bluuebunny/crossref_metadata_2025_split huggingface dataset:
FRI560 A Rare Case Of Hashimoto Encephalopathy
Authors: Nokhbehzaeim Mahrokh , Keane Driscoll Henry , Sanati Milad 
Published: October 2023

Abstract
Disclosure: M. Nokhbehzaeim: None. H.K. Driscoll: None. M. Sanati: None.
Background: Hashimoto encephalopathy is a rare syndrome associated with Hashimoto’s thyroiditis. It presents as an encephalopathy without central nervous system infection or tumor. It is also described as steroid-responsive encephalopathy. It is characterized by high titers of antithyroid peroxidase antibodies. Case: 60-year-old female, with known Hashimoto thyroiditis on levothyroxine 200 mcg daily was admitted to the hospital with generalized weakness and lethargy. On admission, her TSH level was 0.085 with a Free T4 of 2.15. Subsequently, the Levothyroxine dose was decreased to 175 mcg and then was held for a few days. During her hospital stay her mental status deteriorated, and she became altered. As a part of the work-up, an MRI of the brain was done. It showed nonspecific left-sided dural thickening with enhancement, consistent with meningitis, but no signs of brain metastasis. She also developed persistent plantar flexion of both ankles and staring forward. MRI of Cervical, Thoracic, and Lumbar spine was done and leptomeningeal carcinomatosis was ruled out. She underwent a lumbar puncture; however, the CSF meningitis panel was negative. She was started on a trial of Dexamethasone 4 mg PO q 12 hours for 5 days. Her repeat thyroid function test revealed TSH: 54.4 and normal Free T4: 1.1. Levothyroxine 150 mcg was restarted, and the dose of Levothyroxine was increased to 175 mcg daily. She underwent a brain biopsy, which was unremarkable. Paraneoplastic panel and GAD 65 antibody were not detected. She was started on a trial of methylprednisolone and IVIG, and she showed significant improvement. Discussion: Hashimoto encephalopathy may involve a relapsing and remitting course and include seizures, stroke-like episodes, cognitive decline, neuropsychiatric symptoms, and myoclonus. Thyroid function is usually clinically and biochemically normal. It is characterized by high titers of antithyroid peroxidase antibodies. The other causes of encephalopathy such as toxic, metabolic, and infectious should be excluded with neuroimaging and CSF examination. Common differential diagnoses when these conditions are excluded are Creutzfeldt-Jakob disease, rapidly progressive dementias, and paraneoplastic and nonparaneoplastic limbic encephalitis. It is a rare disorder but it is responsive to treatment with corticosteroids. Clinical improvements are also observed with IV immunoglobulin and plasmapheresis.
Presentation: Friday, June 16, 2023
SAT558 Papillary Thyroid Carcinoma With Anaplastic Transformation in a Filipino Male Treated With Radioactive Iodine and Radiotherapy: A Case Report
Authors: Apa-Ap Archimedes Almasol , Abarquez Paolo Patrick , Sedurante Myrna Buenaluz , Ongbit Ann Marie , Miguel Perez Carlos , Acenas Francis Dale , Gacula John Kenneth , Lee Vannah Marjolaine , Estreller Sachiko 
Published: October 2023

Abstract
Disclosure: A.A. Apa-ap: None. P. Abarquez: None. M.B. Sedurante: None. A. Ongbit: None. P. Carlos Miguel: None. F. Acenas: None. J. Gacula: None. V. Lee: None. S. Estreller: None.
INTRODUCTION. Papillary thyroid carcinoma (PTC) is an epithelial malignancy showing follicular differentiation and distinctive nuclear features, with a generally excellent prognosis. On the other hand, anaplastic thyroid cancer (ATC) is rare and highly lethal, with an incidence of only 0.7% in the Philippines. CASE DESCRIPTION. This paper describes a 43year old Filipino male with a 3-year history of anterior neck mass with an initial fine needle aspiration biopsy read as probable PTC. He underwent total thyroidectomy and bilateral radical lymph node dissection and histopathology revealed conventional PTC, solid variant with 5% focal anaplastic transformation. Metastatic workup revealed multiple sub-centimeter pulmonary nodules and a tiny hypodense nodule on left adrenal gland on CT scan. Bone scintigraphy showed increased tracer uptake in the posterior 10th rib and the sphenoid's greater wing. While the multidisciplinary team wanted to proceed with Radiation therapy (RT) first to address the more aggressive histology, this was not possible logistically and thus RAI was performed first. This slight deviation from guidelines was deemed acceptable by Nuclear Medicine due to the possible decrease in iodine absorption from post radiation fibrosis. After a dose of 150 mci, 4 weeks after surgery, patient then completed 33 fractions of RT. Surveillance revealed stable pulmonary nodules and adrenal mass but increasing TSH-stimulated thyroglobulin and antithyroglobulin. Patient underwent another cycle RAI and is currently stable on his 16th month from diagnosis, 15th month post initial RAI, and 13th month post-RT. DISCUSSION. In several case series, patients with minor anaplastic component had 1- and 2-year survival rates of 88% and 43%, respectively with a median survival of 17 months. The overall prognosis of patients with PTC with minor ATC component points to the value of active surveillance compared to more aggressive treatment modalities in the setting of conventional ATC.
Presentation Date: Saturday, June 17, 2023
FRI208 Treating Pheochromocytoma For Glycemic And Blood Pressure Control
Authors: Santiago Carrion Ada Marie , Gonzalez Bossolo Alex N 
Published: October 2023

Abstract
Disclosure: A.M. Santiago Carrion: None. A.N. Gonzalez Bossolo: None.
Pheochromocytomas are catecholamine-secreting tumors of the adrenal medulla chromaffin cells. They account for 0.2-0.6% of hypertension (HTN) in patients of general outpatient clinics. In addition, is known that this excess of catecholamine production is associated with impaired insulin release from pancreatic beta-cells leading to hyperglycemia. However, pheochromocytoma as a cause of diabetes and HTN is frequently overlooked. A 60-year-old woman with medical history of arterial HTN and type 2 diabetes mellitus since 10 years ago was referred to the Endocrine clinic by Nephrology for glycemic control. Her medical regimen consisted of linagliptin 5mg daily, metformin 1000mg twice daily, insulin glargine 40 units daily, losartan 50mg twice daily, amlodipine 10mg daily and hydrochlorothiazide 25mg daily. Physical examination was unremarkable, including adequate vital signs with blood pressure 110/64 mmHg and heart rate 93/min. Laboratories were notable for glycated hemoglobin 8.7%, fasting blood glucose 118 mg/dL, and urine albumin-creatinine ratio (UACR) 4622 mg/g with adequate glomerular filtration rate. Upon further investigation of nephrotic-range proteinuria, patient was incidentally found with a right adrenal mass on ultrasound, which was confirmed to have absent signal dropout on MRI and measured 5 cm. On retrospective, patient revealed feeling episodic palpitations, diaphoresis, and occasional headaches which she attributed to menopausal symptoms. Plasma free metanephrines were negative, but free normetanephrines were elevated at 2160 pg/mL (range &lt;148 pg/mL). Patient underwent successful right adrenalectomy and pathology confirmed a right 5 cm pheochromocytoma with negative margins. Unfortunately, she wished to defer genetic testing. Four weeks after surgery, plasma normetanephrines fell to normal levels 141.8 pg/mL. Furthermore, patient was able to wean off basal insulin completely and HTN remained controlled with losartan monotherapy. Glycated hemoglobin improved to 6.7%. Additionally, UACR improved to 1352.94 mg/g and empagliflozin was added to avoid diabetic kidney progression. In this case we presented a patient with common comorbidities encountered in the clinic, but with an unusual etiology. Moreover, this patient presented with nephrotic-range proteinuria that has significantly improved after removal of the tumor, which has only been seen in case reports. Some authors propose a possible immunological pathogenesis or glomerular hyperfiltration. Detection of resistant HTN should had prompted earlier detection of pheochromocytoma in this patient. As physicians, we should be aware of endocrine causes of diabetes and HTN, such as Cushing syndrome, Acromegaly, Primary Aldosteronism, and Pheochromocytoma and Paraganglioma, as treating these may lead to better quality of life, and less disease-related complications and polypharmacy.
Presentation: Friday, June 16, 2023
SAT565 Is Firefighting An Occupational Risk For Papillary Thyroid Cancer? A Case Report
Authors: Diamond-Rossi Samantha Anne , Lee Patty , Thomas Cheddhi , Stanislawski Rita , Kim Jina 
Published: October 2023

Abstract
Disclosure: S.A. Diamond-Rossi: None. P. Lee: None. C. Thomas: None. R. Stanislawski: None. J. Kim: None.
Background: Papillary thyroid cancer (PTC) is the most prevalent histologic type of thyroid cancer. The toxic exposures associated with firefighting from both protective gear and combustion products have brought to question whether this population is at an increased risk of developing certain malignancies, including thyroid cancer. The objective of this case report is to raise awareness of the possible association between firefighting and thyroid cancer.
Case Presentation: A 46-year-old asymptomatic male career firefighter presented to our clinic following a mobile screening thyroid ultrasound which revealed bilateral suspicious nodules. FNAs of a 1.0 cm right nodule and 1.2 cm left nodule were consistent with papillary thyroid cancer. Lymph node mapping revealed bilateral suspicious lymph nodes in levels 3 and 4 with FNAs confirming metastatic PTC. He underwent total thyroidectomy with bilateral central and lateral lymph node dissections confirming multifocal disease, which included numerous submillimeter foci of PTC. 21 of 68 lymph nodes had metastatic disease. He is now scheduled for adjuvant radioactive iodine therapy.
Discussion: Compared to the general population, firefighters have a higher incidence of thyroid cancer. In 2022, the International Agency for Research on Cancer (IARC) announced that occupational exposure as a firefighter would be considered a Group 1 carcinogen, designating this exposure as having sufficient evidence for carcinogenicity in humans. In recognition of this risk, our local fire department, the Fairfax County Fire & Rescue, has implemented a cancer screening protocol offered to firefighters which includes a mobile thyroid ultrasound. Our patient had extensive disease with bilateral lymph node involvement requiring radioactive iodine after surgical resection. Without a screening ultrasound, it can be presumed that our patient would have presented later and perhaps with even more advanced disease. Firefighters are exposed to carcinogens and possible endocrine disrupting chemicals as part of their work, either via their protective equipment or products of the fire itself. More research is needed to determine if the higher incidence of thyroid cancer can be attributed to this occupational exposure versus surveillance bias due to increased screening of this population in recent years.
Presentation: Saturday, June 17, 2023
SAT573 Incidental Discovery Of CHEK2 Mutation With Papillary Thyroid Cancer
Authors: Thelmo Franklin L , Mehrab Amina , Jabbour Serge A 
Published: October 2023

Abstract
Disclosure: F.L. Thelmo: None. A. Mehrab: None. S.A. Jabbour: None.
A 66-year-old white male had been following steadily with our practice for greater than a decade where we were managing his post-ablative hypothyroidism from toxic multinodular goiter 20 years prior. Many years later, the patient developed hypercalcemia and was found to have asymptomatic primary hyperparathyroidism. He did not meet any indication for parathyroidectomy. In early January 2021, the patient was referred to a clinical geneticist at the behest of his wife as she was concerned that he had a very high rate of cancer in his family. The patient had a total of 11 relatives who either currently had or previously died from a malignancy, of which only one of them was on his maternal side. The patient’s father had a personal history of breast cancer, prostate cancer, and bone cancer at the time he passed away. The patient underwent a full panel of genetic testing and was found to be positive for a CHEK2 (Checkpoint Kinase 2) mutation. Around this time, the patient’s hypercalcemia escalated up to 13mg/dL without evidence of hypoalbuminemia. Decision was made to pursue surgery. In order to localize the parathyroid adenoma, a technetium sestamibi scan and a neck ultrasound were obtained. Sestamibi scan showed increased parathyroid adenomatous activity in the left lower portion of the thyroid bed and ultrasound confirmed the left lower pole parathyroid adenoma with a dominant hypoechoic 2.5cm TI-RADS 5 thyroid nodule which on biopsy was suspicious for papillary thyroid carcinoma. The patient underwent a total thyroidectomy in addition to the parathyroidectomy. Pathological evaluation confirmed a parathyroid adenoma and a classic variant of papillary thyroid carcinoma without angioinvasion and minimal extrathyroidal invasion classified as Stage 1 T2N0M0. The patient did not meet criteria for treatment with radioactive iodine and at the time of this writing is doing well, on levothyroxine with low-normal TSH and undetectable thyroglobulin with normal serum calcium. He has no other signs of malignancy at this time. However, given his CHEK2 mutation, he will require ongoing multi-disciplinary cancer surveillance. We would like to draw attention to this not uncommon oncogenic mutation that due to its incomplete penetrance does not always present such a staggering family history of cancer and thus may go undetected. CHEK2 mutations occur in roughly 1% of all individuals who undergo genetic evaluation for cancer. The mutation moderately increases the patient and their family’s risk for multiple malignancies including: breast, thyroid, prostate, stomach, kidney, and plasma cell malignancies. Individuals with this mutation should be screened thoroughly by a multi-disciplinary team based on the severity of their inherited mutation. These individuals should also receive counseling on family planning if conception is desired.
Presentation: Saturday, June 17, 2023
SAT313 Therapeutic Dosing Of Steroids In Patients On Mitotane
Authors: Pham Christopher , Cannon Sophie M , Lopresti Jonathan S 
Published: October 2023

Abstract
Disclosure: C. Pham: None. S.M. Cannon: None. J.S. Lopresti: None.
Introduction: Mitotane is a known inhibitor of steroidogenesis with some patients requiring supraphysiologic dosing for steroid replacement. Clinical Case: A 43-year-old female with a history of adrenal cell carcinoma (ACC) on multiagent chemotherapy and mitotane presents with a pruritic rash. Three weeks prior, she had received her first dose of pembrolizumab. Her other home medications included mitotane 500mg QID and hydrocortisone 180mg (40mg-40mg-30mg -20mg QID). Labs on admission were notable for an ALT of 458 (ref 10-35 U/L), AST of 272 (ref 10-35 U/L), and normal alkaline phosphatase and bilirubin with otherwise normal electrolytes. Due to the temporal relation of her transaminitis and rash to recent pembrolizumab infusion with an otherwise negative work-up, the patient was diagnosed with immune-mediated liver injury from checkpoint inhibitors (ILICI). Per National Comprehensive Cancer Network (NCCN) guidelines, high dose methylprednisolone was initiated (1mg/kg) equivalent to approximately 525 mg hydrocortisone total daily. Over five days, the patient’s transaminases continued to rise and methylprednisolone was increased to 3mg/kg/day to account for the known effects of mitotane on steroid metabolism. The patient’s rash subsequently resolved followed by slow improvement of her transaminitis with steroid taper. Mitotane level obtained one week after discontinuation of mitotane was noted to be 21mcg/mL demonstrating persistent therapeutic levels (14-20mcg/mL).
Mitotane has a profound inhibitory effect on steroidogenesis through multiple mechanisms, inhibiting glucocorticoid production by the adrenal and increasing hepatic disposal of glucocorticoids leading to subtherapeutic doses1, Furthermore, mitotane can persist in the body for a prolonged period, with a half-life between 18-154 days. As such, patients who develop adrenal insufficiency on mitotane typically require higher doses for physiologic replacement up to three times standard physiologic dose1. In this case, only when the patient’s methylprednisolone dose was tripled did her ILICI begin to improve. Conclusion: This is one of the first cases to describe ILICI in a patient with ACC highlighting unique clinical considerations in management. Higher doses of corticosteroids may be required to overcome the effects of mitotane on steroid metabolism in patients with adverse effects from immune checkpoint inhibitor therapy.
1.)Puglisi, S., Calabrese, A., Basile, V., Pia, A., Reimondo, G., Perotti, P., & Terzolo, M. (2020). New perspectives for mitotane treatment of adrenocortical carcinoma. Best Practice & Research Clinical Endocrinology & Metabolism, 34(3), 101415. https://doi.org/10.1016/j.beem.2020.101415
Presentation: Saturday, June 17, 2023
SAT316 Severe Cushing’s Syndrome Due To Ectopic ACTH In Newly Diagnosed Small Cell Lung Cancer: The Pivotal Role Of Etomidate In Acute Treatment
Authors: Belal Heiba , Gandhi Gunjan Yogendra 
Published: October 2023

Abstract
Disclosure: H. Belal: None. G.Y. Gandhi: None.
Introduction: Ectopic ACTH syndrome can present as an endocrine emergency. Etomidate, the only parenteral steroidogenesis inhibitor, can rapidly treat critically ill patients with Cushing’s syndrome (CS) and concurrent significant metabolic derangements, severe hypertension, or psychosis. Case Description: A 66-year-old female presented with abdominal pain, vomiting, proximal muscle weakness, and confusion for 6 months. Physical exam revealed hypertension, bilateral supraclavicular fat pads, and abdominal distention, while laboratory evaluation uncovered severe hypokalemia &lt;1.5 mmol/L, metabolic alkalosis, and elevated proBNP. A hilar soft tissue mass with mediastinal lymphadenopathy and bilaterally enlarged adrenal glands were seen on CT imaging. Serum cortisol was high at 114 mcg/dL. ACTH was inappropriately elevated at 111 pg/mL. MRI revealed a normal pituitary gland. She developed hypertensive emergency and status epilepticus, requiring intubation. Due to her life-threatening presentation, a guideline-based hypercortisolism workup was forgone for emergent treatment with continuous etomidate infusion. Serum cortisol normalized after 4 days of etomidate therapy. Due to concerns of reduced seizure threshold with etomidate therapy, it was weaned off after 14 days and replaced by ketoconazole. She subsequently developed recurrent hypercortisolism (serum cortisol 92 mcg/dL). After 14 days of etomidate, 9 days of ketoconazole, and 3 days of chemotherapy for biopsy-proven small-cell lung carcinoma, serum cortisol reached a trough of 22 mcg/dL. At this point, hydrocortisone therapy was initiated (block-and-replace). Recurrent clinical hypercortisolism then developed, and hydrocortisone was discontinued. Hypercortisolism comorbidities were treated appropriately: trimethoprim-sulfamethoxazole for pneumocystis pneumonia prophylaxis, anticoagulation for DVT prophylaxis, antibiotics for acute diverticulitis, and insulin for hyperglycemia. Despite treatment, her condition worsened; she was transitioned to comfort care one month after admission and died 4 days later. Discussion: Etomidate inhibits steroidogenesis through 11 beta-hydroxylate blockade and works rapidly (within 12-24 hours) to decrease cortisol levels versus ketoconazole (several days) and has a reduced risk of hepatic injury. Side effects of etomidate include respiratory depression, myoclonus, and reduced seizure threshold. Several experts have suggested a block-and-replace strategy in severe CS treatment. While effective, this strategy poses risks of etomidate toxicity and makes serum cortisol interpretation unreliable. Etomidate analogs are on the horizon that retain the potent adrenostatic action of etomidate without its sedative-hypnotic activity, offering an alternative treatment option for treating patients with severe CS in non-ICU hospital settings.
Presentation: Saturday, June 17, 2023
SAT329 Case Series Of Patients With FGFR-1 Related Pheochromocytoma With A Focus On Biochemical And Imaging Signatures
Authors: Jhawar Sakshi , Del Rivero Jaydira , Pacak Karel 
Published: October 2023

Abstract
Disclosure: S. Jhawar: None. J. Del Rivero: None. K. Pacak: None.
Introduction: Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors arising from chromaffin cells of adrenal gland and neural crest, respectively. 40% of cases are associated with germline mutations and 30-40% somatic driver mutations. Here, we present two patients with PCC who were found to have somatic FGFR1 mutation. FGFR signaling is involved in regulation of tissue repair, angiogenesis, and inflammation. Patient 1: A 70-year-old female with a history of resistant hypertension, episodic palpitations and sweating presented with diffuse abdominal pain and chest tightness. She was noted to have a blood pressure of 220/160 mm Hg. CT scan showed a 4.2 x 3.9 cm left adrenal mass, suspicious for malignancy and a &lt;1 cm retroperitoneal mass. Blood work revealed elevated plasma normetanephrine (NM) 3543 pg/mL (normal 0- 145) and metanephrine (MN) 871 pg/mL (normal 0-62). 18F-FDG PET/CT showed a left adrenal mass without abnormal FDG activity. 68Ga-DOTATATE PET/CT was compatible with left PCC (SUVmax 28). 18F-DOPA PET/CT showed left PCC and a small retroperitoneal PGL. Two weeks after initiating phenoxybenzamine, she underwent total left adrenalectomy. Pathology was consistent with PCC. Molecular testing revealed FGFR1 pathogenic somatic mutation (c.1638C&gt;G p.Asn546Lys). Patient 2: A 73-year-old male with known left renal cell carcinoma presented to our institution for management of recurrent right PCC. He was found to have a 3.8 cm right adrenal incidentaloma eight years ago and elevated plasma NM 6.3 nmol/L (normal &lt;0.9), MN 1.5 nmol/L (normal &lt;0.5). He underwent total right adrenalectomy, and the diagnosis of PCC was confirmed on histopathology. Two years later, he developed a 5.1 x 4.7 cm nodular mass in the right adrenal fossa and a &lt;1 cm liver lesion on CT scan. The adrenal lesion showed intense avidity on 68Ga-DOTATATE PET/CT and 18F-DOPA PET/CT and mild avidity on 18FDG-PET/CT. 18F-DOPA PET/CT also showed avidity towards the liver lesion. He was started on doxazosin and underwent laparoscopic right adrenalectomy. Pathology demonstrated PCC. Whole exome sequencing of the tumor showed pathogenic FGFR1 mutation (c.1638C&gt;G, p.Asn546Lys). Discussion: Activating FGFR1 mutations are associated with sporadic PCC. The biochemical phenotype of our patients showed higher levels of NM than MN. The imaging profile revealed that 18F-DOPA PET/CT was more sensitive than 68Ga-DOTATATE PET/CT and 18F-FDG PET/CT as it showed avidity towards other extra-adrenal lesions. Further studies in larger cohorts are needed to enhance understanding of pathogenesis, biochemical and imaging phenotype, which will help tailor the treatment and potential development of novel targeted therapies.
Presentation: Saturday, June 17, 2023