bluuebunny/crossref_metadata_2025_split huggingface dataset:
PSAT261 Use of Steroids Pre- and Post-Teprotumumab in Thyroid Eye Disease Patients
Authors: Holt Robert J , Qashqai Anahita , Vesel Claudia , Taylor Stephanie D , Barretto Naina 
Published: November 2022

Abstract

Background
Thyroid eye disease (TED), an autoimmune disease, causes inflammation of the periorbital fat and muscle often leading to eye-bulging (proptosis), double-vision (diplopia), pain, redness and swelling. Steroids are effective in reducing inflammation, but have limited efficacy for proptosis and diplopia, and higher doses can be associated with adverse effects. Teprotumumab, an anti-insulin like growth factor-1 receptor inhibitory antibody, demonstrates significant improvements in proptosis, diplopia and inflammatory symptoms.1 Here we examined steroid use in the period leading up to, and the period after a full course of teprotumumab.

Methods
Deidentified claims data (IQVIA) from patients completing 8 infusions of teprotumumab (full course) before Dec 31, 2020 were examined for steroid use before and after treatment. To ensure that claims data were adequately captured, patients must have been in the database for at least 1 year before and 6 months after teprotumumab and have had a claim within 4 months of the first dose. The median cumulative dose of steroids (prednisone equivalent) was also determined.

Results
565 patients met the inclusion criteria with a mean age of 59.5 (13.3) years. 407 (72%) were female and 158 (28%) were male. 460 (81%) had ≥1 steroid prescription at any time before, during, or after teprotumumab within 69 months before teprotumumab and 16 months afterward with 78% use before, 10% use during and 25% after. 314 (56%) had ≥1 steroid prescription within 1 year before or 6 months after a course of teprotumumab. Of 283 patients who received steroids within 1 year pre-teprotumumab, the median cumulative dose per patient (MCDPP) increased from 295 to 607 mg 3-0 months prior to teprotumumab initiation and 82% (233) stopped steroid use after teprotumumab initiation. The MCDPP decreased by 80% (607mg to 120mg) in the 0-3 months pre-teprotumumab to 3-6 months post-teprotumumab. Finally, the number of patients receiving steroids 3-6 months after teprotumumab decreased by 74% from 3-0 months pre-teprotumumab.

Conclusions
While reasons for the escalating incidence, and magnitude, of steroid use pre-teprotumumab and reduced use post-teprotumumab could not be definitively determined, remote steroid use increased up until teprotumumab initiation with a majority of patients who had a steroid in the year preceding teprotumumab having no steroid use in the 6 months after teprotumumab. Further, the number of patients receiving steroids and the median cumulative dose of steroids post-teprotumumab was reduced by more than 70% and 80%, respectively from pre-teprotumumab doses, indicating that teprotumumab is a steroid sparing agent. Follow-up analyses will confirm the duration of this effect.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT267 Performance of ThyroSeq V3 molecular testing in assessing indeterminate thyroid nodules for thyroid cancer at an urban endocrinology clinic.
Authors: Bao Ginny , Chang Christine , Yin Anthony 
Published: November 2022

Abstract

Introduction
While fine needle aspiration (FNA) biopsy can accurately classify thyroid nodules as benign versus malignant in most cases, roughly 20% of cases remain indeterminate. Molecular testing can assist with risk stratification of indeterminate nodules, with the goal to identify benign nodules and therefore decrease further testing. We aimed to validate the accuracy of ThyroSeq v3 molecular testing for predicting malignancy in real-world practice in a diverse, urban setting.

Methods
This was a retrospective, cohort study of 41 adults with 43 indeterminate thyroid nodules, for which molecular analysis using the multigene classifier (GC) ThyroSeq v3 was performed at an outpatient endocrinology practice in San Francisco, CA between December 2011 and December 2021. The primary outcome was positive predictive value for malignancy in indeterminate thyroid nodules, using ThyroSeq v3. The secondary outcome was prediction of cancer by specific genetic alterations.

Results
Most patients were female (78.0%), with a median age of 56 years (range 30-84). Nodules were classified as low (n=28; 65.1%), low-intermediate (n=2; 4.7%), or intermediate-high (n=12; 27.9%) risk for malignancy using ThyroSeq v3. Risk could not be assessed in one nodule due to inadequate sample[1] . Of patients with low-risk nodules, only 1 underwent total thyroidectomy due to compressive symptoms[2] . Of patients with low-intermediate or intermediate-high risk nodules, most (13/14) underwent surgical resection[3] . Papillary thyroid cancer was identified in 72.7% (8/11) patients with intermediate-high risk nodules who underwent surgery. In patients with papillary thyroid cancer, the most common mutation was BRAF V600E (n=5). Among indeterminate nodules (Bethesda III and IV cytology[4]), the positive predictive value of ThyroSeq v3 was 54.5%[5] .

Conclusions
Of patients with thyroid nodules of indeterminate risk based on FNA biopsy and intermediate to high risk for malignancy as determined by ThyroSeq v3 molecular sequencing, most underwent surgical resection and were found to have thyroid cancer in most cases, with BRAF V600E being the most common mutation. ThyroSeq v3 is a useful stratification tool for those patients with indeterminate nodules.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT278 A Curious Case of Thyroglossal Duct Cancer
Authors: Faisal Sobia , Ibrahim Waleed , Gunay Canan 
Published: November 2022

Abstract

Introduction
Thyroglossal duct (TGD) cyst is a common congenital anomaly. Thyroid cancer rarely occurs in TGD cysts, affecting less than 1% of cysts. Here we present a case of TGD cancer presenting as a central neck mass.

Case Presentation
60 y.o female presented to her primary care physician with a neck mass without any compressive symptoms. She had no history of head/neck radiation exposure and no family history of thyroid cancer. She was noted to have a non-tender central neck mass with palpable cervical lymphadenopathy. Neck ultrasound revealed a 5.7 cm multilobulated cystic and solid mass anterior to the thyroid gland. CT scan of the neck revealed a lobulated mixed solid cystic mass at the level of the thyroid cartilage projecting anteriorly, with an abnormal right submandibular lymph node measuring 2.2×1.0 cm.
She was evaluated by ENT and underwent resection of the neck mass and the hyoid bone (Sistrunk's procedure). Level 1b lymph node was also removed. Operative findings were concerning for a malignant process. This was confirmed on pathology with findings of TGD papillary thyroid cancer (PTC) and level 1b lymph node demonstrating metastatic PTC with cystic features.
She was then referred to endocrine. Thyroid ultrasound revealed multiple bilateral intermediate suspicion nodules. Left inferior thyroid nodule was noted to have high suspicion sonographic features with abnormal right sided level 2 and 3 lymph nodes. She was referred for total thyroidectomy, pathology revealed multifocal PTC with lymph node involvement with extra nodal extension and metastasis to the left sternohyiod muscle, pT2, pN1b, AJCC stage 2.
6-week post-surgical labs revealed TSH of 1.69 mIU/L (ref range 0.40-4.50), thyroglobulin level of 0.8 ng/ml (ref range athyrotic &lt;0.1), with undetectable thyroglobulin antibodies &lt;1 IU/ml (ref range &lt; or = 1 IU/mL). She underwent RAI with 104.6 mci. I-131 Metastatic survey revealed increased uptake in the thyroid bed, otherwise no increased activity elsewhere. Stimulated TSH and Thyroglobulin levels were done but not collected appropriately and therefore not mentioned here. Repeat labs 12 weeks after RAI revealed a TSH of 2.86 mIU/L, Thyroglobulin level of 0.2 ng/ml, and undetectable Thyroglobulin antibody.

Conclusion
There are no standardized guidelines for management of TGD cancer given the rarity of this condition. FNAB (Fine need aspiration biopsy) may be associated with high false negatives due to inadequate sampling, given the cystic nature of these lesions. In majority of these cases definitive diagnosis of cancer is made after excision of the TGD cyst. Some experts favor a conservative approach via Sistrunk's procedure and pursuing total thyroidectomy if there is high suspicion of thyroid gland involvement as well. Whereas other authors have suggested a more aggressive approach with total thyroidectomy even without evidence of concomitant involvement of the thyroid gland.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT282 A Case of Papillary Thyroid Cancer in a Thyroglossal Duct Cyst
Authors: Priyanka Mahali Lakshmi , Whitlatch Hilary B , Stefan Simona 
Published: November 2022

Abstract

Background
Thyroglossal duct cysts (TGDC) are common congenital neck anomalies. They persist through adulthood in 7% of the general population. Associated malignancy is rare, occurring in &lt;1% of cases. Over 90% of such malignancies are papillary thyroid carcinoma (PTC). Twenty to 60% of patients with TGDC malignancies are found to have concomitant cancer in the thyroid. The rarity of TGDC malignancy make its management particularly challenging, given limited long term outcome data. We describe a case of a 4cm TGDC PTC.

Case
A 49-year-old female with a history of autoimmune thyroid disease presented for evaluation of a goiter. She reported 6 months of painful midline neck swelling. No family history of thyroid cancer and she had no history of radiation to her head or neck. She denied swallowing, dyspnea, or hoarseness. Physical exam was notable for a mildly tender, firm 2.5 cm submental midline neck mass overlying the hyoid. TSH was normal and thyroid peroxidase antibody was &gt;1000 (n: &lt;5 IU/mL). Neck ultrasound showed a 3.7×2.5×1.6 cm mixed solid/cystic midline neck mass and a mildly enlarged, heterogeneous, and vascular thyroid without discrete intrathyroidal nodules. A neck CT with contrast indicated that the midline mass was heterogeneously enhancing with tiny internal cystic spaces. The differential diagnosis included an infected thyroglossal duct cyst versus malignant transformation. Fine needle aspiration of the mass was suggestive of PTC. The patient underwent the Sistrunk procedure, with excision of the thyroglossal duct cyst, the middle of the hyoid bone, and the tissue surrounding the thyroglossal tract. Final pathology was consistent with a 4 cm PTC, classical type, without lymphatic, vascular or perineural invasion. Surgical margins were negative for malignancy. Two months postoperatively, TSH remained normal, and the thyroglobulin (TG) level was 78 with negative TG antibodies. She declined a thyroidectomy.

Conclusion
To date, there is no consensus guideline regarding optimal management for malignancy involving TGDC. Studies suggest that surgical decision making be based on patient risk stratification. High risk factors include age older than 45 years, tumor &gt;4 cm in size, extension of tumor to adjacent soft tissue, and/or the presence of nodal or distant metastases. In high-risk patients, treatment with Sistrunk procedure combined with thyroidectomy, neck dissection and radioactive iodine therapy have shown excellent results. In younger than 45 years with tumor size &lt;1.5 cm, without extension beyond cyst wall and normal thyroid ultrasound Sistrunk procedure alone might be sufficient. Despite low risk factors patients should still be advised to have thyroidectomy as there could be a 20% risk of coexisting thyroid cancer. Additional longitudinal studies are needed to confirm that this approach leads to optimal long-term outcomes for patients with TGDC-associated malignancy.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT287 Metastatic Thyroid Carcinoma from an Old Teratoma
Authors: Shaukat Mishal , Ahmad Mobeen , Ucciferro Peter 
Published: November 2022

Abstract

Introduction
Struma ovarii is rare and makes up 1% of all ovarian tumors out of which only 5-10% are malignant. We present the third reported case of delayed bony metastasis of malignant struma ovarii years after surgical removal.

Clinical Case
A 63 years old female with a medical history of cerebral palsy and teratoma resected in 2013, presented with complaints of right arm pain for 6 months in 2019. Routine lab work and X-rays of the right humerus were normal. An arthrogram showed a destructive process involving the proximal humeral meta-diaphysis, presumably malignant in etiology which was confirmed by a CT scan of the upper arm. A PET scan subsequently showed increased tracer activity associated with the right humeral head and neck along with replacement of marrow cavity by soft tissue density material. A fine needle aspiration of the lesion showed metastatic carcinoma of thyroid follicular origin. Serum TSH was 2.369 miu/ml (normal 0.350-5.500 miu/ml). An ultrasound of the neck demonstrated a normal thyroid without any identifiable nodules. She underwent radical resection of the right proximal humerus with endo-prosthetic reconstruction followed by postoperative radiation to the right shoulder. A repeat bone scan 3 months later showed increased activity in the ribs bilaterally and the distal right humerus. This was followed by a total thyroidectomy that showed no evidence of follicular cancer. A repeat TSH 8 weeks later came back at 88.36miu/ml but surprisingly even after the removal of thyroid gland the serum thyroglobulin remained elevated at 62.2 ng/ml (normal 2.8-40.9 ng/ml). This prompted a nuclear medicine thyroid whole body scan which revealed metastatic disease to the lumbar spine and multiple metastases to the abdomen. Treatment with radioactive iodine was started. The treatment, however was considered suboptimal in her case and she was started on radiation therapy which she is still receiving.

Discussion
Struma ovarii is a specialized ovarian teratoma made up of more than 50% mature thyroid tissue. Of these only 5-10% have malignant potential with less than 200 cases ever being reported. The NRAS, BRAF and JAK3 oncogene mutations have been implicated in the pathogenesis but the exact cause is unknown, resulting in the absence of any guideline directed therapy. The most common sites of metastasis are the peritoneum, mesentery and omentum. Bones are a less common metastatic site. Current treatment strategies include total thyroidectomy with iodine ablation and radioiodine scanning with serum thyroglobulin levels being followed as a tumor marker. It can rarely present as a late metastatic disease years after surgical resection of the tumor and a high index of suspicion is required to make the correct diagnosis.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT289 Extranodal Marginal Zone B-Cell Lymphoma of the Mucosa-Associated Lymphoid Tissue (MALT) Type of the Thyroid: A Rare Tumor That Requires a Second Look
Authors: Isabel D. Villonco Francesca , Cadeliña Jekrylei I , Arquiza Christine Joy S , Villa Michael L 
Published: November 2022

Abstract

Background
Extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT) type of the thyroid, or MALT lymphoma of the thyroid, is a rare, indolent tumor that accounts for only 1-2% of extranodal lymphomas. It is often associated with lymphocytic thyroiditis - a benign condition that might not be given additional investigation.

Clinical case
A 79-year-old male consulted due to a 3-year history of a gradually enlarging, painless anterior neck mass accompanied by hoarseness. He denied fever, weight loss, dysphagia, dyspnea, palpitations, heat or cold intolerance, or changes in bowel movement. On physical exam, a 4×3×1 cm firm, fixed, non-tender right anterior neck mass and a 2×2 cm right supraclavicular mass were palpated. Thyroid function tests showed: TSH 3.301 uIU/mL (0.55 - 4.78 uIU/ml), FT3 2.560 pg/mL (2.30 - 4.20 pg/mL) and FT4 0.720 ng/dL (0.89 - 1.76 ng/dL). Neck ultrasound revealed multiple cervical lymph nodes measuring 1-2 cm in the right anterior, posterior, submandibular, and left submandibular areas. A lobulated heterogeneous mass measuring 9.7×2.9×4.3 cm occupied almost the entire right thyroid lobe, with intralesional and peripheral vascularity. The left thyroid lobe had an irregular, solid, isoechoic nodule measuring 0.9×0.5×0.9 cm. Fine needle aspiration biopsy of the right thyroid mass showed lymphocytic thyroiditis (Bethesda category II). The patient underwent total thyroidectomy with frozen section and selective neck dissection on the right (levels II, III, IV, and V). Final histopathology and immunohistochemical stains revealed extranodal marginal zone lymphoma of the thyroid gland, with high grade transformation to diffuse large B-cell lymphoma in 10% of the tumor. Level II, III and V lymph nodes were positive for marginal zone lymphoma. Histopathology also showed an adenomatous goiter with lymphocytic thyroiditis. The patient was started on levothyroxine 75 mcg once daily. He completed 1 cycle of chemotherapy (R-CHOP). Two weeks after chemotherapy, he developed febrile neutropenia and expired due to sepsis.

Conclusion
In thyroid nodules and masses where lymphocytic thyroiditis is identified on fine needle aspiration biopsy, particularly in large masses accompanied by cervical lymphadenopathy, the possibility of an existing MALT lymphoma of the thyroid should be considered, and further investigation may be warranted.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
PSAT293 Uncommon Presentation of Hashimoto Thyroiditis
Authors: Alzenaidi Ahlam 
Published: November 2022

Abstract
Hashimoto thyroiditis (HT) is the most common cause of hypothyroidism in developed countries, which presents with diffuse goiter or atrophic gland. Staii et al. reported a 7.4 per cent prevalence of localized HT diagnosed by fine needle aspiration (FNA) cytology in euthyroid patients during thyroid cancer screening. Although, a transient solitary focal presentation of HT in euthyroid patients has not been described so far. Here we describe a case of localized HT presented with a thyroid mass peripartum, which progressed into diffuse thyroiditis after FNA biopsy.
A 27-year-old woman who is 10-day post-partum presents to our endocrine clinic for thyroid mass evaluation noticed by her spouse a few weeks before delivery. The patient has no hypothyroid, hyperthyroid, or mass effect symptoms. On the physical exam: BP 100/64 mmHg, Temp 36.5 C, HR 76 bpm, RR 20 bpm, BMI 24.6 kg/m2. There is a palpated 5-cm non tender right thyroid nodule. Thyroid stimulating hormone (TSH) is 2.04 uiu/ml (N: 0.3-4.2). Thyroid ultrasound (US) shows a 47×25×31 mm right hyperechoic nodule with some septation of hypoechoic areas. It has thin continuous halo, smooth but lobulated margins and Grade 3 doppler. No microcalcification is seen. Left lobe and isthmus are homogenous with no nodules. There is a reactive lymphadenopathy on the right cervical region. An ultrasound guided-FNA cytology shows clusters of follicular cells forming micro- and macro-follicles with background of hemorrhage but no colloid is seen. Cytology is suspicious of a follicular neoplasm, Bethesda IV category. Diagnostic Lobectomy is discussed but the patient opts for repeat biopsy at a different facility. She presents with a similar cytology result after 3 months. She elects follow up as she remains asymptomatic. After 6 months follow up, she has spontaneous reduction of the nodule size and it is not palpated. Neck US shows diffusely heterogeneous gland with hypoechoic areas and significant reduction in size of the right hyperechoic nodule to 22×14×11 mm. Ultrasound findings are suggestive of thyroiditis and further labs are requested. Thyroid peroxidase antibodies &gt;1000 iu/mL (N: &lt;5.61), anti-thyroglobulin antibodies 161.71 iu/mL (N: &lt;4.11), TSH 3.4 uiu/ml (N: 0.35-4.9), free T4 is 10.38 pmol/L (N: 9-19), thyroglobulin 76.76 ng/mL (N: 3.5-77).
These findings suggest that HT may present as a localized form in euthyroid phase and should be considered in the differential diagnosis of thyroid nodule. Serologic evidence of HT and serial US follow-up should be recommended instead of other unnecessary procedures.
Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.
OR04-5 Circulating Cell-Free DNA-Based Biomarkers For Prognostication and Disease Surveillance in Adrenocortical Carcinoma
Authors: Altieri Barbara , Appenzeller Silke , Arlt Wiebke , Asia Miriam , Chortis Vasileios , Elhassan Yasir S , Fassnacht Martin , Kircher Stefan , Landwehr Laura-Sophie , Lippert Juliane , Prete Alessandro , Smith Gabrielle , Steinhauer Sonja , Urlaub Hanna , Ronchi Cristina 
Published: November 2022

Abstract
Adrenocortical carcinoma (ACC) is a rare aggressive cancer with heterogeneous behaviour. Disease surveillance relies on frequent imaging, which comes with significant radiation exposure. Here, we investigated the role of circulating cell-free DNA (ccfDNA)-related biomarkers for prognostication and monitoring of ACC.
We investigated 79 patients with ACC (29M/50F, 52±14yrs; 35 primary tumors [ACC-P] and 44 recurrences [ACC-R]); while 27 patients with adrenocortical adenomas [ACA] (9M/18F, 56±16yrs) and 19 healthy subjects (HS; 9M/10F, 37±9yrs) served as controls.
We extracted ccfDNA from 1-4 ml EDTA-plasma using Nonacus Cell3 Xtract or Qiagen QIAamp MinElute kit and quantified by fluorimeter (ccfDNA concentrations, Biomarker 1). Targeted next-generation sequencing (Illumina NextSeq500) was performed in a first subgroup of 52 baseline ccfDNA samples (23 ACC-P, 20 ACC-R, 8 ACA) using a customised panel of 30 ACC-specific genes (Cell3 Target Nonacus). Leucocyte DNA was sequenced to discriminate germline from somatic variants (Biomarker 2). Sequencing data from matched tumor DNA were available for 28 ACC (20 ACC-P, 8 R-ACC). A combined Biomarker score was calculated for prediction of clinical outcome. ACC-P had the highest ccfDNA concentrations (mean±SD 1.08±1.50 ng/µl) compared to ACC-R (0.29±0.23 ng/µl, P&lt;0.05), ACA (0.17±0.13 ng/µl, P&lt;0.005) and HS (0.11±0.07 ng/µl, P&lt;0.005). Using a cutoff of 0.204 (median HS+2SD), 68% of ACC-P were postitive for Biomarker 1 (vs. ACC-R 55%, ACA 28%, HS 5%; P&lt;0.0001 by Chi square test). At ccfDNA sequencing, 43% of ACC-P showed at least one somatic mutation (=positive Biomarker 2), vs. 15% in ACC-R and 0% in ACA. Mutational status at ccfDNA level matched with tumor DNA in 80% of cases. In 23 ACC-P with available sequencing data, the combined Biomarker score was strongly associated with both progression-free and overall survival (P&lt;0.0001, HR 8.56, 95%CI 2.55-58.7, and P=0.0008, HR 13.3, 95%CI 3.77-47.1, respectively).
10 ACC-P were followed up for at least 6 months: 6 patients tumor-free at last CT scan were negative for Biomarker 1 whereas 3 out of 4 patients with early disease relapse were positive. In two recurrent cases, somatic mutations in ACC driver genes, i.e. MEN1 and ZNRF3, observed at baseline in both tumour and ccfDNA, remained detectable during monitoring.
In conclusion, ccfDNA-related biomarkers are frequently detected in patients with primary ACC. They may represent a promising, non-invasive tool to predict early disease progression and complement imaging in disease surveillance. Our findings on ccfDNA-based liquid biopsy will be validated in a larger cohort of ACCs with long-term follow up.
Presentation: Saturday, June 11, 2022 12:30 p.m. - 12:45 p.m.