bluuebunny/crossref_metadata_2025_split huggingface dataset:
SUN-169 A Rare Case of Adrenocortical Carcinoma Presenting as Hyperaldosteronism Combined with Cushing’s Syndrome
Authors: Punni Emma , Lim Jonea 
Published: May 2020

Abstract
Introduction: Adrenocortical carcinoma (ACC) is a rare malignancy with poor prognosis. Most ACC cases are hormonally functional. Commonly produced hormones are cortisol, followed by adrenal androgens. Aldosterone secretion is quite rare (&lt; 2%) (1). Co-secretion of multiple hormones is further rare. Case Presentation: A 59 year old female presented with progressive worsening hypertension. Initial biochemical evaluation confirmed primary hyperaldosteronism. Her potassium was low at 2.9 mEq/L (N: 3.5-5.3 meq/L). Aldosterone level was elevated at 23 ng/dL (N: &lt; or = 28 ng/mL), renin value was 0.90 ng/mL/h (N: 0.25-5.82 ng/mL/h). Aldosterone Renin Ratio was 25. CT abdomen with and without contrast showed 2 lesions within the right adrenal gland. The larger nodule was 3.7 x 2.7 x 4.9 cm with an absolute washout of 61%. A smaller nodule was 2.3x1.8 cm with an absolute washout of approximately 64%. Left adrenal gland was unremarkable.
Further biochemical workup showed plasma catecholamines and metanephrines were normal. A random cortisol value was 22.8 mcg/dL (N: 3.0-16.0 mcg/dL) with a low ACTH level of 3.3 pg/mL (N: 7.2 - 63.3 pg/mL). Given suspicion for adrenal Cushing’s syndrome, we further obtained a 24 hour free urinary cortisol which was elevated at 84.1 mcg/24 h (N: 4-50 mcg/24 h). 8 am Cortisol after an overnight 1 mg Dexamethasone failed to suppress at 17.5 mcg/dL (n&lt;1.8 mcg/dL). DHEAS level was low at 14.1 ug/dL (N: 29.4-220.5 ug/dL). The patient eventually underwent a right adrenalectomy. Post-operatively, her cortisol was suppressed at 1.9 mcg/dL (8 am ref range: 4-22 mcg/dL), and Hydrocortisone replacement dose was initiated. Surgical pathology report was consistent with adrenocortical carcinoma. The patient continues to follow-up with the endocrinology and oncology department for treatment. Conclusion: This case is particularly interesting given the co-secretion of both aldosterone and cortisol by an adrenocortical carcinoma which has been reported in only a few cases in literature. The case highlights the importance of completing a comprehensive biochemical workup pre-operatively in patients with suspicious adrenal mass. There should especially be a low threshold for initiating workup for cortisol hypersecretion as early intervention can help avoid an adrenal crisis in the post-operative period for such patients. A low DHEAS level should raise suspicion for cortisol hypersecretion in a patient with adrenal lesions. As ACTH is the primary stimulant of DHEA, the suppression of ACTH secretion in the setting of adrenal Cushing’s syndrome can contribute to a low DHEAS level. Reference 1.Else, T et al. Adrenocortical Carcinoma. Endocr Rev. 2014; 35(2):282-326.
SAT-074 A Rare Endocrine Cause of Pseudotumor Cerebri
Authors: Gazzaz Nour , Patel Trisha , Metzger Daniel L 
Published: May 2020

Abstract
Introduction: Idiopathic intracranial hypertension, also known as pseudotumor cerebri, can be associated with various medications, obesity, systemic conditions, and inherited disorders. To the best of our knowledge, this is the second pediatric case of a GnRH agonist reported to cause pseudotumor cerebri.
Case presentation: Our patient, a 12 5/12-year-old transgender male (birth-assigned female), started depot leuprolide acetate to suppress puberty at 11 10/12 years of age (early Tanner 2 breast development). He received Lupron Depot® 7.5 mg intramuscularly for 4 doses, then 22.5 mg intramuscular every 13 weeks thereafter. Five months after his first injection, a routine eye examination revealed bilateral papilledema and enlarged blind spots, which was confirmed by a Pediatric Ophthalmologist. He was asymptomatic. There was no marked weight gain in the previous year with a BMI of 24.5 kg/m2 (+1.85 SD). His blood pressure was 110–123 mmHg systolic and 71–85 mmHg diastolic. Neurological examination was normal. CT head was normal. Cranial MRI showed slight flattening of the optic nerve heads, mild engorgement of optic nerve sheath fluid, and no space-occupying mass. Sedated lumbar puncture revealed elevated opening pressure of 31 cm H2O. CSF analysis, including pathology, was benign. He was managed with acetazolamide. Based on these findings, he was diagnosed with pseudotumor cerebri secondary to the GnRH agonist. Follow-up by the Ophthalmologist one month after starting acetazolamide showed significant improvement of the papilledema.
Conclusion: This case highlights that patients on GnRH agonist therapy are at risk for pseudotumor cerebri, and we recommend periodic ophthalmologic surveillance.
MON-670 An Overlooked Cause of Diabetic Pain
Authors: Arzeno Luis Enrique , Lessard Kimberly Kochersperger , Jabbour Serge A 
Published: May 2020

Abstract
Persistent hyperglycemia has been associated with vascular damage in patients with uncontrolled diabetes. Special emphasis has been placed on the heart, kidneys, eyes, and brain since those major organs are vital. However, little has been studied in terms of the vascular supply to the muscle and how it could be affected by high blood glucose. Here we present a 26-year-old female with a history of uncontrolled Type 1 Diabetes Mellitus treated with insulin pump who presented with muscle aches on her right lower extremity. During the evaluation at the Emergency Department (ED), the patient was noted to have diabetes ketoacidosis, intravenous fluids and insulin drip were started. As part of the workup for the muscle aches multiple blood studies were ordered including Creatinine Phosphokinase (CPK) 26 IU/L (25 - 185 IU/L), Erythrocyte Sedimentation Rate (ESR) 102 (0 - 20), C-Reactive Protein (CRP) 3.4 mg/dL (&lt;=0.80 mg/dL), Aldolase 7.5 U/L (&lt;=8.1 U/L), White Blood Cell (WBC) was 13.1 B/L (4.0 - 11.0 B/L). At this point, a muscle biopsy was considered given the lack of evidence to support a definite diagnosis. Before proceeding with the biopsy, a Magnetic Resonance Imaging (MRI) of the low extremities was done, showing diffuse intramuscular edema, predominantly in the right vastus intermedius, with additional patchy intramuscular edema in the right vastus lateralis, vastus medialis, and biceps femoris, as well as the left gluteus maximus, vastus lateralis which were compatible with myositis. Also, discrete areas of myonecrosis in the right vastus intermedialis (1.7 x 1.1 x 3.6 cm), left vastus lateralis (1.7 x 0.8 x 6 cm) and left gluteus maximus (2.8 x 3 cm x 6 cm). Given her previous history of uncontrolled diabetes, the clinical presentation with low CPK levels, lack of data to support another diagnosis, and MRI findings the possibility of diabetes myonecrosis was raised. The patient was managed with conservative therapy: intravenous fluids, pain control and aspirin with improvement in myalgias and muscle strength. Diabetic myonecrosis is a rare condition that appears to be related to vasculopathic changes on uncontrolled diabetics. The lack of specific diagnostic tools and the nonspecific symptoms could make this condition to be overlooked easily; leading to unnecessary studies like muscle biopsy with consequences from complications and increased health care expenditure. A high index of suspicion is essential for timely treatment, which is limited to rest, optimal glycemic control, pain control and patients who are candidates low-dose aspirin. This condition resolves spontaneously over a few weeks to months in most patients and acknowledging this condition could provide timely relief and reassurance.
MON-255 Oral Contraceptive Pills Mask an Aggressive Crooke’s Cell Pituitary Adenoma
Authors: Gnanapragasam Helen Prathiba , Idupuganti Amrutha , Karunakaran Abhijana 
Published: May 2020

Abstract
Background
Crooke’s cell tumors are rare and aggressive forms of pituitary adenomas. This variant of Cushing’s disease requires prompt diagnosis to avoid life-threatening complications. We report a unique case of Crooke’s cell tumor with longstanding history of irregular menstrual cycles, undiagnosed and later presented as acute unilateral ptosis and diplopia due to aggressive tumor invasion.
Clinical Case
23-year-old female presented to the ER with facial swelling, left eye droop and diplopia for 3 days. She had a past medical history of oligomenorrhea and hirsutism which was normalized by oral contraceptive pills (OCP)- a combination of ethinyl estradiol and drospirenone for the last 3 years. Years prior, workup of her oligomenorrhea showed normal androgenic profile with normal DHEA-S, testosterone and 17-OH progesterone. Current exam was also significant for elevated blood pressure 200/110mmHg, BMI 37, pigmented abdominal striae and terminal hair over her chin. Labs remarkable for hypokalemia K+ 2.7 mmol/L (3.5-5.3), elevated AM cortisol 51 mcg/dL (4-20), low TSH 0.152 mcUnit/mL (0.4-5.0), low IGF-1 170 ng/mL (222-566) and FSH 1.4 mUnit/mL (1.0-9.0), with normal prolactin 24.3 ng/mL (&lt;0.5-25) and free T4 0.87ng/dL (0.8-1.8). MRI brain showed 2.8cm homogenous enhancing soft tissue mass involving the central skull base, sphenoid sinus, sella, suprasellar cistern, and parasellar regions; displacing the optic chiasm, and invading the cavernous sinuses bilaterally and orbital apices. Post trans-sphenoidal surgery (TSS) of the pituitary mass, her left eye ptosis and diplopia resolved. Post-op MRI showed subtotal resection of the extra-axial enhancing abnormality at the central skull base with extension to multiple other anatomic spaces. Pathology read consistent with aggressive Crooke’s cell adenoma, showing invasive biologic behavior without an elevated proliferation index with positive ACTH immunohistochemistry supportive of corticotroph cell adenoma. Post-op ACTH level 73 pg/mL (6-50) and cortisol 12.5 mcg/dL (4-20), while on dexamethasone. Repeat TSS was performed for residual adenoma. Cortisol remains elevated at 15.7mcg/dL despite high dose dexamethasone taper by the neurosurgery team for post-op development of right eye ptosis. She is currently awaiting proton beam radiation therapy.
Conclusion
Crooke’s cell tumors are an aggressive form of pituitary adenoma for which early diagnosis is crucial for its prognosis. Our case highlights the importance of maintaining a wide differential in evaluating young women with menstrual irregularities and to include screening for Cushing’s syndrome. Empiric treatment with OCPs can mask symptoms in the earlier course of Cushing’s disease as in our patient, causing recognition only after presentation with significant tumor growth. Earlier detection could have prevented adenoma invasion and potential neurological sequelae.
SAT-485 Variations in the Time Course of Pembrolizumab-Induced Thyroiditis: A Case Series
Authors: Atalay Hande , Mon Sann Y 
Published: May 2020

Abstract
Variations in the Time Course of Pembrolizumab-Induced Thyroiditis: A Case Series
Introduction:
Thyroid dysfunction is reported in 5 - 9.5 % of patients treated with Pembrolizumab, one of the PD-1 immune checkpoint inhibitors. However, the underlying mechanism of Pembrolizumab-induced thyroid disorders remains unclear.
We present 3 clinical cases with Pembrolizumab-induced painless thyroiditis. They initially presented with thyrotoxicosis, but later converted to hypothyroidism. The onset of thyrotoxicosis after the treatment and time course of changing in thyroid function were varied among these cases. All three cases had normal thyroid function prior to the treatment.
Case 1: 63-year-old M with bladder cancer, NSCLC and multiple brain metastases developed overt thyrotoxicosis with TSH 0.02 (0.34 - 4.82 mIU/mL) and Free T4 2.2 (0.8 - 1.5 ng/dL) at 108 days after the first cycle of Pembrolizumab. He had high TgAb (856) and TPO antibodies (196). In 14 days, he converted to hypothyroidism confirmed by TSH 14.6 and Free T4 0.8. Patient was started on Levothyroxine.
Case 2: 69-year-old M with Stage IV NSCLC presented with thyrotoxicosis (TSH 0.01, Free T4 1.9) at days 41 after starting Pembrolizumab. TPO antibodies and TgAb were negative. During 51 days of follow up, he converted to hypothyroidism (TSH 8.89 and continued to trend up above 60). He eventually required levothyroxine therapy.
Case 3: 57-year-old M with invasive verrucous squamous cell carcinoma of the perineum, urethra and prostate had normal TSH at baseline. Within 39 days after the first dose of pembrolizumab, TSH dropped to 0.013 and Total T4 increased to 14.8 (4.5 - 12.1 ug/dL), consistent with overt hyperthyroidism. In 108 days, patient developed hypothyroidism with Free T4 0.7.
Discussion:
All 3 cases we present did not have overt signs or symptoms of hyperthyroidism. Thyrotoxicosis spontaneously resolved without requiring steroid treatment. Studies suggest symptomatic treatment of beta blockers in cases of tachycardia, otherwise steroid use is not routinely recommended in PD1-induced thyroiditis.
One cohort study analyzed the dynamic course of thyroid functions in patients receiving Pembrolizumab. Among the patients who developed thyrotoxicosis while on PD-1 inhibitor therapy, median onset time was 47 days (14–447 days). The median time for transition from thyrotoxicosis to hypothyroidism was 42 days (21–169 days).
Conclusion:
Our case series highlight the variations in the time course and nature of the Pembrolizumab-induced thyroiditis. Clinicians should be aware of the relatively short period of fluctuations of thyroid functions in patients treated with PD-1 inhibitors.
References: Lee, Hyunju et al. “Characterization of Thyroid Disorders in Patients Receiving Immune Checkpoint Inhibition Therapy.” Cancer Immunology Research 5, no. 12 (2017): 1133–40. https://doi.org/10.1158/2326–6066.cir-17–0208.
SUN-267 Dissecting Type 2 CRH Receptor Signaling Characteristics in the Hypothalamic Cell Line MHYPOA-2/30
Authors: Alcantara-Alonso Viridiana , Gortari Patricia de , Dallmann Robert , Grammatopoulos Dimitris 
Published: May 2020

Abstract
The stress peptides coticotropin-releasing hormone (CRH) and urocortins (Ucns) exert anorectic effects acting mainly through the type 2 CRH receptor (CRH-R2) in the hypothalamus. Impairment of CRH-R2 signaling in chronically stressed rats has been linked with the development of hyperphagia (Alcantara-Alonso et al. Neuropeptides, 2017) however the exact mechanisms and molecular defects are unknown. In the present study we used the mHypoA-2/30, a hypothalamic immortalized cell line derived from adult mice (Belsham et al. FASEB J, 2009) to further explore the signaling molecules mediating the anorexigenic effect of the CRH-R2 cognate agonist urocortin 2 (Ucn2). Specifically, we investigated mRNA, protein expression and cellular localization of CRH-R2 in the mHypoA-2/30 neurons. Additionally, we examined the effects of Ucn2 on the phosphorylation of CREB and AMPK, as well as its transcriptional effects on genes of feeding-related peptides and molecules involved in modulation of circadian rhythms. Both CRH-R2 mRNA and protein expression were detected in mHypoA-2/30; indirect immunoflourescence experiments using a specific CRH-R2 antibody demonstrated widespread localization in the plasma membrane and cytoplasm. Moreover, the receptor sub-cellular localization was redistributed in response to activation by Ucn2 (100 nM), as the plasma membrane immunofluorescent signal was decreased after 4h of agonist treatment, suggesting CRH-R2 homologous internalization. We also observed a 50% increase in the phosphorylation of CREB associated with a concomitant decrease in AMPK phosphorylation after 30 min of Ucn2 treatment. Among the panel of hypothalamic genes analyzed, we identified after 24h of Ucn2 treatment increases in the gene expression of the anorexigenic peptides neurotensin and proopiomelanocortin. Interestingly, sustained CRH-R2 activation also led to an increase in the mRNA levels of Aryl Hydrocarbon Receptor Nuclear Translocator Like (ARNTL), a protein involved in the control of circadian rhythm. A luciferase reporter gene analysis of ARNTL showed that the mHypoA-2/30 cells also exhibit circadian patterns of expression and that the treatment with Ucn2 enhanced circadian amplitude of ARNTL reporter on these cells, which in turn may be involved in glucocorticoid release in circadian cycles and stimulating appetite during the activity phase of the animals. In conclusion, we found that the mHypoA-2/30 cell line expresses endogenous functional CRH-R2 that is linked to downstream regulation of anorexigenic gene expression. This cell line appears to be a useful in vitro tool to study hypothalamic CRH-R2 signaling machinery involved in central control of food intake and circadian cycles.
SUN-395 Pelvic Bone Density Is Lower Than Bone Density of Hip and Femoral Neck in Postmenopausal Women
Authors: Mohseni Mahshid , Civitelli Roberto , Stum Shannon , Toennies Bridgett , Eisen Seth 
Published: May 2020

Abstract
Pelvic fractures represent 7% of all fragility fractures; they account for 5% of the cost of osteoporotic fracture care, and are commonly (&gt; 50%) associated with loss of independence in the elderly. The incidence of pelvic fractures has increased significantly over the past 3 decades. However, little is known about the relationship between bone mineral density (BMD) and pelvic fractures. We have conducted a pilot cross-sectional study to establish a method of measuring pelvic BMD and to correlate BMD of the pelvis with BMD at other skeletal sites. Postmenopausal women without a history of pelvis and hip fragility fractures were enrolled. Hip, spine, and pelvis DXA scans were obtained using a Hologic DXA machine. Pelvic BMD was calculated using Hologic Research Software from 3 areas of the pelvis (R1: public symphysis, R2: inferior pubic ramus, and R3: superior pubic ramus), corresponding to common fracture locations. Pelvis BMD was the average of the 3 pelvis sites. Pelvic BMD measurement precision error was calculated using the root mean square method (Recommended by International Society of Clinical Densitometry (ISCD)). Statistical analysis was used to compare BMD at different sites. Alpha error was set at 0.05. Of 73 postmenopausal women who were enrolled in the study (average age 64 years, average 15 years postmenopausal), 3% had chronic kidney disease, 7% had type 2 DM, 3% were on corticosteroids and none were smokers. BMD of femoral neck assessed on pelvic DXA was not significantly different from femoral neck BMD measured on standard DXA (P=0.09). To assess pelvis BMD measurement precision, 15 patients underwent 3 separate pelvic DXA images after repositioning. BMD precision error was 0.011g/cm2 which is slightly lower than the precision total hip BMD at our center (0.007 g/cm2). BMD of R1, R2, and R3 pelvic areas were measured as 0.44±0.15, 0.41± 0.15, and 0.62 ±0.19 g/cm2, respectively. Notably, BMD of R3 was significantly higher than the other 2 areas (P&lt;0.001, ANOVA). Average BMD (0.49±0.14 g/cm2) of pelvis was significantly lower than BMD of femoral neck (0.72± 0.16 g/cm2), total hip (0.86±17 g/cm2) and spine (0.97± 19 g/cm2)(P &lt;0.001). Average BMD of pelvis was significantly lower in participants with osteopenia and osteoporosis of the hip and femoral neck compared to participants with normal BMD in those locations. In summary, we report a precise method of measuring BMD of commonly fractured areas of the pelvis. Pelvis BMD is lower than hip, femoral neck, and spine. Bone density of the pelvis correlates with hip and femoral neck bone density. The results of this pilot study can be used for future studies looking at pelvic low bone density in patients with pelvic fragility fractures which could help identify patients at risk for pelvic fragility fractures and change how osteoporosis is defined based on DXA images.
SUN-114 Successful Long-Term Medical Management of Insulinomas
Authors: Spiro Andrew , Hoang Thanh Duc , Shakir Mohamed K M 
Published: May 2020

Abstract
Background: Insulinomas are usually benign, solitary tumors. Surgical resection is the treatment of choice and only method of cure. Surgical cure is not possible with unresectable metastatic disease, unlocalizable disease, or contraindications to surgery. Long-term medical management may be required. Clinical Cases: Patient 1: A 57-year-old female was diagnosed at age 42 when imaging confirmed a 2.4cm insulinoma in the pancreatic tail and two lesions in the right hepatic lobe. She underwent distal pancreatectomy with splenectomy, and hepatic wedge resection. Follow-up imaging showed two new hepatic lesions. She underwent chemoembolization with resolution of her symptoms. By age 48, her symptoms returned. Imaging showed new lesions in the liver, not amenable to embolization. She was placed on Octreotide 20mg IM monthly and her symptoms resolved soon after initiation of therapy. She has since had an unremarkable clinical course. Additional therapies have been deferred as follow-up imaging has not shown disease progression. Patient 2: A 48-year-old female was diagnosed at age 37. CT and MRI did not find a source. A DOPA-PET showed increased uptake in head and tail of the pancreas. An 8-hour ex-laparotomy with EUS and palpation of the pancreas did not localize a mass. A second surgery, with 75% removal of the distal pancreas, including the tail and body, did not relieve her symptoms. She had unsuccessful trials of verapamil, verapamil with low-dose diazoxide, and octreotide. She started diazoxide 300mg daily, but had fluid retention and tachycardia. Her dose was decreased to 200mg daily and HCTZ 25mg daily was started. Due to persistent symptoms, prednisone was started at 5mg in the morning and 2.5mg in the afternoon, which gave symptom relief. At her request it was decreased to 5mg daily. By age 42 she had self-decreased it to 2.5 mg daily, but continued her other meds. She endorsed the return of symptoms, but felt she could manage them without increasing her dose. At age 44, as part of a clinical trial, an MRI and nuclear medicine study showed a 9mm lesion in the head of the pancreas. Another exploratory surgery did not find a lesion. Currently, she is on the same medication regiment with strict diet and frequent meals. She endorses only rare hypoglycemic episodes. Conclusion: The patients have been managed with medical therapy for 15 and 11 years respectively. Long-term survival data of patients with metastatic insulinoma indicates that prolonged survival is unlikely; however, most data is from the 1990s or earlier. It does not incorporate new medical treatments, or advancements in technology and the field of medicine. Patients who are not candidates for surgical cure, may have better survival rates and undergo longer courses of medical management. Additionally, while oral prednisone is not standard therapy for insulinoma, there are cases of its use to control hypoglycemia when other treatments are exhausted.