bluuebunny/crossref_metadata_2025_split huggingface dataset:
5142 Pheochromocytoma In The ICU- Hiding In Plain Sight
Authors: Chandrashekar N , Arafah B 
Published: October 2024

Abstract
Disclosure: N. Chandrashekar: None. B. Arafah: None.
Introduction: Pheochromocytomas are catecholamine-secreting tumors that are rare but dangerous and occur in &lt;0.2% of patients with hypertension. Case: A 28 year old female with a 10 year history of hypertension and invasive stage 2b cervical cancer s/p chemoradiation presented to the hospital with circulatory shock from Takutsubo cardiomyopathy requiring intubation, vasopressors, CVVH, and ECMO. The endocrinology service was consulted due to a highly vascular, 3.6 cm, ∼23 Hounsfield units right adrenal mass evidenced on CT abdomen done months prior to admission. There were elevations in plasma normetanephrine to 15 nmol/L (ref &lt;0.90 nmol/L), plasma metanephrine to 1.5 nmol/L (ref &lt;0.50 nmol/L), urinary metanephrine to 565 ug/24-hours (ref 36 - 209 ug/24-hr), and urinary normetanephrine to 2580 ug/24-hr (ref 95 - 449 ug/24-hr). With her acute illness and recent use of vasopressors and quetiapine, these labs could not be accurately interpreted, and outpatient follow up was recommended. She was discharged home on metoprolol and hydralazine for hypertension management by her primary team. She was re-admitted to the CICU 2 days later for hypertensive emergency with flash pulmonary edema. With further questioning, she endorsed longstanding hypertension with episodes of headaches, palpitations, and vomiting. Her cancer therapy related menopause caused frequent nighttime hot flashes while inpatient. These episodes precipitated witnessed symptoms of catecholamine release with severe hypertension and palpitations. It was noted during her hospitalization that her symptoms were controlled during the day with frequent escape during the night despite continuous alpha and subsequent beta blockade every 6 hrs. Estrogen therapy would have been beneficial, however given a recent DVT, this was avoided and instead megestrol acetate and venlafaxine were used. With these additions, her adrenergic spells completely resolved. She then underwent a right adrenalectomy and had no significant fluctuations in her BP intraoperatively with complete resolutions of symptoms postoperatively. The pathology revealed a right 2.7 cm pheochromocytoma with no local invasion or distant metastasis. Conclusion: This is a case of pheochromocytoma crisis with cardiogenic shock followed by subsequent hypertensive emergency in the setting of inappropriate beta-blocker use. Confounders like vasopressors (increases catecholamines), intrinsic stress of critical illness (increases catecholamines), and quetiapine use (can increase normetanephrine levels) make it difficult to analyze labs. In these cases, a thorough history and imaging are key to establishing a diagnosis and initiating life-saving treatment. Another unique feature of this case is the control of the patient’s triggering menopausal hot flashes with megace and venlafaxine to preempt adrenergic spells during her admission.
Presentation: 6/2/2024
6794 Primary Adrenal Insufficiency Following Bilateral Adrenal Hemorrhage Secondary To Antiphospholipid Syndrome
Authors: Alameri Majid , Mohamed Alnuaimi Abdulla , Alabrach Yousef , Abbas Aymen , Baroud Sarah , El Khazendar Mohammed 
Published: October 2024

Abstract
Disclosure: M. Alameri: None. A.M. Alnuaimi: None. Y. Alabrach: None. A. Abbas: None. S. Baroud: None. M. El Khazendar: None.
Introduction: Adrenal insufficiency (AI) is a rare life-threatening complication of antiphospholipid antibody syndrome (APS) accounting for 0.4% to 0.5% of Al cases. Case report: A 26-year-old female with background history of primary antiphospholipid syndrome (PAPS) presented with 3 weeks history of dull abdominal pain and fatigue. Examination revealed dehydration, orthostatic hypotension and hyperpigmented skin. Biochemistry assessment was consistent with primary adrenal insufficiency (hyponatremia 125 mol/L, hyperkalaemia 6.8 mmol/L, low random cortisol 142 mol/L, high ATCH 440 pmol/L [N 1.6-13.9], low aldosterone &lt;1.05 ng/dL and high renin 128 ng/L [N 4-23.7]). She received 100mg hydrocortisone followed by regular IV hydrocortisone q6hrs. CT abdomen revealed bilaterally enlarged hyperdense adrenal glands suggestive of haemorrhage. Adrenal MRI confirmed subacute bilateral adrenal haemorrhage (BAH) replacing whole adrenal glands with hemosiderin depositions and calcifications without evidence of underlying tumor. Anti-21-hydroxylase antibody came back negative. She was discharged on hydrocortisone, fludrocortisone and hydroxychloroquine. Education was given for sick days rule and hydrocortisone emergency kit was provided for adrenal crisis. Discussion and conclusion: We describe a patient with APS, who developed Al due to bilateral subacute adrenal haemorrhages. Adrenal haemorrhage is rare but life-threatening complication of APS. High clinical vigilance is important to consider adrenal haemorrhage in differential diagnosis of Al, specifically in patients with known antiphospholipid antibody syndrome (APS).
Presentation: 6/3/2024
7446 Primary Adrenal Mature Teratoma Initially Diagnosed As An Adrenal Myelolipoma
Authors: White-Cotsmire A , Kodali B , Avram A M , Shah S S 
Published: October 2024

Abstract
Disclosure: A. White-Cotsmire: None. B. Kodali: None. A.M. Avram: None. S.S. Shah: None.
Background: Teratomas are a type of germ cell tumor containing different types of tissue (e.g., hair, teeth, bone adipose, muscle). Most teratomas are benign (i.e., mature), but they may also be malignant (i.e., immature). Both immature and mature teratomas contain derivatives of ectoderm, endoderm, and mesoderm; however, immature teratomas are differentiated by the presence of embryonic, usually neuroectodermal, tissue. Mature teratomas have the potential for malignant transformation (1–2% of cases), usually squamous cell carcinoma within the teratoma. Extra-gonadal teratomas are rare (only 1–5%); reported locations include sacrococcygeal, head and neck, retroperitoneum, mediastinum, brain and spine, liver, and rarely adrenal glands. (1) Only 18 cases of adult primary adrenal teratomas have been reported since 1952. (2)Clinical Case: A 48-year-old woman with 4 days of worsening abdominal pain underwent CT imaging, which demonstrated a 6cm complex left adrenal mass containing peripheral and scattered internal-coarse calcifications with internal soft tissue and fat components, suggestive of adrenal myelolipoma with probable sequela of previous hemorrhage. Subsequent endocrine follow-up ruled out a cortisol secreting tumor with a negative dexamethasone suppression test and pheochromocytoma with normal serum free metanephrines and normetanephrines. Dedicated adrenal protocol CT scan further characterized the mass as a mature adrenal teratoma containing a rudimentary mandible and two teeth within the soft tissue associated with internal microscopic fat. The left renal vein was anteriorly displaced by this left adrenal mass and severely narrowed by the large adrenal mass, with development of left retroperitoneal collateral venous drainage from the left kidney into the lumbar venous system. The patient opted for conservative management with active clinical surveillance. Conclusion: This is a rare case of adrenal mature teratoma in an adult, originally diagnosed as an adrenal myelolipoma. This case highlights the use of more dedicated adrenal imaging for the characterization of adrenal tumors and the broad differential of benign non-functional adrenal tumors, including myelolipomas, lipomas, angiomyolipomas, and nonfunctional adenomas. However, a definitive diagnosis can only be confirmed by surgical removal of the tumor, usually through a laparoscopic approach, with histopathologic evaluation. References: (1) O’Donovan EJ, Thway K, Moskovic EC. Extragonadal teratomas of the adult abdomen and pelvis: a pictorial review. Br J Radiol. 2014;87(1041):20140116. doi:10.1259/bjr.20140116(2) Zhou L, Pan X, He T, et al. Primary adrenal teratoma: A case series and review of the literature. Mol Clin Oncol. 2018;9(4):437-442. doi:10.3892/mco.2018.1687
Presentation: 6/1/2024
8104 Rare Case Of Ectopic Cushing’s Syndrome Secondary To Metastatic Parotid Pleomorphic Adenocarcinoma
Authors: Sean Neutel Bradley , Manroa Pooja 
Published: October 2024

Abstract
Disclosure: B.S. Neutel: None. P. Manroa: None.
Background: Pleomorphic adenocarcinoma of the parotid gland is a rare malignancy and ectopic ACTH secretion from these tumors is very rarely described. We report a case of ectopic Cushing’s syndrome (CS) in such a patient and describe the clinical course. Clinical Case: A 69-year-old female with left parotid pleomorphic adenocarcinoma status post surgery and adjuvant radiotherapy presented to her local hospital approximately 7 months after initial treatment with proximal muscle weakness and near-syncope. Workup revealed profound hypokalemia at 1.6 mmol/L (n 3.5-5.3 mmol/L) along with new-onset hypertension (BP 175/110 mm Hg) and hyperglycemia with a HbA1c 6.5 % (n &lt;5.7%), as well as metabolic alkalosis with a bicarbonate level of &gt;40 mmol/L (n 22-32 mmol/L). A CT scan showed multiple liver metastases and a biopsy confirmed metastatic parotid pleomorphic adenocarcinoma. She was then transferred to our comprehensive cancer center for further management. She reported muscle weakness, easy bruising, and facial edema. She denied acne, hirsutism, or abdominal striae. Endocrine workup revealed a random cortisol of 56 mcg/dL (n 3-18 mcg/dL) and ACTH of 378 pg/mL (n 7.2-63.3 pg/mL). Her AM cortisol level remained unsuppressed at 79.2 and 59.4 mcg/dL (n&lt; 1.8 mcg/dL) after 1 mg and 8 mg overnight dexamethasone suppression tests respectively. Late-night salivary cortisol was 7.9 mcg/dL (n &lt;0.112 mcg/dL). An MRI of the sella was negative for any pituitary lesions. After stabilizing her clinical condition and correcting electrolyte abnormalities, she was started on chemotherapy with paclitaxel and carboplatin by the oncologist. Based on clinical suspicion of ectopic CS, ketoconazole was started and titrated upwards with rapid improvement of blood pressure, hypokalemia, and hyperglycemia. She was discharged home and antihypertensives were tapered off. ACTH staining on the liver biopsy was unable to be performed due to paucity of the specimen. A Ga-68-DOTATATE PET CT revealed increased radiotracer uptake in the liver lesion suggestive of SSTR 2 receptor positivity. Despite initial clinical improvement, her disease progressed with widespread liver, lymph node, and osseous metastases within 3 months necessitating escalating doses of ketoconazole, spironolactone, and potassium supplementation. The patient elected to pursue hospice care due to intolerance to chemotherapy and passed away shortly afterward. Conclusion: Although ectopic CS caused by parotid pleomorphic adenocarcinoma is rare, prompt recognition and treatment is necessary because ectopic ACTH production is associated with poor prognosis in these patients.
Presentation: 6/3/2024
8180 Rare Variants Of Adrenocortical Carcinoma: A Retrospective Cohort Study
Authors: Villavicencio Torres Camila A , Bancos Irina 
Published: October 2024

Abstract
Disclosure: C.A. Villavicencio Torres: None. I. Bancos: None.
Background: Adrenocortical carcinoma (ACC) is a rare malignancy with an estimated annual incidence of 0.7-2 cases per million per year. Scarce data are available on rare histological variants of ACC. Objective: To describe the clinical presentation and outcomes of patients diagnosed with oncocytic, sarcomatoid and myxoid ACC variants. Methods: Retrospective single center cohort study, 1992-2021 of patients with rare histological ACC variant. Results: A total of 20 patients were diagnosed with oncocytic (17, 85%), sarcomatoid (2, 10%), or myxoid (1, 5%) ACC. Median age of diagnosis was 47 years (range 17-80), and 14 (70%) were women. All patients had unilateral ACC (12, 60% left) with a median tumor size of 11 cm (range 4-29). Adrenal hormone excess was documented in 10 (50%) patients, mostly with either glucocorticoid or androgen excess. ENSAT stage I, II, III, and IV was documented in 2 (10%), 10 (50%), 6 (30%), and 2 (10%) patients, respectively. All patients were treated with adrenalectomy (laparoscopic in 5 and open in 15). In addition, patients were treated with mitotane (10, 50%), radiation (7, 35%), and chemotherapy (5,25%). Mitotane was administered for a median of 12 months (range 4-31) and was discontinued mainly due to side effects in 9 (90%) patients. Patients were followed for a median follow up of 6.5 years (range 0.4-12). In addition to 2 patients who presented with ENSAT stage IV metastatic disease, metastatic disease developed in additional 6 (30%) patients who initially presented with localized ACC (3 stage 2, 3 stage 3, 5 oncocytic, 1 sarcomatoid). Metastatic sites included lung (n=2), omentum (n=1), duodenum (n=1), liver (n=4), and bones (n=1). At the end of follow up, 11 (55%) patients remained disease free (53% oncocytic, 50% sarcomatoid, 100% myxoid), 5 (25%) patients were alive with metastatic ACC (23% oncocytic, 50% sarcomatoid), and 4 patients died of ACC (median time to death 21 months (range 10-50) post-adrenalectomy). Conclusions: Patients with oncocytic, sarcomatoid, and myxoid ACC may have a better prognosis than in reported literature. Larger multicenter studies are needed to identify patient and tumor-specific factors that guide management to assure improved outcomes in these very rare patients.
Presentation: 6/2/2024
7432 Secondary Adrenal Insufficiency Caused By Concurrent Treatment With Budesonide And Itraconazole
Authors: Anjum F , Jakoby M G 
Published: October 2024

Abstract
Disclosure: F. Anjum: None. M.G. Jakoby: None.
Introduction: Inhaled corticosteroids (ICS) are unlikely to affect corticotroph axis function even at high doses due to low systemic absorption. However, co-treatment with drugs that reduce hepatic clearance of ICS may predispose patients to secondary adrenal insufficiency (AI). We report a case of secondary AI that occurred during concurrent treatment with budesonide and itraconazole. Case presentation: A 42-year-old female with longstanding asthma managed with inhaled budesonide (160 μg twice daily) was started on itraconazole (200 mg twice daily) for management of pulmonary histoplasmosis. Over the next three months, she experienced severe fatigue, and laboratory evaluation was notable for an undetectable (&lt; 0.4 μg/dL) 8 AM cortisol level. Serum sodium (140 mM, 136-145) and potassium (4.6 mM, 3.5-5.1) were unremarkable, and a diagnosis of drug induced secondary AI was made. Treatment with hydrocortisone (15 mg in the morning, 5 mg in the evening) was started, and the patient’s infectious disease specialist stopped itraconazole, substituting posaconazole (300 mg daily) for an additional six months. Her pulmonologist substituted fluticasone (250 μg twice daily) for budesonide. After an approximate two-month hydrocortisone taper, 8 AM ACTH level was easily detectable (37.2 pg/mL, 7.2-63.3), though 30 min stimulated cortisol level was subnormal (11.1 μg/dL, expected &gt; 18). Hydrocortisone was successfully tapered off over an additional two-months (30 min stimulated cortisol 23.4 μg/dL). Discussion: The literature on ICS induced secondary AI is generally case reports and series, and the incidence is very low (1.1/10,000 patient-years at risk). Limited oral bioavailability, high deposition in lungs, extensive protein binding, and robust hepatic clearance are factors that minimize the effects of ICS on corticotroph axis function. However, concurrent treatment with potent CYP3A4 inhibitors like itraconazole can significantly reduce hepatic clearance of ICS and predispose to systemic effects, including Cushing syndrome when high dose ICS are prescribed or secondary AI at lower doses. In a series of 25 cystic fibrosis patients co-treated with itraconazole and budesonide, 11/25 (44%) developed secondary AI. Our patient likely developed secondary AI but not Cushing syndrome due to treatment with low dose budesonide. This case illustrates the importance that endocrinologists recognize CY3A4 inhibiting drugs as a risk factor for secondary AI in patient treated with ICS.
Presentation: 6/3/2024
7213 Severe IGF-2 Mediated Hypoglycemia In A Patient With Adrenocortical Carcinoma: A Case Report
Authors: Pereira M Camelo , Itersky P , Sternlieb S , Manuel N , Dendy J 
Published: October 2024

Abstract
Disclosure: M. Camelo Pereira: None. P. Itersky: None. S. Sternlieb: None. N. Manuel: None. J. Dendy: None.
Background: Hypoglycemia has been rarely described as a complication of adrenocortical carcinoma (ACC). Tumor cells produce insulin-like growth factor II, causing a paraneoplastic syndrome with severe refractory hypoglycemia. We report the unusual case of a patient with known ACC presented with seizures and severe IGF-2 mediated hypoglycemia. Clinical case: A 21-year-old man presented to a regional hospital due to new onset seizures in September 2023. He had been diagnosed with ACC in July 2022, when he presented to the hospital with frank Cushing’s syndrome. CT scan of his chest and abdomen at that time revealed a large left suprarenal mass and multiple lung and liver masses; a biopsy from the liver lesion confirmed the diagnosis of ACC and he was started on treatment with mitotane, levoketoconazole and hydrocortisone. Oncology attempted treatment with 3 cycles of cisplatin and etoposide, with no significant response; subsequently, treatment with Keytruda initially decreased tumor size, but ultimately his CT scan showed progression of disease and mitotane was stopped in August 2023. When brought to the hospital in September 2023 due to seizures and altered mental status, the patient’s blood glucose (BG) was 25 mg/dL (70-110 mg/dL) on point of care testing, so he was started on IV 10% dextrose with improvement in BG and mental status. He underwent endotracheal intubation and was taken to the intensive care unit, showed improvement and was eventually transferred to the general medical floor, where the dextrose infusion was discontinued. However, the following day, he had another hypoglycemic episode complicated with a seizure and had to be reintubated, returning to intensive care. The patient was subsequently transferred to a large tertiary hospital. Evaluation showed undetectable point-of-care BG (&lt; 5 mg/dL) as well as hypokalemia. Fasting labs showed undetectable BHB, undetectable insulin, C-peptide of 0.32 ng/mL (0.78 - 5.19 ng/mL), and negative insulin antibodies. IGF-2/IGF-1 ratio was elevated at 15.6, suggesting IGF-2 mediated hypoglycemia. He was started on IV glucose 20% and glucagon infusions. Mitotane was restarted aiming to decrease tumor burden and consequently IGF-2 levels. Given his hypokalemia, his levoketoconazole dose had to be increased; he was also treated with stress dose steroids. His BG levels improved, and he had partial neurologic recovery, however ultimately curative treatment was not possible given the extent of his disease, so he was transitioned to comfort-focused care. Conclusion: This case illustrates the importance of appropriate workup of IGF-2 mediated hypoglycemia, allowing for the best possible therapeutic approach. In our case, reducing tumor burden was not feasible since the patient had widely metastatic disease, leading to a poor outcome.
Presentation: 6/3/2024
7777 The Adrenal Tumor Volumes Help Predict Surgical Indications And Postoperative Recurrence Of PMAH
Authors: Suzuki Sawako , Ishiwata Kazuki , Ruike Yutaro , Igarashi Katsushi , Watanabe Suzuka , Sugo Masafumi , Fujimoto Masanori , Koide Hisashi , Yokote Koutaro 
Published: October 2024

Abstract
Disclosure: S. Suzuki: None. K. Ishiwata: None. Y. Ruike: None. K. Igarashi: None. S. Watanabe: None. M. Sugo: None. M. Fujimoto: None. H. Koide: None. K. Yokote: None.
Introduction: Primary macronodular adrenal hyperplasia (PMAH) is characterized by benign bilateral enlarged adrenal masses, causing Cushing's syndrome (CS). Unilateral adrenalectomy of the largest gland has been proposed as a treatment for PMAH. However, there are cases of recurrence or persistence of hypercortisolism after unilateral adrenalectomy, and remaining tumor volumes might be associated. Objective: This study aimed to analyze whether tumor volume helps select treatment and predict postoperative recurrence. Patients and methods: We conducted a retrospective study of thirteen patients (five males and eight females, mean age 59 years) with CS due to PMAH in a single center. Eight patients with PMAH underwent unilateral adrenalectomy, but five PMAH were not. Long-term clinical and biochemical outcomes were analyzed in operated and non-operated patients (112±57 months after unilateral adrenalectomy and 144±49 months after initial diagnosis, respectively). Results: Eight PMAH patients who underwent unilateral adrenalectomy had total tumor volumes greater than 1500 mm2. After unilateral adrenalectomy, when the size of the remaining adrenal tumor was greater than 1000 mm2 (1053, 1268, and 1910, respectively), recurrence occurred along with enlargement of the residual adrenal tumor in two cases, and diabetes worsened in one case. However, when the size of the remaining adrenal tumor was less than 500 mm2 (18.2, 201, 214, 327, and 446, respectively), CS resolved. Among five PMAH patients who did not receive surgery, three PMAH patients with total tumor volume less than 1500 mm2 (856, 1038, and 1496, respectively) were biochemically controlled. However, in two PMAH patients with a total volume greater than 1500 mm2 (1610 and 1650), Cushing's signs, including buffalo hump and central obesity, appeared and worsened metabolic complications during follow-up. Conclusions: Our data suggest that adrenalectomy should be considered in PMAH with a total tumor volume greater than 1500 mm2. Unilateral adrenalectomy might have a risk of recurrence if the remaining adrenal tumor is over 1000 mm2. Further large-scale examination is required.
Presentation: 6/3/2024