest LHR+ and DOR. It was possible to verify that the EU-TIRADS 5 category was a good predictor of risk to malignancy, which reinforces the importance of categorizing the nodules for selective perform of fine needle aspiration.
SUN-275 A Rare Mutation in the TBX19 Gene Leading to Isolated ACTH Deficiency in Two Siblings
Authors: Albers Norbert , Bartels Amelie , Peters Renate , Banzer Jens 
Published: May 2020

Abstract
Hypoglycemia and hyperammonemia (&gt;500µg/dl) in a newborn led to the suspicion of THAN (transient hyperammonemia of the newborn). Subsequently, hypoglycemic and salt losing episodes with low cortisol (&lt;0,1 ug/dl) and ACTH (&lt;0,16 pg/ml) levels pointed to ACTH deficiency. Genetic analysis showed a homozygous mutation c.302G&gt;A for p.(Trp101*) in the TBX19 gene (a positive regulator of the transcription of POMC and the terminal differentiation of the corticotrophs), generating a premature stop codon. This mutation has been described only once and very recently by Abali et al (Hormones 18:229; 2019) in a 4 year old girl, but unlike our patients, this girl was obviously unaffected during her neonatal period. All other pituitary axes in our patient were normal, thus congenital isolated ACTH deficiency was the final diagnosis. Hyperammonemia resolved spontaneously and the suspected diagnosis of THAN could be dismissed. Hyperammonemia had probably been due to metabolic stress.After 16 months, a younger brother was born and showed hypoglycemia, hypotension and respiratory infection during his neonatal period. Cortisol and ACTH levels were also very low, thereafter, the same TBX19 mutation was detected.Both brothers were successfully treated with oral hydrocortisone substitution (6–10 mg/sqm/day q8 with increases during stress) and thrive well, except for several infections of the upper respiratory tract in the younger brother. In summary, we report the very rare condition of familial isolated congenital ACTH deficiency with a mutation of TBX19 that has never been described in newborns. Initial presentation may be accompanied by confounding pathological lab findings, while genetic analysis together with extremely low ACTH and cortisol levels confirm the correct diagnosis.
SUN-481 A Coexisting Primary Papillary Thyroid Carcinoma in a Case of Malignant Struma Ovarii with Follicular Thyroid Carcinoma: 2 Types of Thyroid Cancer in the Same Patient
Authors: Elshimy Ghada , Bhattarai Richa , Tran Kelvin , Correa Ricardo Rafael 
Published: May 2020

Abstract
Introduction: Struma ovarii is a rare monodermal variant of ovarian teratoma diagnosed when thyroid tissue is more than 50% of the overall tissue. It represents only 1% of all ovarian tumors. The vast majority of struma ovarii are benign (95%); however, malignant tumors have been reported in a small percentage of cases. The most common type is papillary carcinoma(PTC), followed by typical follicular carcinoma found in the pathology after surgical oophorectomy. We report a case of malignant struma ovarii with follicular carcinoma in the setting of additional micro PTC found after total thyroidectomy. Case report: A 48-year-old female presented with right-sided pelvic pain and a mobile pelvic mass. Pelvic MRI showed noted a large complex solid mass with cystic components in the right adnexa. It measured 7.7 x 8.4 x 6.7 cm. Subsequently, the patient underwent a robotic hysterectomy, bilateral salpingo-oophorectomy (TAHBSO), bilateral pelvic and para-aortic lymph node sampling, omentectomy and resection of nodules. Final pathology noted an ovary with struma ovarii with well-differentiated follicular carcinoma, peritoneal nodules containing thyroid tissue and benign lymph nodes. Subsequently, iodine 123 SPECT CT showed physiologic uptake in the thyroid with increased uptake in the pelvis, right perirectal region indicating residual thyroid tissue. The patient underwent total thyroidectomy with radioactive ablation with I131 (154.7 mCi). Pathology detected incidental 0.5mm micro PTC and it was classified as staged pT1aN. 6 months postoperatively, thyroglobulin (Tg) and Tg antibodies were undetectable with no abnormalities found on repeat whole-body scans. The patient has been following up with no new issues for the past 5 years indicating a good prognosis with low risk of recurrence. Discussion and Conclusion: In the literature, Struma ovarii containing thyroid-type carcinoma and papillary or follicular thyroid carcinoma metastasizing to the ovary has been documented. The standard treatment of a patient with malignant struma ovarii is TAHBSO and complete surgical staging, including peritoneal washings for cytology, pelvic and para-aortic lymph node sampling, and omentectomy. In cases with the residual malignant disease after surgery, total thyroidectomy and radioactive ablation are recommended. Our case is a unique case given the presence of 2 different thyroid carcinoma in the same patient. In addition, our patient had multiple risk factors for recurrence including large lesions&gt;4 cm, extra ovarian extension, and the coexisting synchronous primary thyroid cancer, however, she had a good prognosis with no recurrence during the 5 years follow up period.
MON-298 IGF-1 in the Early Post-operative Assessment of Acromegaly
Authors: Donegan Diane , Algeciras-Schimnich Alicia , Erickson Dana Z 
Published: May 2020

Abstract
Introduction: Transsphenoidal surgery is recommended as first line therapy in the vast majority of patients with acromegaly. Successful surgical intervention is determined by normalization of insulin like-growth factor (IGF) - 1 and adequate suppression of growth hormone (GH) during glucose GH suppression test or random GH levels &lt;1.0 ng/mL. Given the long half-life of IGF-1, evolution of IGF-1 and GH assays and limitations of GH suppression test, clinicians remain reliant on IGF-1 results 3–6 months following surgery to assess disease remission following surgical intervention. This can lead to delayed treatment of patients with persistent disease and significantly increase patient anxiety.
Aim: To determine if IGF-1 levels at 6 weeks were equally predictive of surgical outcomes when compared to IGF-1 levels at 3–6 months postoperative.
Methods: Retrospective review of patients with newly diagnosed acromegaly who had surgery between 2010–2019 and had post-operative IGF-1 level measured at 6 weeks and 3–6 months. IGF-1 was measured using the Siemens Immulite assay until 2016 when it was replaced by a LC-MS/MS assay. IGF-1 measurements at 6 weeks are obtained based on clinician discretion.
Results: 69 patients (mean age 49 ± 14.8, female 31/69, 44.9%) with acromegaly had surgery and IGF-1 follow-up measurements at 6 weeks and 3–6 months. Persistent acromegaly was noted in 45/69 at 3–6 months. The median IGF-1 pre-operative was 701 ng/ mL (289–1600), 6 weeks postoperative was 286 ng/ mL (109–1038) and at 3–6 months was 267 ng/ mL (77–996). The median pre-operative IGF-1 was significantly higher than the median IGF-1 level at 6 weeks and 3–6 months (P=&lt;0.001). Although a statistically significant difference was seen between IGF-1 levels at 6 weeks and 3–6 months, the mean difference between these time points was small (20 ng/mL, P=&lt;0.001). There was a greater percent reduction in IGF-1 at 6 weeks following surgery in those who had a normal IGF-1 at 3–6 months compared to those who did not (63% vs 35%, P=&lt;0.01). Among patients who were deemed in remission at 3–6 months (24/69, 35%), 3/24 patients (all male with macroadenoma and no cavernous sinus invasion) had mildly elevated IGF-1 levels at 6 weeks (≤1.12 upper limit of normal) which subsequently normalized. In all of these patients, GH was &lt;1 ng/mL post-op. In 1 male (macroadenoma and cavernous sinus invasion), IGF-1 levels increased from normal at 6 weeks to abnormal when measured at 3–6 months.
Conclusion: In the vast majority of patients, 6 weeks post-operative IGF-1 can be used to assess response to surgery avoiding a delay in treatment. However, we would recommend repeating IGF-1 levels at 3–6 months in those who have marginally elevated IGF-1 above normal (≤ 1.12), without cavernous sinus invasion and a post-operative GH of &lt;1 ng/mL prior to intervening if the IGF-1 is elevated 6 weeks as the IGF-1 may normalize.
SUN-158 Testosterone and Cortisol Secreting Large Adreno- Cortical Adenoma
Authors: Madhavan Parvathy , Sukumaran Neenu , Luthra Pooja 
Published: May 2020

Abstract
Background: Functional adrenal adenomas most commonly secrete cortisol or aldosterone. Secretion of multiple hormones is commonly seen in adrenocortical carcinomas and is rare in benign adrenal adenomas. We present a case of a patient with a 6 cm benign adrenal adenoma that was secreting both cortisol and testosterone.
Clinical case: A 44-year-old Caucasian female with past medical history of Grave’s disease, hypertension, prediabetes and hyperlipidemia presented with hirsutism and weight gain. Physical exam was suggestive of cushing’s disease with elevated blood pressure, hirsutism, supraclavicular fullness, moon facies, facial plethora, dorsocervical fat pad and abdominal striae. Further workup revealed elevated testosterone level of 152ng/dL (9-55ng/dl) and elevated androstenedione level of 2.520ng/ml (0.130–0.820 ng/ml). DHEA 0.741 ng/ml (0.630–4.7ng/ml) and DHEAS 65ug/dl (32-240ug/dl) were both within reference range. The 24-hour urine free cortisol was also elevated at 107.1ug/gram of creatinine (&lt; 24 ug/gram of creatinine) with an undetectable ACTH level of &lt; 5 pg/ml indicating adrenal source of Cushing’s syndrome. CT abdomen and pelvis with and without contrast revealed a 6.3 x 5.2 x 5.6 cm left adrenal mass which measured 22 Hounsfield units (HU) on non-contrast imaging, 85 HU on 70 second imaging and 48 HU on 15 min delayed phase. Calculated percent relative washout was 43.5 % and absolute washout was 59.4 %. There was another 1.1 cm low density nodule on left adrenal genu. The right adrenal gland also had a 1.2 cm low density nodule with 0 HU on all phases compatible with a lipid rich adenoma. Patient then underwent left open adrenalectomy. Pathology revealed a 6 cm adrenocortical adenoma. There was no tumor invasion into adjacent sites, no vascular invasion or tumor necrosis noted. MIB1 index was &lt;1 %. Patient had resolution of hypertension and prediabetes after the surgery. Her cortisol and androgen levels also normalized. Biochemical follow up 6 months post-surgery has not demonstrated any increase in cortisol or androgen levels.
Conclusion: Both androgen and cortisol hypersecretion can cause severe hyperandrogenism and virilization but these hormones are commonly secreted in isolation in functional adrenal adenomas. Adrenal masses co-secreting androgen and cortisol are usually indicative of adrenocortical cancer. In this rare case report, both of these hormones were secreted by a unilateral adrenal adenoma, with resolution of symptoms and normalizing of androgen and cortisol levels post left adrenalectomy.
MON-441 Primary Thyroid Maltoma: A Rare Non-hodgkin Malignancy
Authors: Abuaisha Munder , Gibbs Otto , Saand Mariam , Bier Rachel 
Published: May 2020

Abstract
Introduction: Thyroid lymphoma is a rare malignancy; only 2% of extranodal lymphomas arise within the thyroid gland. Furthermore, lymphomas represent no more than 2% of all malignant thyroid tumors and almost always non-Hodgkin type. Case: A 58-year-old female with a medical history of rheumatoid arthritis treated with Golimumab for years. Exam revealed a right thyroid lobe enlargement with bilateral palpable nodules. Labs include TSH of 1.4 mU/L, Free T4 of 1.76 ng/dL, normal anti-thyroid peroxidase antibodies, but elevated anti-thyroglobulin. US of the thyroid showed right-sided, hyperechoic nodule measuring 1.3cm, and left-sided solid hypoechoic nodule measuring 2.2cm. US-guided FNA of the right nodule yielded atypical cells of undetermined significance (2017 Bethesda category III). FNA of the left nodule showed severe, chronic thyroiditis, with small clonal population of cells and suspicion for lymphoma. She underwent total thyroidectomy with left central lymph node dissection. Microscopic evaluation showed a predominant lymphocytic population with plasmacytoid/plasma cells. The histological finding was consistent with extranodal marginal zone lymphoma (EMZL) of mucosa-associated lymphoid tissue (MALT lymphoma or MALToma). However, thyroid tissues were negative for clonal immunoglobulin gene rearrangements (both immunoglobulin heavy and light chains). Immunohistochemistry demonstrated that the tumor cells were CD19, CD 20, CD 22, PAX5, CD78A positive, highlighting predominantly small to medium-sized B- cell lymphocytes, along with plasmacytoid/plasma cells (MUM1 and CD 75A positive). Furthermore, occasional reactive germinal centers (CD10, BCL6 positive, BCL2 negative) were noted. In conclusion, these features were consistent with a low grade, non-Hodgkin’s lymphoma of MALT type. Discussion: EMZL is a clinically indolent non-Hodgkin lymphoma, and growing evidence suggests that numerous cases originate in the background of chronic immune stimulation. MALToma is associated with Hashimoto’s thyroiditis or other immune diseases. Whereas half of EMZL can still arise without a Hashimoto thyroiditis background. Characteristics of the MALToma include reactive follicles, with the neoplastic cells invading the marginal zone (small lymphocytes, marginal zone B-cells, and plasma cells). The key findings are the presence of clonality (light chain restriction), and confirmation of B-cell origin (presence of B-cell markers). The immunophenotype is confirmed by immunohistochemistry and flow cytometry. Localized EMZL of the thyroid can be effectively treated with radiation alone. Surgery is usually used for diagnostic biopsy only. Chemotherapy is usually reserved for those with advanced stages
MON-082 The Neonatal Screen That Cried Wolff
Authors: D’Ambrosio Fabiola , Nur Laika M , Mantis Stelios , Minutti Carla Z 
Published: May 2020

Abstract
Introduction:
Hypothyroidism is one of the major causes of preventable mental retardation. Neonatal screening aids in the prompt diagnosis of newborns with congenital hypothyroidism. There are other clinical conditions that can alter thyroid function during the newborn period, including exposure of high iodine concentrations.
Case Presentation:
One day old female born at 37 3/7 weeks of gestational age by C-section with imperforated anus and congenital heart disease was transferred to our children’s hospital within the first day of life for a hybrid cardiac procedure of bilateral pulmonary artery banding and PDA stenting. She had an Illinois Neonatal screen done at 36 hours of life that was normal. Her cardiac surgery was performed at 10 days of life, where she was exposed to iodine products transdermally. At 14 days of age, she had a repeat Illinois Neonatal screen that was positive for congenital hypothyroidism with a TSH of 78 mIu/mL (normal &lt; 20 mIu/mL) and reflex total T4 of 5.4ug/dL (normal is &gt; 8ug/dL). No family history of thyroid disease; mother was healthy during pregnancy and was not on medications that could affect baby’s thyroid function. Subsequent serum laboratory testing confirmed a TSH of 74.3mIu/mL and Free T4 of 0.6ng/dL. Patient was diagnosed with Wolff-Chaikoff effect, which is the phenomenon of transient hypothyroidism caused by exposure to high doses of iodine (iodine containing contrast agents or topical antiseptics). Pediatric Endocrinology was consulted at 2 weeks of life and she was started on 25mcg of levothyroxine PO daily. Levothyroxine dose decreased at 16 month of age to 12.5mcg due to stable thyroid function tests. The patient was last seen at 30 months of age by Pediatric Endocrinology. She is still on the low dose of levothyroxine and her thyroid labs have been within normal limits for an infant. She will likely not require lifelong thyroid supplementation.
Conclusion: Risk of hypothyroidism among neonates must be considered seriously after large iodine exposure and monitoring for transient hypothyroidism should be performed. It is thus recommended that attempts should be made to reduce the amount of iodine used during procedures and to carefully monitor thyroid function in all neonates exposed to an excess of iodine.
Reference:
Markou, K., et al. “Iodine-Induced Hypothyroidism.” Thyroid, vol. 11, no. 5, 2001, pp. 501–510., doi:10.1089/105072501300176462.
Linder N, Sela B, German B, et al. Iodine and hypothyroidism in neonates with congenital heart disease. Archives of Disease in Childhood - Fetal and Neonatal Edition 1997;77:F239-F240.
Kovacikova, Lubica, et al. “Thyroid Function and Ioduria in Infants after Cardiac Surgery: Comparison of Patients with Primary and Delayed Sternal Closure.” Pediatric Critical Care Medicine, vol. 6, no. 2, 1 Mar. 2005, pp. 154–159., doi:10.1097/01.pcc.0000154960.59452.06.
SUN-026 Unusual Presentation of Aromatase Excess Syndrome
Authors: Al Sabie Farah Laith , Ghareebian Hagop , Mariash Cary N 
Published: May 2020

Abstract
Introduction: Aromatase excess syndrome is a rare disorder with gynecomastia being the main symptoms. Its prevalence is unknown with approximately twenty cases reported. We describe an unusual case of Aromatase excess syndrome. It was diagnosed incidentally at a much older age than expected while evaluating for hypersomnia. Case presentation: A 28 year old male with no significant past medical history, presented with complaint of hypersomnolence, developed during puberty. He had multiple evaluations with no apparent etiology; sleep study and all his other laboratory tests were normal including testosterone levels, normal IGF-1 and cortisol. When patient was evaluated in the Endocrine clinic, he was found to have bilateral gynecomastia, which he had for many years. His estradiol was 150 pg/ml (Normal &lt;50 pg/ml). Repeat was 137 pg/ml with normal DHEA-S Subsequent concomitant estradiol of 204 pg/ml with an estrone of 35.7 pg/ml (9–36). Total testosterone was normal at 588 ng/dl. Evaluation for a tumor with abdominal CT, testicular ultrasound, and HCG was negative. As his symptoms of fatigue and hypersomnolence were not improving and his estradiol to testosterone ratio was &gt;10, he was started on an aromatase inhibitor and his ratio dropped from 1:40 to 1:24, as his estradiol went down to 75 pg/ml. Discussion: Gynecomastia is the benign proliferation of breast tissue due to imbalance between estrogen and testosterone. It could be caused by medications or medical illnesses. Occasionally its presence can harbor a serious endocrine issue especially if presenting in the prepubertal period. Thus, evaluation is often necessary. Among the pathological causes is the Aromatase excess syndrome. In this syndrome there are three types of cryptogenic genomic rearrangements identified. Those rearrangement affect the aromatase gene CYP19 and results in gain of function of the aromatase enzyme. Patients will have high estradiol and estrone level, lower FSH and LH levels that will normalize after treatment with aromatase inhibitor. Their testosterone levels could be low or normal. For the clinical diagnosis, there are four criteria; bilateralgynecomastia, pre or peripubertal onset, exclusion of other causes of gynecomastia and having a genetic trait. The first three criteria are indispensable for diagnosis while fourth one is not obligatory, but rather pathognomonic. An elevated estradiol to testosterone ratio above 1:10 is a supportive finding, as well as having a low FSH with low to normal LH. Genetic identification of the CYP19 A1 mutation remains the definitive method of diagnosis. Our patient met the first three criteria and also had estradiol to testosterone ratio is &gt; 1:10. Genetic confirmation is challenging. Consequently, whole genome sequencing may be required. Though unusual, this case highlights the importance of looking deep in the differential when evaluating gynecomastia.