bluuebunny/crossref_metadata_2025_split huggingface dataset:
12257 Diagnosis Of Pheochromocytoma With Atypical Imaging Characteristics
Authors: Park Sanghwa E , Duc Hoang Thanh , Shakir Mohamed K M , Joseph Spiro Andrew 
Published: October 2024

Abstract
Disclosure: S.E. Park: None. T.D. Hoang: None. M.K. Shakir: None. A.J. Spiro: None.
Background: Diagnosis of pheochromocytoma is based on characteristic findings on imaging and lab studies. CT and MRI are the primary imaging modalities used when evaluating adrenal lesions. Gadolinium-68(68Ga)-DOTATATE PET/CT is somatostatin receptor based form of imaging used in the evaluation of patients with neuroendocrine tumors, including pheochromocytomas and paragangliomas (PPGLs). 68Ga-DOTATATE PET/CT is reported to have high sensitivity for the detection of PPGLs. Clinical Case: A 50-year-old man was evaluated for a 1.7cm right adrenal nodule, incidentally found on spinal MRI. He had no significant medical history and was not taking medications. He was asymptomatic without paroxysms of headache, palpitations, or diaphoresis. He had no notable family history. His physical exam was unremarkable. A low-dose dexamethasone suppression test showed appropriate cortisol suppression. Aldosterone and renin were in the normal range. Plasma fractionated metanephrines showed normal metanephrine level with mildly elevated normetanephrines, 194 pg/mL (ref. 0-145 pg/mL). 24hr-fractionated urine metanephrines showed normal metanephrine and normetanephrine levels. Adrenal CT showed a 1.9 x 1.4 x 1.9cm right adrenal lesion with 38 HU on noncontrast imaging. 15-minute washout showed absolute and relative washout of -27.1% and -11.1% respectively. Adrenal MRI showed a T2-hyperintense/T1-hypointense lesion. Due to concern for an asymptomatic pheochromocytoma, 68Ga-DOTATATE PET/CT was performed, and the right-side nodule showed relative absence of uptake compared to the rest of the adrenal gland. The results were interpreted as reassuring that the lesion was unlikely to represent a pheochromocytoma. During follow-up, two years later, a repeat adrenal CT showed an increase in size of the lesion to 2.0 x 1.9 x 2.6cm, with similar HU and washout. Plasma fractionated metanephrines increased to 420 pg/mL (ref. 0-145 pg/mL) and urine fractionated metanephrines were elevated 822 mcg/24hr (ref. 156-729). A clonidine suppression test was performed. During the test, the normetanephrine levels did not suppress, consistent with a pheochromocytoma. He underwent laparoscopic adrenalectomy, with pathology confirming a 2.3cm pheochromocytoma. Labs checked one month after surgery showed normalization of serum and urine normetanephrines. Conclusion: 68Ga-DOTATATE PET/CT is reported to have high sensitivity for the detection of PPGLs. In our patient, 68Ga-DOTATATE PET/CT showed relative absence of uptake in the adrenal lesion, which led to decreased suspicion for pheochromocytoma. Diagnosis was eventually made during follow-up, based on CT imaging, biochemical testing, and clonidine suppression test. More research is needed to determine the utility of 68Ga-DOTATATE PET/CT in the evaluation of adrenal pheochromocytomas.
Presentation: 6/1/2024
12363 A Unique Case Of hüRthle Cell Carcinoma of the Thyroid Gland and Meningioma
Authors: Elsayed Iya , Khan Rumana , Dhaliwal Harmeet 
Published: October 2024

Abstract
Disclosure: I. Elsayed: None. R. Khan: None. H. Dhaliwal: None.
Introduction: MEN1 gene is a tumor suppressor gene on chromosome 11q13 that encodes the nuclear protein menin, which regulates cell growth and cycle. When menin protein is deficient, MEN1 develops. MEN1 mutation usually involves tumors within the parathyroid glands, pancreas, or pituitary. Case Description: A 66-year-old male with past medical history of hypertension, hyperlipidemia, and coronary artery disease presented with sudden onset dizziness, nausea and vomiting for 1 hour. He had similar symptoms on and off for several months, however on the day of presentation, these symptoms were much more acute and severe. Stroke work-up was done including CT and CTA head which revealed a 1.7cm enhancing lesion to R temporal lobe with surrounding edema with an incidental 3 x 3 cm heterogeneous mass in the large portion of the right thyroid gland. Steroids initiated. MRI brain imaging revealed findings consistent with meningioma with local mass effect and pronounced parenchymal edema. Patient underwent successful resection of the mass and pathology confirmed diagnosis of Meningioma, WHO Grade 1. Ear Nose Throat specialist was consulted for incidental thyroid mass. Ultrasound guided Fine needle aspiration biopsy was, Pathology report returned suspicious for follicular neoplasm, Hürthle cell-type. Patient initially underwent right substernal semi-thyroidectomy. Pathology confirmed diagnosis of Oncocytic (Hürthle cell) carcinoma, minimally invasive, 4.3 cm with a single focus of capsular invasion, negative for lymphovascular invasion Discussion: This case presents a 66-year-old male with a rare coexistence of two distinct tumors: a meningioma and a Hürthle cell carcinoma of the thyroid gland. While meningiomas and Hürthle cell carcinoma are not commonly associated with MEN1 syndrome, there have been reports of atypical cases with unusual tumor associations. MEN1 is usually characterized by abnormalities in at least two affected endocrine glands, but variations can involve other endocrine and non-endocrine tumors as well. 25% of thyroid disease can be seen in MEN 1 syndrome; typically papillary thyroid cancer. Only one case in literature found Hürthle cell carcinoma and meningioma in the setting of MEN-1 phenotype. A second case unique case was Hürthle cell carcinoma with metastases to the meninges. The discovery of both a meningioma and a Hürthle cell carcinoma in this patient emphasizes the importance of clinicians being vigilant for the possibility of multiple neoplasms. This case highlights that there may be a relation between role of MEN1 with atypical neoplasms not commonly seen.
Presentation: 6/2/2024
8225 Amiodarone-Induced Thyrotoxicosis; Unusual Outcome for a Relatively Common Problem
Authors: Abobaker A , Naji A , Ansari F , Mushref M 
Published: October 2024

Abstract
Disclosure: A. Abobaker: None. A. Naji: None. F. Ansari: None. M. Mushref: None.
Background: Amiodarone-Induced Thyrotoxicosis (AIT) affect a significant proportion of patients taking Amiodarone. AIT manifests in different types and can be challenging to treat. Herein, we present a case of Amiodarone-induced thyrotoxicosis complicating ventricular tachycardia (VT) in a patient with prior history of non-ischemic dilated cardiomyopathy (DCM) requiring plasmapheresis and total thyroidectomy. Clinical case: A 38-year-old male with history of non-ischemic DCM, VT status post multiple endocardial ablations, ICD placement, and maintenance medical treatment with amiodarone and mexiletine, who was admitted to hospital on account of symptomatic episodes of ventricular tachycardia. Initial laboratory work showed significant hyperthyroidism; TSH &lt; 0.03 µIU/ml (n 0-3-5.6 µIU/ml), free T4 4.06 ng/dl (n 0-6-1-6 ng/dl) and free T3 5.1 pg/ml (n 2.5-3.9 pg/ml). Thyroid ultrasound showed moderate thyromegaly without thyroid nodules. Amiodarone was stopped, and he was started on methimazole and prednisone for a presumed mixed type AIT. During the hospital course, he developed multiple episodes of VT requiring multiple shocks by ICD. He was subsequently treated with sotalol and discharged after he was stable with improvement in his thyroid function. Consequently, he was re-admitted several times with VTs and worsening thyroid hormones level. Endocrinology team was consulted, who initially treated the patient with increasing dose of both methimazole, glucocorticoids and cholestyramine. However, due to lack of clinical response and the complicated cardiovascular presentation, total thyroidectomy after achievement of euthyroid-state was recommended. To expedite the process, the patient received plasmapheresis for 7 days with significant improvement of thyroid hormones level, and he successfully underwent total thyroidectomy. His hospital course after thyroidectomy was complicated by the development of refractory VT necessitating endocardial ablation. He was then discharged on antiarrhythmics and levothyroxine. Despite normal thyroid function after surgery, the patient was re-admitted after 15 months with VT storm and eventually underwent heart transplant surgery. Conclusion: Amiodarone-induced thyrotoxicosis, especially when unresponsive to medical treatment, poses a critical challenge, particularly in patients with arrhythmias complicating cardiomyopathy. In our case, the patient necessitated plasmapheresis followed by total thyroidectomy due to the failure of medical interventions. This approach should be considered in patients who require prompt attainment of euthyroid state.
Presentation: 6/3/2024
7965 An Extremely Rapid Progression of Anaplastic Thyroid Carcinoma!
Authors: Makadsi Sarah , Altaweel Tamara , Seyoum Berhane , Garg Ayushi 
Published: October 2024

Abstract
Disclosure: S. Makadsi: None. T. Altaweel: None. B. Seyoum: None. A. Garg: None.
Introduction: Anaplastic Thyroid Carcinoma (ATC) is a very rare and aggressive form of thyroid cancer. It mostly occurs in people over 60 years of age, has unexpected local and distant metastases with short average survival rate of 6 months and approximately 1 in 5 alive after 12 months. Though there are long term survivors, here we are presenting an unfortunate rapid case of a highly aggressive anaplastic thyroid carcinoma. Case Presentation: A 63-year-old female with history of widely metastatic stage IV lung adenocarcinoma diagnosed 3 months prior, alcohol and tobacco abuse, hypertension and arthritis who presented to the hospital with dyspnea and dysphagia. During her hospitalization, she was found to have a large neck mass and hypercalcemia. CT neck showed interval development of a 7.5 cm thyroid mass, with mild narrowing of the airway and bilateral necrotic cervical nodes. A PET/CT a month and a half prior to admission showed no evidence of this mass. Core biopsy of the mass on day 3 of admission showed poorly differentiated thyroid carcinoma, favoring anaplastic histology, stage IVB (T3a N1b M0). The pathology showed variously sized and spaced thyroid follicles with infiltration by highly atypical cells with high mitotic activity, focal prominent nucleoli and hyperchromasia without any specific architectural configuration. On day 6, she underwent tracheostomy placement and was started on chemoradiation with carboplatin and paclitaxel. Additionally, she completed 6 of 20 sessions of radiation to the thyroid. The mass effect on her airway continued to worsen daily. On day 12 of admission, she developed acute hypoxic respiratory failure and shock and was transferred to the ICU. She required aggressive care in the ICU for 3 days and was then transferred back to the medical floor. Subsequently, her clinical status stabilized, however she continued to have a very poor prognosis given the aggressive behavior of the tumor and the airway compromise. Supportive services and primary team had discussion with family regarding the goals of care and the plans were made to transition to comfort measures only. Conclusion: ATC is a rare yet devastating diagnosis. The patient in the discussion demonstrated an ultra-rapid progression of disease. Despite intensive treatment, the patient quickly deteriorated within a month and was placed under hospice care. Currently, treatment options for anaplastic thyroid carcinoma are limited and the prognosis is poor. Investigation into new, fast-acting therapies is necessary to help alter the outcomes in these patients.
Presentation: 6/1/2024
8086 An Interesting Yet Uncommon Presentation of Sensorineural Hearing Loss Due to Hypothyroidism
Authors: Kiani Amna N , Solanki Naisargee N , Lattouf Ali , Chaudhary Ahmed jamal 
Published: October 2024

Abstract
Disclosure: A.N. Kiani: None. N.N. Solanki: None. A. Lattouf: None. A. Chaudhary: None.
Introduction: Thyroid hormone is needed for the normal development of body structures including the auditory system. Apart from some common manifestations, hypothyroidism can also cause hearing loss. This hearing loss is more commonly bilateral and sensorineural. We present a case of improvement of hearing loss in an elderly female with hypothyroidism, after starting levothyroxine Case presentation: A 64-year-old female, with a past medical history of hypertension, and hypothyroidism Secondary to radioactive iodine ablation, presented after a fall. Home medications include thyroxine 175 mcg daily, however, she is not compliant with her medication. Thyroid profile 2022 showed thyroid stimulating hormone (TSH) 54.80, and free T4 0.3. A few months ago, the patient developed sudden bilateral sensorineural hearing loss that is more pronounced in the left ear. The audiogram showed moderate to profound mixed hearing loss on the right and moderate mixed hearing loss on the left, with asymmetry on her word recognition score - worse on the right. Cerum disimpactation improved hearing loss partially. Recent thyroid profile showed a TSH level 44.05, free T4 &lt; 0.25, and free T3 2.3. The patient was restarted on a home dose of levothyroxine. A week later, the patient reported subjective hearing improvement bilaterally with a repeat TSH of 19.88. Conclusion: Hearing loss secondary to hypothyroidism is not uncommon and is partially or completely reversible with treatment. Patients with newly diagnosed hypothyroidism should get a baseline and annual audiograms to monitor any changes in hearing. Additionally, evaluation for hearing loss should also include TSH levels.
Presentation: 6/3/2024
6581 Case of Conversion from Hashimoto’s Thyroiditis to Graves’ Disease: An Unusual Sequence
Authors: Atri Avica , Anastasopoulou Catherine 
Published: October 2024

Abstract
Disclosure: A. Atri: None. C. Anastasopoulou: None.
Introduction: The two most common autoimmune thyroid diseases are Hashimoto’s thyroiditis (HT) and Graves’ disease (GD). HT is a destructive thyroiditis, via autoantibodies most commonly against the thyroid peroxidase enzyme (anti-TPO), while GD results in hyperthyroidism, due to TSH-receptor stimulating antibodies (TSAb) and Thyroid Stimulating Immunoglobulin (TSI). Case Report: A 23-year-old male presented to the clinic with complaints of weight gain, dry skin and excessive hair loss. His physical examination was non-contributory and laboratory evaluation revealed a TSH of 130 uIU/ml (ref: 0.45-4.5), free T4 (fT4) 0.4 ng/dl (ref: 0.82-1.77), and positive anti-TPO antibodies, for which he was started on levothyroxine (LT4) supplementation for newly diagnosed HT. Over the first five months on treatment, his TSH and T4 progressively improved to 31.8 uIU/ml and 1.48 ng/dl, respectively. Six months later, his TSH rapidly decreased to 0.01 uIU/ml, with an fT4 elevation to 2.45 ng/dl, alongside developing hyperthyroid symptoms like weight loss, insomnia and palpitations. Due to persistently positive anti-TPO antibody titers, his hyperthyroidism was considered iatrogenic, and LT4 dose was gradually reduced, but eventually required to be discontinued. A thyroid ultrasound depicted generalized increase in vascularity, consistent with active thyroiditis. He continued to have a significantly suppressed TSH &lt;0.005 uIU/ml and elevated fT4, off all medications, and was also found to have multiple positive antibody titers [TSI of 1.02 IU/L (ref: &lt; 0.55), thyroglobulin antibody of 351 IU/ml (&lt; 0.9) and anti-TPO Ab &gt;600 IU/ml (ref&lt; 34)]. He was then diagnosed with new GD, and thyroid suppression therapy with 10mg/day of methimazole was initiated, to which he rapidly responded, with a TSH of 0.059 uIU/ml and reduced fT4 of 1.37 ng/dl. His diagnosis of GD was confirmed via an I-123 thyroid uptake scan which revealed a homogenous increased tracer uptake of 57% at 24 hours (ref 15-35%), following which he received definitive therapy for GD, with radio-active iodine (RAI) ablation with 20.6 mCi of I-131. Discussion: As HT and GD have a common autoimmunity-based pathophysiology, conversions between the two clinical states have been described, though HT to GD, as seen in our patient is much less common than the reverse conversion. Some described mechanisms include an antibody switch from thyrotropin receptor blocking to stimulating action, HT-induced thyrotroph destruction resulting in GD antibody formation, and treatment with LT4. Simultaneously elevated levels of both TSI and anti-TPO can occur, and therefore treatment and diagnosis should be based on thyroid function tests and clinical presentation. Patients with autoimmune thyroid disease require close monitoring, due to the potential risk of conversion from one clinical end of the spectrum to the other, and early recognition is important.
Presentation: 6/1/2024
6723 Development and Validation of Long-Acting Recombinant Human Thyroid-Stimulating Hormone Using SAFA Technology
Authors: Kim D , Kang M , Lee S , Cho Y , Zi G , An J , Park J , Han J , Chi S , Cha S h , Lee E 
Published: October 2024

Abstract
Disclosure: D. Kim: None. M. Kang: None. S. Lee: None. Y. Cho: None. G. Zi: Employee; Self; AprilBio Co. J. An: Employee; Self; AprilBio Co. J. Han: Employee; Self; AprilBio Co. S. Chi: Employee; Self; AprilBio Co. S. Cha: Owner/Co-Owner; Self; AprilBio Co.. E. Lee: None.
Thyrogen, a recombinant human thyroid-stimulating hormone (rhTSH), has been useful in improving diagnostic radioiodine scanning and thyroglobulin testing for patients with thyroid cancer. It is also used with radioiodine therapy to eliminate remaining thyroid tissue. However, its short half-life in the bloodstream necessitates multiple doses during treatment. We developed a new long-acting rhTSH using anti-serum albumin Fab-associated (SAFA) technology. SAFA-TSH was produced using a Chinese hamster ovary expression system. To verify its biological activity, we generated Nthy-ori 3-1 cells stably overexpressing TSHR and measured the production of cAMP. In a rat study, slow-release T3 pellets were implanted three days prior to the administration of Thyrogen or SAFA-TSH, allowing for measurement of only the amount of T4 release resulting from exogenous administration. SAFA-TSH increased cAMP production dose-dependently, but less effectively than Thyrogen at similar concentrations. SAFA-TSH required 5∼6 times the dose of Thyrogen to achieve similar cAMP levels, likely due to steric hindrance from the TSH fusion to SAFA. In a rat study, SAFA-TSH produced elevated T4 levels well after the decline in the response to Thyrogen. SAFA-TSH had significantly higher cumulative effects on T4 and free T4 levels compared to Thyrogen, as observed by nearly 2-fold higher average area under the effect curve of 335.73 vs 192.06 μg×h/dL and 135.24 vs 68.52 μg×h/dL, respectively. Long-acting TSH with bioactivity was successfully created using SAFA technology. These data support further development of SAFA-TSH in a clinical setting, potentially representing an important advancement in the treatment of patients with thyroid cancer.
Presentation: 6/3/2024
7016 Glucocorticoid Resistance Syndrome In Two Patients With Diverse Phenotype And Genotype
Authors: Battiola Tess , Francoise Couldwell Marie 
Published: October 2024

Abstract
Disclosure: T. Battiola: None. M.F. Couldwell: None.
Introduction: Glucocorticoid resistance syndrome (GRS) is a rare genetic disorder characterized by diminished sensitivity of target tissues to cortisol due to impaired glucocorticoid receptor function. Patients lack clinical manifestations of Cushing’s syndrome but may present with mineralocorticoid and/or androgen excess related to ACTH hypersecretion. We report two cases of GRS that highlight its phenotypic and genetic heterogeneity. Case description: Patient A A 66-year-old female presented with anxiety, hypertension, and newly diagnosed diabetes. Her biochemical evaluation demonstrated persistent hypercortisolism, however she had no clinical features of Cushing’s syndrome. Subsequent evaluation showed inappropriately normal ACTH. No abnormal lesions were seen on MRI of the pituitary. Inferior petrosal sinus sampling (IPSS) was not consistent with a pituitary source. Investigation for ectopic ACTH secretion with full body Cu-68 DOTATATE-PET scan was unrevealing. Her clinical picture was consistent with GRS. Genetic testing did not identify a mutation. Patient B A 43-year-old female presented with weight loss, anxiety, and fatigue. She had persistently elevated cortisol levels but no clinical findings of Cushing’s syndrome. Biochemical evaluation revealed inappropriately normal ACTH. There was no pituitary lesion on MRI. IPSS localized ACTH secretion to the pituitary however resected pituitary tissue was negative for ACTH on immunostaining. The patient was found to be heterozygous for NR3C1 c.1322G&gt;C. Discussion: We describe two cases of persistent ACTH-dependent hypercortisolism in the absence of clinical Cushing’s syndrome, leading to a diagnosis of GRS. GRS is diagnostically challenging due to a wide clinical spectrum. When suspected, evaluation should include 24h UFC and dexamethasone suppression testing. ACTH may be inappropriately normal or increased. Genetic testing confirms the diagnosis. The molecular pathogenesis of GRS has been attributed to inactivating mutations in the NR3C1 gene, which encodes the human glucocorticoid receptor (hGR). There have also been reported cases of GRS without a recognizable mutation of hGR, as in Patient A. These cases may be due to mutations in other steps of the glucocorticoid signaling pathway. Treatment of GRS is aimed at suppression of the HPA axis to reduce ACTH-mediated hypercortisolism, mineralocorticoid, and/or androgen excess. Dexamethasone can be titrated to symptoms and biochemical endpoints.
Presentation: 6/2/2024