bluuebunny/crossref_metadata_2025_split huggingface dataset:
6629 Pure Androgen Secreting Adrenal Adenoma In A Premenopausal Woman
Authors: Syed S , Patel S B 
Published: October 2024

Abstract
Disclosure: S. Syed: None.
Background: Pure androgen secreting adrenal adenomas are extremely rare; most of the reported cases occur in post-menopausal women, with significant virilization of acute onset and are usually malignant. Here we present a case of an androgen secreting adrenal adenoma found in a young female with regular menstrual cycles and hirsutism. Clinical Case: A 27-year-old female with no significant past medical history was referred to Endocrinology clinic for evaluation of elevated testosterone level noted on a routine OBGYN exam. Patient had miscarriage six months prior to initial endocrine clinic visit. She had a routine follow up visit with her gynecologist after miscarriage and noted to have mild male pattern hair growth on her chin, worse after stopping birth control pills two years ago. Patient had menarche at 14 years of age and had regular menstrual cycle until started birth control pills. The skin examination revealed coarse male pattern hair on chin. Scattered acne was present on back. No signs of hypercortisolism were present. Labs done at time of initial consultation showed unremarkable comprehensive metabolic panel, thyroid and gonadotropic hormones. Testosterone was elevated at 122ng/dl (0-75ng/dl). Pelvic ultrasound done in the year prior showed normal uterus and adnexal structures. Further work up showed elevated DHEAS 781 µg/dL (18-391 µg/dL) and normal 17 OH progesterone and androstenedione levels. MRI abdomen with and without contrast done at outside facility showed a 4 cm x 3.3 cm x 4.1 cm well-circumscribed round shaped, mildly heterogeneously enhancing mass arising from right adrenal gland, with restricting diffusion, no signal dropout in out-of-phase imaging. Normal ovaries were noted. Given the large size of lesion, adrenalectomy was recommended. Patient underwent laparoscopic right adrenalectomy. The histologic features on pathology specimen were consistent with an adrenal cortical adenoma. No evidence of malignancy was seen (based on modified Weiss criteria). 3 weeks post op labs showed Testosterone level reduced to 19 ng/dl (0-75ng/dl) and DHEAS 138 ug/dl (18-391 µg/dL). Patient is recovering from recent childbirth. Conclusion: In adults, pure androgen secreting adrenal tumors are extremely rare. Diagnosis of malignancy is made with histopathological examination and Weiss score calculation. Our patient has unique presentation of having mild hirsutism in setting of regular menstrual cycles with no other symptoms of virilization and is noted to have unexpectedly large adrenal adenoma. Given the size of adenoma, there was concern for adrenal cortical carcinoma, however histological examination was consistent with a benign lesion.
Presentation: 6/1/2024
8034 Putting the Pieces Together: Carney Complex, A Hyper-Rare Congenital Neoplastic Syndrome
Authors: Fontanazza Gia , Conforti Anthony R , Bird Kimberly , Caplan Michael 
Published: October 2024

Abstract
Disclosure: G. Fontanazza: None. A.R. Conforti: None. K. Bird: None. M. Caplan: None.
Intro: Carney complex (CNC) is an autosomal dominant disorder characterized by multisystem neoplasia that primarily affects the thyroid, pituitary, and adrenal glands. Its prevalence is not well established, as only 750 cases have been reported worldwide. This condition arises when an inactivating PRKAR1A gene mutation results in dysregulated cell proliferation and tumorigenesis. Complications include the potential for malignant transformation and systemic manifestations, necessitating comprehensive management and ongoing surveillance of disease progression. Case: The patient is a 58-year-old woman who initially presented as a teenager with complaints of hirsutism, truncal obesity, and violaceous abdominal striae. Initial workup revealed a low-dose dexamethasone suppression test that resulted in a non-suppressed cortisol levels. She also had undetectable adrenocorticotropic hormone (ACTH) levels. Imaging revealed no pituitary masses and demonstrated slight bilateral adrenal enlargement without adenomas. Despite these results, she was empirically treated for presumed pituitary-dependent Cushing's disease and received external beam radiation. Despite treatment, she continued to have episodes of hypercortisolism that were apparent clinically and biochemically. Over time, she developed a cardiac myxoma with six cerebrovascular accidents and multiple benign tumors. As an adult, she underwent extensive endocrine evaluation, which revealed urine cortisol excretion that paradoxically rose in response to dexamethasone. ACTH levels were always undetectable, even when she was significantly hypercortisolemic. Genetic testing revealed a homozygous mutation in the melanocortin-2 receptor (MC2R). Accordingly, she elected to have a bilateral adrenalectomy and was started on hydrocortisone and fludrocortisone. Discussion: The patient exhibited ACTH-independent micronodular adrenal hyperplasia and its most frequent variant, primary pigmented nodular adrenocortical disease. Bilateral pigmented nodular hyperplasia is a finding which can occur in association with CNC and cause episodic hypercortisolism. These patients have been shown to respond to dexamethasone with a paradoxical increase in glucocorticoid excretion. While management requires ongoing surveillance, this patient had not been diagnosed until later in life and did not have regular screening. When speculating on the late detection, her story reflects the parable of the blind men and the elephant: endocrinology managed Cushing's disease, cardiology managed atrial myxoma, and oncology managed osteochondromyxomas. Limited perspective with specialized management and an inability to see the whole picture led to prolonged diagnosis with unfortunate sequelae. This report encourages physicians to develop perspective awareness and to consider the entire picture when treating patients.
Presentation: 6/2/2024
8239 Recurrent Superior Laryngeal Nerve Glucocorticoid Injections: A Likely Cause Of Secondary Adrenal Insufficiency In An Otherwise Healthy Man
Authors: Lorin A , Pranger M C , Park J M , Clutter C A 
Published: October 2024

Abstract
Disclosure: A. Lorin: None. M.C. Pranger: None. J. Park: None. C.A. Clutter: None.
Background: Secondary adrenal insufficiency is well described in the literature with various offending agents implicated. Superior laryngeal nerve (SLN) glucocorticoid injections, however, have rarely been recorded as causal agents of secondary adrenal insufficiency. We present a case of a generally healthy man with secondary adrenal insufficiency after chronic administration ofsuperior laryngeal nerve glucocorticoid injections without other identifiable etiologies. Clinical Case: A 68-year-old man with no significant medical history presented to endocrinology clinic due to persistent nausea and severe fatigue. His initial labs were notable for morning cortisol of 1.66 mcg/dL (5-25) and an undetectable ACTH (&lt;3.2 pg/mL, 10-60) suggestive of adrenal insufficiency. FSH/LH, testosterone, FT4, and IGF-1 were otherwise normal. On initial review of his medication history, the only glucocorticoid exposure appeared to be intranasal beclomethasone, with remote courses of oral prednisone. Further history revealed that the patient was receiving superior laryngeal nerve glucocorticoid injections for chronic cough every three weeks for over a year. The injection consisted of 1:1 mixture of 1 mL of triamcinolone acetonide 200 mg/5 mL and local anesthetic, which is equivalent to triamcinolone 40 mg or hydrocortisone 200 mg. Given the volume and chronicity of the glucocorticoid injections, the patient developed secondary adrenal insufficiency as above. Since the diagnosis, the patient discontinued SLN glucocorticoid injections and was placed on physiologic hydrocortisone replacement, with gradual recovery of his HPA axis. Conclusions: Sequelae of percutaneous glucocorticoid injection into the superior laryngeal nerve for treatment of chronic cough are not well documented in terms of their effects on the HPA axis. While an effective and increasingly common treatment for chronic cough, SLN glucocorticoid injection has the potential to cause suppression of the HPA axis and cause secondary adrenal insufficiency. Excess glucocorticoid appeared to solely inhibit ACTH production, as the other anterior pituitary hormones were not affected. Understanding consequences of glucocorticoid use in any formulation is important when considering such therapies, as these may alter normal HPA function with recurrent dosing. Recovery from a disrupted HPA axis may be achieved with the use of physiologic doses of hydrocortisone replacement in the case of secondary adrenal insufficiency.
Presentation: 6/3/2024
8092 Senecence Cell Accumulation In Cushing Syndrome
Authors: Alexandra Hanzu Felicia , Abbas Maha , Diaz-Catalan Daniela , Maria Santillan Cintia , Capo Julia , Casals Gregori , Macip Salvados 
Published: October 2024

Abstract
Disclosure: F.A. Hanzu: None. M. Abbas: None. D. Diaz-Catalan: None. C.M. Santillan: None. J. Capo: None. G. Casals: None. S. Macip: None.
Background: Senescence is a terminal differentiation state that can be induced in cells in response to different stresses, including exposure to glucocorticoids. Accumulation of senescent cells in tissues induce functional impairment of different organs and age-related pathologies, due to the secretion of factors that create a pro-inflammatory microenvironment and induce further senescence in neighbour cells.Aim: We hypothesize that persistence of senescent cells in glucocorticoid target tissues can mediate pathological changes long time after cure of Cushing syndrome (CS). Quantifying the presence of these cells could have prognostic value and allow the stratification of patients into groups that could receive senotherapies (drugs specific to reduce senescent cell accumulation) as adjuvant treatment. Methods: Fractionation and unbiased mass spectrometry screen to detect proteins related to senescence in glucocorticoid target tissues (muscle, brown and white adipose tissue, liver, adrenals, hypothalamus and pituitary) and plasma in 12 weeks old male C5BL/6J mice with active CS induced by chronic oral cortiscosterone administration (5 weeks) and mice with cured CS (10 weeks after treatment) as compared to CTR mice. Proteins have been extracted by digestion of the tissue samples and tryptic peptides measured by OPA assay prior to be injected on column to a Waters nanoacquity nanoUPLC coupled to a Waters Synapt G2Si. Data has been acquired using Ion mobility enabled data independent acquisition (HDMSE). Results: Raw data extraction and analysis using Progenesis QI for Proteomics identified a tissue-specific profile of proteins linked to senescence and/or ageing persistent after remission of CS in target mice tissue, which can be used for diagnostic and therapeutic purposes.
Presentation: 6/3/2024
7392 Unilateral Adrenal Embolization In Cushing Disease-A Lifesaving Emergent Procedure
Authors: Sadiq M , Irshad B , Haider A 
Published: October 2024

Abstract
Disclosure: M. Sadiq: None. B. Irshad: None. A. Haider: None.
Case presentation: A 59-year-old female was diagnosed with Cushing Disease (CD) thirteen years ago with symptomatic hypercortisolism. Hypokalemia and elevated 24-hour urine cortisol (392 ug/24 hr, n 0-50 ug/24 hr) were noted at the time of diagnosis. Following LDDST, morning cortisol was 30.5 ug/dl, and HDDST showed greater than 50% suppression. A pituitary macroadenoma with bilateral cavernous sinus extension was noted on MRI Sella. Due to cavernous sinus invasion, she was not considered a surgical candidate and underwent Gamma knife radiation. 24-hour urine cortisol decreased significantly (20 ug/24 hours). Two years follow-up MRI brain showed an empty Sella and AM cortisol was noted to be normal at 12.2 ug/ dl with no evidence of AI. She was lost to follow-up and reestablished almost ten years after her initial gamma knife and was symptomatic from hypercortisolism. 24-hour urine cortisol at 85.4 mcg/24 hr, abnormal salivary cortisol x 2 (0.24 and 0.20 mcg/dl n &lt; 0.09 mcg/dl) confirming hypercortisolism. MRI of Sella showed recurrent adenoma with bilateral sellar masses. She was referred to Neurosurgery and was started on Ketoconazole and Paseriotide once-monthly injections. She developed new onset diastolic heart failure & upper GI bleeding, requiring hospitalization. High ACTH level of 668 pg/ml (n. 6.6- 65 pg/ ml). 8 am cortisol was 107.6 ug/dl which was suppressed to 16.1 after 8 mg overnight Decadron. Repeat Gamma knife was planned but due to her decline, it was decided to do unilateral adrenal artery embolization to reduce hypercortisolism. IR-guided adrenal artery embolization of the left middle & inferior adrenal arteries was performed. She required stress dosing of HC post-embolization which was tapered off while monitoring 8 am cortisol which started to rise & 24-hour cortisol levels were noted to be normal (51.2 mcg/24 H) on post-embolization day eight. Gamma knife radiation treatment was performed a month later after which she was noted to have GWS and required Hydrocortisone 15/10 mg twice daily, which she has been taking to date. 24-hour urine cortisol remains normal at 11.8 mcg/24 h. Discussion: Treatment for CD is primarily surgical. Radiotherapy is a second-line alternative in tumors that are non-amenable to surgery. Radiotherapy takes time to achieve its full effect. Cushing Disease patients with significant hypercortisolism and resultant complications require urgent treatment. Unilateral Adrenalectomy is described in literature to curb the cortisol burden . Similarly Unilateral Adrenal artery embolization offers less invasive approach to treat patients considered unfit for surgery. Conclusion: Unilateral adrenal artery embolization can be considered a bridging treatment in CD to reduce cortisol burden and improve the metabolic profile following which a more definitive treatment such as repeat surgery or radiotherapy can lead to long-term remission.
Presentation: 6/1/2024
12386 Unilateral Spontaneous Adrenal Hemorrhage In Pregnancy
Authors: Gondhi A , Campana M , Riofrio M , Fazendin J , Vodopivec D 
Published: October 2024

Abstract
Disclosure: A. Gondhi: None. M. Campana: None. M. Riofrio: None. J. Fazendin: None. D. Vodopivec: None.
Unilateral Spontaneous Adrenal Hemorrhage In Pregnancy Introduction: Spontaneous adrenal hemorrhage (SAH) in pregnancy is a rare and under recognized entity with an incidence of 0.14% - 1.1%, mostly unilateral. We present a case of unilateral SAH managed with adrenalectomy during pregnancy. Case presentation: A 24-year-old 14-weeks pregnant woman was referred for right flank pain and nausea due to right adrenal lesion. Medical history significant for 4 first trimester miscarriages and family history of Breast cancer and Uterine cancer in mother, Pancreatic cancer in multiple family members on paternal side. She was hemodynamically stable. Abdominal-US showed right adrenal lesion. Non-contrast MRI abdomen showed a large collection in the right adrenal gland measuring 9.6cm x 9cm. Ability to assess for underlying lesion challenging given the amount of blood products and lack of IV contrast. Laboratory analysis showed an appropriately suppressed cortisol with 1 mg overnight dexamethasone suppression test (cortisol 1.3 mcg/dl, N&lt;1.8 mcg/d Dexamethasone level 332 ng/dl, N&lt;180-550 ng/dl), normal DHEA-S (135 mcg/dl, N=18-391mcg/dl), non-suppressed ACTH (18 pg/ml, N 7.2-63 pg/ml), normal aldosterone (8.1 ng/dl, N=3-39.2 ng/dl) with a non-suppressed renin (17.8 pg/ml, N=4.2-52.2 pg/ml), normal fractionated plasma metanephrines (Free metanephrines &lt;0.2 nMol/L, N&lt;0.0.5nMol/L Free normetaphrines &lt;0.2 nMol/L, N&lt;0.9nMol/L), negative Antiphopholipid antibody testing (Beta 2 Glycoprotein I IgM &lt;2 U/ml, N&lt;20U/ml, Beta 2 Glycoprotein I IgG 2.2 U/ml, N&lt;20U/ml). The patient underwent a laparoscopic converted to open right adrenalectomy. Pathology showed a 9.6cm adrenal gland with organizing hematoma, vascular congestion, and hemorrhage. It also showed periadrenal adipose tissue with atypia, MDM2 gene amplification by FISH negative – ruling out liposarcoma. Post-operatively, her symptoms resolved without complications to the pregnancy. Differentials for adrenal hemorrhage in this pregnant patient include: 1) underlying adrenal tumor (most commonly pheochromocytoma), 2) coagulopathy (e.g. antiphospholipid syndrome), and 3) pregnancy-related adrenal hemorrhage (rare, mostly unilateral due to increased vascular flow to the adrenal gland and the hypercoagulability). After evaluating the adrenal functionality, APLAS and assessing the malignant potential with imaging studies; conservative management is appropriate in stable patients. Our patient underwent surgery due to recurrent intractable pain, and uncertainty regarding malignant potential of the adrenal mass given history of multiple cancers in the family. Conclusion: Our case highlights that when indicated, adrenalectomy in the II trimester is a safe and effective option in pregnancy. Individualized treatment should be chosen for each patient following shared decision-making.
Presentation: 6/1/2024
6999 Utilizing Machine Learning for One-Step Diagnosis Of Adrenal Diseases: Integrating Clinical, Serum Steroid, And Body Composition Data
Authors: Park S , Noh J , Kim J , Kim T , Seo H , Choo J , Choi M , Kim J 
Published: October 2024

Abstract
Disclosure: S. Park: None. J. Noh: None. J. Kim: None. T. Kim: None. H. Seo: None. J. Choo: None. M. Choi: None. J. Kim: None.
Background: With the increasing incidence of adrenal incidentalomas, the precise diagnosis of subtypes, such as mild autonomous cortisol secretion (MACS), adrenal Cushing’s syndrome (ACS), primary aldosteronism (PA), pheochromocytoma and paraganglioma (PPGL), or nonfunctioning adrenal adenomas (NFA) is becoming more critical. However, the process of making accurate diagnoses remains intricate and challenging. This study aims to explore a streamlined, one-step method for diagnosing adrenal nodules, employing a machine learning (ML) model that integrates clinical data (CL), serum steroid profiles (SSP), and computed tomography-based body composition data (BC). Methods: We utilized ML models for subtyping adrenal diseases within a cohort of 641 patients (comprising MACS=141, ACS=64, PA=265, PPGL=78, and NFA=93), incorporating 37 CL, 64 SSP, and 15 BC features. Patients were randomly split into training and test cohorts at a 4:1 ratio, with each disease proportionally represented. The ML-based pipeline involved ML model selection, outlier detection, feature selection, and re-sampling algorithm. An ML model was developed to classify each of the five distinct adrenal diseases individually. In addition, separate ML models were also developed and optimized for individual comparisons between NFA and each of the other four diseases. The performance was evaluated using balanced accuracy, sensitivity, specificity, area under the curve (AUC), and F1 score. Results: The OneVsRest Classifier exhibited the best performance in the multi-class classification of the five adrenal diseases, achieving a balanced accuracy (0.78), sensitivity (0.77), specificity (0.93), and an AUC of 0.89. In this process, 10 CL features and 39 SSP features were utilized, whereas BC did not yield significant features. Using only SSP, the ML model achieved a balanced accuracy of 0.71 and an AUC of 0.89, whereas the ML model using only CL demonstrated a relatively lower accuracy of 0.58 and an AUC of 0.82. In models comparing MACS, ACS, PA, and PPGL with NFAs, the accuracies were 0.85, 0.94, 0.78, and 0.86, respectively, with AUCs of 0.96, 0.99, 0.90, and 0.94, respectively. The ML model differentiating between NFA and the other functioning adrenal diseases exhibited an accuracy of 0.75 and an AUC of 0.79 Conclusion: We have developed a highly sensitive and specific one-step ML model for differentiating adrenal diseases using CL, SSP, and BC. This approach is anticipated to facilitate precise diagnoses of adrenal nodules, thereby enhancing patient care and improving clinical outcomes.
Presentation: 6/2/2024
5131 Vanishing Act: Uncommon Case Of Pheochromocytoma With Positive Biochemical Work-up With Absence Of Tumor And Medullary Tissue
Authors: Chan Nicole , Kaufman Ronald , Farhat Nada , Kim Grace Y 
Published: October 2024

Abstract
Disclosure: N. Chan: None. R. Kaufman: None. N. Farhat: None. G.Y. Kim: None.
Pheochromocytomas are rare neuroendocrine tumors derived from adrenal chromaffin cells that result in hyperactivity of the sympathetic nervous system. In the United States, they occur in about 0.05% to 0.1% patients with sustained hypertension. We are presenting a case of a patient with biochemical evidence of pheochromocytoma, but surgical pathology revealed absence of tumor and residual normal medulla.80-year-old female with a history of follicular lymphoma and hypertension presented for evaluation of an incidental right-sided adrenal nodule noted on a PET-CT. She had a history of hypertension managed on lisinopril 40 mg, amlodipine 10 mg and atenolol 100 mg daily. She denied a history of weakness, dizziness, headaches, palpitations, or easy bruising. Workup was negative for primary aldosteronism and Cushing’s Syndrome. Plasma normetanephrines was high at 675.7 pg/mL (0-285.2 pg/mL). 24-hour urinary normetanephrine was also elevated at 562 ug/g (0-400 ug/g). CT abdomen and pelvis with and without contrast revealed a 1.4 cm right adrenal nodule approximately 16 Hounsfield units. These findings were suggestive of pheochromocytoma. She was pre-treated with alpha blockade and subsequently had a robotically assisted laparoscopic right adrenalectomy. Postoperatively, the patient was hypotensive and required vasopressors. She was eventually weaned off vasopressors and discharged on lisinopril 20 mg daily. Soon after discharge, the patient was complaining of hypotension and this dose was halved. 4 weeks postoperatively, laboratory evaluation showed normalization of plasma normetanephrines to 0.83 nmol/L (0-0.89 nmol/L). Surgical pathology revealed an infarcted benign adrenal cortical proliferative lesion with background of cortical hyperplasia, fibrotic adrenal medullary region, and hemosiderin deposition. There was no identifiable medullary neoplasm and no residual medulla.Pheochromocytomas are highly vascular tumors as angiogenesis is a critical step in tumor growth, which can lead to frequent infarcts. This case shows classic biochemical evidence of pheochromocytoma. Interestingly, the resulting clinical examination displayed obliteration of the medullary tissue. Complete disappearance of both tumor and residual normal medulla is extraordinarily rare. The pre-operative hormonal levels in association with findings of complete absence of neoplastic and nonneoplastic medullary tissue cannot be explained.
Presentation: 6/1/2024