bluuebunny/crossref_metadata_2025_split huggingface dataset:
SAT059 Metastatic Insulinoma Diagnosed Postpartum
Authors: Faber Alexis , Lam Han , Simon Rebecca 
Published: October 2023

Abstract
Disclosure: A. Faber: None. H. Lam: None. R. Simon: None.
Introduction: Insulinomas are rare neuroendocrine tumors derived from the beta cells of the pancreas. These tumors generally occur at a rate of four cases per million per year, and they are seen across all demographics. Affected patients present with symptoms of hypoglycemia, including confusion, diaphoresis, and palpitations. Insulinomas occur as single or multiple tumors, and can be benign or malignant. Insulinomas with metastases are defined as malignant. The primary treatment is surgical resection. However, in the case of metastatic insulinomas prognosis can be quite poor with limited treatment options. Case Presentation: A 24-year-old female employed as a nurse with otherwise negative medical history originally presented around 3 months postpartum with epigastric pain which was attributed to gas. Symptoms progressed to include lightheadedness, palpitations, and sweating. She checked her blood glucose during these events and noted it to be consistently in the 30s-40s. She would treat these episodes with food and had short-term improvement in symptoms. She presented to an outside hospital where imaging revealed: hepatomegaly with innumerable hypodense liver lesions, hypo-enhancing pancreatic head lesion, sclerotic focus in T4 vertebral body, as well as multiple enlarged upper abdominal lymph nodes including peripancreatic, porta hepatis, and gastrohepatic. Liver biopsy revealed well-differentiated neuroendocrine tumor, WHO grade 3 likely from a pancreatic primary and chemotherapy was initiated with carboplatin and paclitaxel with some response. However, hypoglycemia became refractory to management with 5% dextrose drip, so she was transferred to our facility for escalation of care. TPN was initiated and dextrose drip was escalated eventually to 40% dextrose. She was also given octreotide, hydrocortisone, and eventually diazoxide. Given degree of tumor burden as well as abdominal ascites and clinical condition, surgery was deemed high risk and not recommended. Tumor board meeting was held and her chemotherapy regimen was changed to Carboplatin/Etoposide/Atezolizumab. She subsequently underwent hepatic artery embolization twice with improvement of hypoglycemia. Despite decreasing dextrose drip requirements by over 50%, patient was unable to be liberated from dextrose infusion and had multiple discussions about management options and prognosis. The patient eventually elected to go home with home hospice. Discussion: Insulinoma diagnosed in pregnancy or shortly after pregnancy is extremely rare. There are only three case reports of malignant insulinoma diagnosed in or after pregnancy. Given the degree of extensive metastases at time of presentation, we expect our patient’s insulinoma was present prior to delivery. Hypoglycemia may have only manifested in the postpartum period when there was decreased levels of placenta-derived counterregulatory hormones.
Presentation: Saturday, June 17, 2023
SAT635 Role Of The Human Prolactin Receptor I-tail In The Pathogenesis Of Breast Cancer
Authors: Clevenger Charles V , Shen Shanwei 
Published: October 2023

Abstract
Disclosure: C.V. Clevenger: None. S. Shen: None.
Evidence from epidemiological, cellular, and genetic analyses indicate that the hormone prolactin (PRL) and its receptor (hPRLr) in humans are significantly involved in breast cancer pathogenesis. The intermediate form of hPRLr (hPRLrI) induces significant increases in proliferation and anchorage-independent growth of normal mammary epithelia in vitro when co-expressed with the long form hPRLr (hPRLrL). However, the exact mechanism of how the hPRLrI transforms mammary epithelium is not well understood. The hPRLrI is produced by alternative splicing that induces a frameshift, resulting in a premature stop codon and a novel 13 amino acid tail ("I-Tail") gain. The ubiquitin-like protein neural precursor cell expressed developmentally down-regulated protein 8 (NEDD8) was found to be associated with the I-tail by yeast two-hybrid analysis. Treatment with a selective NEDD8 inhibitor resulted in the altered stability of the hPRLrL and the death of breast cancer cells. Given this, we hypothesized that the hPRLrI I-tail contributes to mammary transformation. hPRLrL, hPRLrI, hPRLrIΔ13 (I-tail removed mutant due to loss of 13 C-terminal amino acid resides) were delivered to MCF10AT cells by lentivirus transduction and subsequently analyzed. Anchorage-independent cell growth was determined by soft agar assay, cell proliferation was determined with a Incucyte analysis, and cell motility was examined by wound healing assay. In addition, signaling pathway and differential gene expression alterations mediated by the I-tail were determined by RNA-seq and signaling analysis. Successful expression of the hPRLrL+hPRLrIΔ13 in the MCF10AT transfectant resulted in significant reductions or alterations of anchorage-independent growth, cell proliferation, ERK1/2 activation, and PRL-induced different gene expression. These findings demonstrate that the hPRLrI I-tail contributes to the transformation of normal mammary epithelia cells.
Presentation: Saturday, June 17, 2023
FRI235 Primary Adrenal Insufficiency As Presenting Symptom Of Disseminated Histoplasmosis And Cryptococcus Co-Infection In Immunocompetent Patient
Authors: Thompson Alicia 
Published: October 2023

Abstract
Disclosure: A. Thompson: None.
Background: Autoimmune adrenalitis remains the most common cause of primary adrenal insufficiency in the United States. Infectious etiologies from fungal sources are much less commonly seen. Below we discuss a case of disseminated histoplasmosis and cryptococcus that initially presented with primary adrenal insufficiency. Case: A 74 y/o F with history of Type 2 DM presented to the hospital with 3 months of fatigue and weight loss. She was initially found to be in DKA and shock requiring vasopressor support. Following the resolution of DKA, she remained hypotensive without obvious cause. A random cortisol was found to be 8.2 [6-25 ug/dL]. She then underwent an ACTH stimulation test and her cortisol level at 30 minutes was 5.9 ug/dL and at 60 minutes was 4.2 ug/dL. An ACTH level was obtained and found to be elevated at 69 [7.2-63 pg/mL]. She was subsequently started on Hydrocortisone and her shock resolved. CT adrenal glands were notable for bilateral adrenal masses measuring 4.5 cm on the right and 4.6 cm on the left with HU of 27. No other suspicious lesions were noted on imaging. An infectious work up was initiated, which was notable for fungal serologies positive of Histoplasmosis with titer of 1:8 (&lt;1:8). Histoplasmosis urine antigen was positive as well. She was tested for Cryptococcus, which was positive with a titer of 1:8 (&lt;1:8). She started treatment with Amphotericin B for 4 weeks followed by Itraconazole for maintenance therapy. Discussion: Primary adrenal insufficiency from invasive fungal infection remains uncommon and is found in less than 10% of cases. Disseminated fungal infections are identified as causes like histoplasmosis and paracoccidiomycosis. Disseminated cryptococcus is an exceedingly rare cause of adrenal insufficiency. These invasive infections can lead to bilateral enlargement of the adrenal glands and calcifications are seen on radiographic imaging. Such cases can also lead to perivasculitis and cause thrombosis and subsequent infarction of the adrenal glands. Disseminated fungal infections are more commonly seen in patients with underlying immunodeficiency such as HIV or those receiving immunosuppressant therapy. This case highlights that these infections can still be seen in those without a compromised immune system. Diagnosis can be challenging given the vague symptoms of adrenal insufficiency but should be considered in patients with electrolyte disturbances, hypoglycemia, and hypotension. There is potential for adrenal recovery following treatment with antifungal therapy. Conclusion: Although autoimmune adrenalitis is the most common cause of primary adrenal insufficiency in the US, this case highlights the importance of evaluating for other potential causes such as infectious sources, which can be treated and potentially have adrenal recovery.
Presentation: Friday, June 16, 2023
FRI238 A Rare Combination Of Thyroiditis And Secondary Adrenal Insufficiency Likely Secondary To Hypophysitis In A Patient Treated With Immune Checkpoint Inhibitors
Authors: Patel Shivani , Aluko Oluwatomisin A , Thwe Ei Ei , Gallagher Thomas , Deshmukh Mrunalini 
Published: October 2023

Abstract
Disclosure: S. Patel: None. O.A. Aluko: None. E. Thwe: None. T. Gallagher: None. M. Deshmukh: None.
Introduction: Immune checkpoint inhibitors have shown significant improvement in treatment of various cancers. Thyroiditis and hypophysitis are well-known reported adverse effects of immune checkpoint inhibitors. However, the co-existence of thyroiditis and hypophysitis in patients treated with immune checkpoint inhibitors is rare. We hereby report a case of a patient with thyroiditis and secondary adrenal insufficiency secondary likely to hypophysitis who was treated with atezolizumab for hepatocellular carcinoma. Case Report: A 65 year old male with past medical history of type 2 diabetes mellitus, hepatocellular carcinoma status post transcatheter arterial chemoembolization on atezolizumab and bevacizumab, thyrotoxicosis, liver cirrhosis secondary to prior alcohol use presented to the ED for evaluation of intermittent dizziness. Vitals signs showed Temperature 97.7°F, BP 137/64 mmHg, HR 82 beats/min, RR 18 breaths/min, SpO2 98%. During the hospital course, work up revealed AM cortisol level of 0.7 μg/dL (N 4.2-22.4 μg/dL), ACTH 33 pg/dL (N 7.2-63.3 pg/dL), TSH &lt;0.007 uIU/mL (N 0.45-4.5 uIU/mL), free t4 3.57 ng/dL (N 0.76-1.46 ng/dL), free t3 6.84 pg/mL (N 2.3-4.2 pg/mL), TSI &lt;0.1 IU/L (0-0.55 IU/L), TRAb &lt;1.1 IU/L (N 0-1.75 IU/L), anti-microsomal antibody &lt;8 IU/mL (N 0-24 IU/mL), anti-thyroglobulin &lt;1 IU/mL (0-0.9 IU/mL). Nuclear medicine uptake scan showed abnormally low 24 hr uptake indicative of thyroiditis in the setting of clinical and biochemical hyperthyroidism. MRI pituitary with and without contrast revealed normal appearing pituitary gland. Patient was subsequently started on prednisolone 30mg in AM and 10mg in PM to treat for thyroiditis as well as secondary adrenal insufficiency likely secondary to hypophysitis. Patient had improvement in symptoms of dizziness on prednisolone. Conclusion: This case highlights the co-existence of endocrinopathies associated with immune checkpoint inhibitor use. Though thyroiditis and hypophysitis are very well associated with immune checkpoint inhibitor use, co-existence of thyroiditis and hypophysitis is rare. This case illustrates the importance of having high index of suspicion for co-existence of endocrinopathies in patients treated with immune checkpoint inhibitors.
Presentation: Friday, June 16, 2023
FRI240 Aspirin Induced Adrenal Hemorrhage: Rare Side Effect Of A Common Medication!
Authors: Qadeer Hafiza Abeera , Samkutty Reshma , Singh Vinita 
Published: October 2023

Abstract
Disclosure: H.A. Qadeer: None. R. Samkutty: None. V. Singh: None.
Introduction: Adrenal hemorrhage is a heterogeneous disorder with various clinical presentations. Bilateral adrenal hemorrhage can result in acute adrenal insufficiency that can be fatal. With the increasing use of DOAC, cases of anticoagulation therapy induced adrenal hemorrhage have been reported. We present a rare case of unilateral adrenal hemorrhage in a patient with pre-existing adrenal myelolipoma after using Aspirin. Based on our literature search, previously only two cases of Aspirin Induced adrenal hemorrhage in a stable adrenal mass have been reported. Case Presentation: 55-year-old male with history of stable 6.2 cm right adrenal gland myelolipoma, fatty liver disease and hypertension, recent use of 2 doses 81 mg Aspirin after receiving right shoulder replacement surgery presented to emergency department with acute onset right sided flank pain, constant, radiating to the back and right upper quadrant associated with an episode of vomiting. He was hemodynamically stable. Blood work showed normal electrolytes, liver function and renal function at baseline. Hemoglobin was stable around 10g/dL. CT scan abdomen/pelvis with contrast showed increase in the size of a previously stable adrenal myelolipoma to 12.9 x 15.6 cm with mass effect upon the liver. Acute hemorrhage was suspected with the blood extending beyond the margins of the mass into the right retroperitoneal space, displacing the right kidney. On admission, aspirin was discontinued. With absence of hemodynamic instability, normal electrolytes, and normal AM cortisol level of 11.9 mcg/dL, adrenal insufficiency was ruled out. Hormonal work up for hyperfunctioning adrenal tumor was performed that ruled out pheochromocytoma, Cushing syndrome and Primary Aldosteronism. With symptomatic improvement, absence of progression of adrenal hemorrhage on repeat CT scan of the abdomen/pelvis, patient was discharged home. He received IR guided embolization of the right superior adrenal artery and middle adrenal artery in the outpatient setting. Discussion: Spontaneous adrenal gland hemorrhage is a rare event. Bilateral adrenal hemorrhage is associated with acute adrenal insufficiency that can be fatal. Major risk factors for adrenal hemorrhage include anticoagulant therapy, heparin Induced thrombocytopenia, hypercoagulable state, trauma and sever sepsis. Here we have reported a rare case of adrenal gland hemorrhage with therapeutic dose of aspirin used after orthopedic surgery. Aspirin is a very commonly used medication and high index of suspicion is required to avoid this side effect as Immediate discontinuation of the medication is necessary to prevent progression of the disease. Conclusion: Aspirin use can be associated with progression of stable benign adrenal tumors by causing spontaneous hemorrhage. A high index of clinical suspicion is required for timely recognition of this side effect.
Presentation: Friday, June 16, 2023
FRI245 Non-classic CAH Presenting As Bilateral Adrenal Incidentalomas
Authors: Chin Yun Ann , Puar Troy , King Thomas 
Published: October 2023

Abstract
Disclosure: Y.A. Chin: None. T. Puar: None. T. King: None.
Introduction: Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is an autosomal recessive disorder. Non-classic CAH usually presents in late childhood or asymptomatic may remain undiagnosed. Adrenal incidentalomas have been reported as a presentation of CAH. Clinical Case: A 56-year-old man presented with a 4-day history of epigastric pain associated with chest discomfort and diaphoresis. There was no postural giddiness, vomiting, paroxysm or constitutional symptoms. He was a smoker, his parents were non-consanguineous and he had fathered 2 children. He was tachycardic at 114 bpm, but normotensive (129/89 mmHg) with no postural hypotension and no abnormal pigmentation. He had evidence of heart failure with bibasal crepitations and pedal oedema. Secondary sexual characteristics were normal and testicular volume was 15 mls bilaterally with no palpable mass.
CT abdomen showed grossly enlarged bilateral adrenal glands with heterogenous nodular appearance with several focal hypodense cystic components, with absolute washout &lt;60%. Biochemistry was consistent with primary adrenal insufficiency (PAI); 8am serum cortisol 188 nmol/L, with peak cortisol post 250mcg synacthen of 195 nmol/L. Serum ACTH was elevated at 254.6 ng/L, aldosterone and renin were normal and 24hr urinary normetanephrine was mildly elevated at 1982 nmol/d (NR 600-1900 nmol/d). Echocardiography revealed severe global hypokinesia (EF 20%) but myocardial perfusion imaging was negative for ischemia.
In view of suspicion for tuberculosis, malignancy or infiltrative disease, CT guided adrenal biopsy was performed. Histology showed hyperplastic adrenal cortex with lipofuscin pigments, focal lymphohistiocytic infiltrates. No lymphoplasmacytic infiltrate with IgG 4 plasma cells, granuloma, malignant cells or amyloid deposits were seen. Serum 17-OHP level later came back elevated at 39.7 nmol/L (NR 1.8-10.4 nmol/L). DNA sequencing of CYP21 revealed compound heterozygous mutations in c.292+1G&gt;A and c.518T&gt;A, both of which have been reported to be causative of CAH. He was transitioned from stress dose to physiological glucocorticoid replacement and ACTH normalised on subsequent outpatient visits. Follow up imaging after 6 months and 2 1/2 years revealed no change in size or morphology of the adrenal glands. Follow up echocardiography showed no improvement in ejection fraction despite intensive heart failure treatment. Conclusion: Nodular adrenal hyperplasia is well described among CAH individuals, especially in those with inadequate glucocorticoid replacement and chronically elevated ACTH levels. There was no change in size of the adrenal lesions despite adequate glucocorticoid replacement. Improvement of cardiac function with glucocorticoid replacement has been reported in paediatric patients with CAH, but our case did not show any improvement on serial echocardiography.
Presentation: Friday, June 16, 2023
FRI249 New Onset Heart Failure In A Patient With Rapidly Progressive Adrenocortical Carcinoma
Authors: Peeples James , Shu Sharon , Romo Karina G , Tereul Jeremy , Grissett Bryan Benjamin 
Published: October 2023

Abstract
Disclosure: J. Peeples: None. S. Shu: None. K.G. Romo: None. J. Tereul: None. B.B. Grissett: None.
Background: Adrenocortical carcinomas (ACC) are rare, relatively large (&gt;4cm), lipid-rich, and often aggressive tumors. They may be functional, which typically presents as Cushing’s syndrome and/or virilization, or non-functional, which typically presents as an abdominal mass or incidental finding. They occur in two peak incidences: at the first decade of life and between 40-50 years of age. Over half of adult patients with ACC present with an advanced tumor stage. This case describes a young man with new onset heart failure and adrenal mass with metastases who was found to have biopsy-confirmed ACC. Clinical Case: A 20-year-old man with a history of cigarette smoking and marijuana use, but otherwise in prior good health, presented as a transfer for new onset heart failure in the setting of hypertensive emergency (SBP 260s), and CT findings of left adrenal mass, with multiple lung and liver masses. He noted 2 months of rapidly progressive weight gain, easy fatigability, dyspnea on exertion, and leg swelling. Exam revealed central adiposity with abdominal striae, bilateral clavicular and cervical fat pad, and bilateral lower extremity swelling. Investigations revealed hypercortisolism and ventricular ejection fraction 20-25%. Liver mass biopsy revealed metastatic ACC. Family history was significant for multiple members with early onset hypertension and congestive heart failure, including the death of maternal grandmother at age 40. The patient was initially managed in the ICU with an etomidate drip for adrenal suppression. Cortisol levels appropriately decreased to goal. Additionally, he started IV dexamethasone while inpatient. He was discharged once euvolemic on PO furosemide and follow-up was scheduled with cardiology, oncology, and endocrinology. At discharge, he transitioned to PO hydrocortisone (HC) and was placed on trimethoprim/sulfamethoxazole for PJP prophylaxis, doxazosin, and ketoconazole. He also started mitotane for his cancer treatment, with HC to prevent adrenal insufficiency while on mitotane. Subsequently, he started and failed carboplatin plus etoposide palliative chemotherapy with a continued increase in adrenal mass size. Now, he is on second line pembrolizumab with an ECOG score of 2. Conclusion: ACC is a rare and often aggressive tumor that should be considered in the setting of hypercortisolism and cushingoid features. This is particularly important for patients with a relatively unremarkable history and new onset heart failure with dyspnea on exertion. In these cases, mitotane can be used for hypercortisolism associated with ACC. Unfortunately, these tumors are usually diagnosed when they have already progressed to a late stage, and the prognosis for ACC is poor.
Presentation: Friday, June 16, 2023
FRI250 Dual Aldosterone And Cortisol Secreting Adrenal Cortical Carcinoma: Pre- And Perioperative Evaluation And Management
Authors: Rosenberg Rebecca , Clay Michael R , Raeburn Chris , Wierman Margaret E 
Published: October 2023

Abstract
Disclosure: R. Rosenberg: None. M.R. Clay: None. C. Raeburn: None. M.E. Wierman: None.
Representing 0.3% of all adrenal tumors, adrenal cortical carcinoma (ACC) is a rare and understudied disease. It is estimated that 60% of patients with ACC present with hormone excess syndromes, while the remainder present incidentally or with compressive symptoms from tumor invasion. ACC presentation with a combination of hormonal excess syndromes from different adrenal zones is rare and only described previously in four case reports. We present a case of dual-secreting ACC with hyperaldosteronism and cortisol excess. The previously healthy patient was noted to have new onset hypertension and hypokalemia during a primary care visit. On hormonal evaluation, he was found to have evidence of hyperaldosteronism and ACTH-independent cortisol excess (aldosterone 28.2ng/dL, plasma renin activity 0.1ng/mL/hr, and overnight 1mg dexamethasone suppression test cortisol of 13ug/dL). Imaging revealed a 2.7 x 3.1 x 3.5cm left adrenal mass suggestive of an adenoma. He underwent laparoscopic adrenalectomy and required glucocorticoid replacement for adrenal insufficiency post-operatively. Pathologic review of the gross specimen showed a sizable neoplasm measuring 6.5 x 3.0 x 2.5 cm, much larger than on CT imaging. Microscopic review showed stage T2N0M0 ACC with modified Weiss score of 6 and 68 mitoses per 50 hpf, indicative of aggressive disease. The patient’s hypokalemia resolved post-operatively, and glucocorticoids were tapered within a month. The mechanism of dual aldosterone/cortisol secretion is hypothesized to be due to overlap between genes coding for 11β-hydroxylase and aldosterone synthase, resulting in a chimeric gene with increased secretion of both enzymes, and, therefore, both hormones. Though dual secretion is rare, this case report stresses the importance of screening for cortisol excess, and hyperaldosteronism in the correct clinical setting, in patients with adrenal masses detected on imaging. A complete biochemical hormone evaluation should be performed regardless of the discovery of other hormone secreting syndromes, ideally preoperatively with a multidisciplinary team including endocrinology. The medical team should continue to bear in mind that the existence of one hypersecretory syndrome does not rule out the presence of a second or third, and hormonal excess syndromes may affect patient outcomes peri- and postoperatively.
Presentation: Friday, June 16, 2023