bluuebunny/crossref_metadata_2025_split huggingface dataset:
FRI317 Simultaneous Occurrence Of A Pineal Germ Cell Tumor And Pituitary Macroprolactinoma In A Young Male
Authors: Monsour Elio Paul , Badour Sanaa , Munoz Pena Juan Manuel , Donahoo William Troy 
Published: October 2023

Abstract
Disclosure: E.P. Monsour: None. S. Badour: None. J.M. Munoz Pena: None. W.T. Donahoo: None.
Background: Germ Cell Tumors (GCTs) are a group of heterogeneous tumors that derive from pluripotent germ cells and originate in the yolk sac of the embryo in early gestation. They account for 10% of all central nervous system (CNS) GCTs, primarily occurring in the pediatric and adolescent populations. The co-existence of macroprolactinoma and a mixed CNS GCT is extremely rare, with an unknown worldwide prevalence. Here we report a case of a pituitary macroprolactinoma occurring with a separate primary CNS mixed GCT. Clinical Case: A 19-year-old male presented with a 2-month history of headaches associated with nausea and vomiting. Relevant physical exam findings included decreased visual acuity, absent facial and axillary hair, and prominence of the left testis. Brain MRI demonstrated a pituitary adenoma measuring 3.5 x 2.3 x 3.2 cm extending into the cavernous sinus and an enhancing necrotic lesion in the third ventricle measuring 5.0 x 3.7 x 4.9 cm. Testicular ultrasound revealed two sub-centimeter ill-defined masses in the left testis. Pre-operative labs showed intact thyroid, adrenal, and growth hormone axis. He had a low total testosterone [14.2 ng/dl, reference range (RR): 230-800 ng/dL] with inappropriately normal gonadotrophins and markedly elevated prolactin (PRL) level (2,517 ng/mL, RR &lt;14 ng/mL). Testicular tumor marker, alpha-fetoprotein, was elevated (15.3 ng/dL, RR &lt;9 ng/dL). He was started on glucocorticoids for vasogenic edema and cabergoline twice weekly. He underwent left orchiectomy and subsequent pineal tumor resection. Testicular tumor pathology was consistent with teratoma and a well-differential neuroendocrine tumor with negative margins. Pineal tumor pathology was diagnostic of a mixed GCT. Despite optimal medical therapy, the patient ultimately opted to pursue transsphenoidal tumor resection. Pituitary mass pathology was compatible with a pituitary adenoma, immunoreactive for PRL. Post-operative PRL levels decreased to 6 ng/dL. He achieved a surgical cure without the need to restart dopamine agonist therapy. Conclusion: Simultaneous occurrence of primary brain tumors with different pathologies is estimated to be ∼0.9% of all brain tumors. To our knowledge, only one other case report described an adolescent with a craniopharyngioma and intracranial GCT. This is the first report in the literature of a pituitary macroprolactinoma synchronous with a mixed CNS GCT, and testicular well-differential neuroendocrine tumor with teratoma.
Presentation: Friday, June 16, 2023
FRI318 A Case Of Mixed Growth Hormone And Prolactin-secreting Giant Pituitary Macroadenoma
Authors: Khan Aysha , Alpertunga Ipek , Kantorovich Vitaly 
Published: October 2023

Abstract
Disclosure: A. Khan: None. I. Alpertunga: None. V. Kantorovich: None.
Introduction Co-secretion of prolactin (PRL) and growth hormone (GH) from a single pituitary adenoma is an established entity in the medical literature and present in about 5% of the cases. Guidelines recommend to obtain an insulin-like growth factor-1 (IGF-1) in patients with prolactinoma at the time of initial diagnosis. Our case describes the management of a giant pituitary macroadenoma co-secreting PRL and GH. Case A 20-year-old male with history significant for schizophrenia presented with visual disturbances and discharge from his left nipple. His prolactin level (PRL) was noted to be elevated at 12,396 (4.0-15.2) ng/ml on work-up during recent prior investigation. On physical exam, he had bilateral temporal visual field deficit as well as dysfunction in upward gaze of left eye. Head imaging showed a 7.5 x 6.2 x 5 cm associated with remodeling of the sella turcica and expanding into the suprasellar space and significant mass-effect on the adjacent frontal lobes bilaterally, mild mass-effect on the adjacent optic chiasm with significant surrounding vasogenic edema and compressing the bilateral anterior cerebral arteries. Patient was started on low dose cabergoline in caution of prevention of possibility of rapid response-related significant volume contraction of the tumor with possibility of reactive edema. The dose of cabergoline was rapidly titrated up to 3 mg bi-weekly with minimal prolactin fluctuation of PRL between 12,000 and 16,000 ng/ml. Because of mild atypical acromegalic facial features, patient had GH axis assessment. IGF-1 level came back elevated at 529 (83-456) ng/ml with a high IGF Binding Protein- 3 of 8.9 (2.9-7.2) mg/l. He was started on octreotide titrated up to a dose of 150 mcg three times daily. Repeat MRI brain did not show tumor shrinkage. Subsequently, patient underwent bifrontal craniotomy for resection/debulking of parasellar tumor. PRL dropped to 931 ng/ml immediately after surgery. At the time of writing, pathology of the tumor and repeat IGF-1 level is pending. Patient was monitored closely in the ICU with a steroid taper postoperatively. Discussion Dopamine agonists are the first-line therapy for medical treatment of hyperprolactinemia but can also suppress GH and IGF-1 levels. However, in acromegaly, cabergoline is utilized mostly for small adenomas with mild elevation of IGF-1. In our case, we decided to use a combination of cabergoline and somatostatin receptor analog, octreotide. However, given the giant adenoma with very high PRL and IGF-1 levels, patient showed a poor response to medical therapy, and given the impending threat to patient’s vision, he underwent surgical debulking. Our case highlights the management of a giant, mixed hormone-secreting pituitary adenoma.
Presentation: Friday, June 16, 2023
THU147 Peritoneal Dialysis To Improve Hyperglycemic Hyperosmolar State In Child With Renal Failure
Authors: Choudhari Pooja , Mootha Sudha 
Published: October 2023

Abstract
Disclosure: P. Choudhari: None. S. Mootha: None.
Background: Hyperglycemic hyperosmolar state (HHS) without ketosis is rare in children. While the crux of management of HHS is extensive hydration followed by insulin, this management can be difficult for children with kidney failure. Clinical Case: A 6-year-old female with complex medical history including Jeune syndrome (asphyxiating thoracic dystrophy), end stage renal disease status post kidney transplant, currently with rejection, now on peritoneal dialysis (PD), and history of steroid induced hyperglycemia was admitted for altered mental status. Several days prior to the current admission, she was admitted for an upper respiratory infection and found to have hyponatremia, which improved with fluid restriction and sodium supplementation. Subsequent worsening of hyponatremia led to adjustments in her PD to include higher amounts of dextrose in the dialysate (1.5% to 2.5%). At the current admission, she was found to have a blood glucose of 2096 mg/dL, uncorrected sodium of 121 mg/dL, corrected to 153 mg/dL, serum osmolality of 406 mOsm/kg, and hemoglobin A1c of 11.3%. She had mild acidemia and beta-hydroxybutyrate of 0.5 mmol/L. She was transferred to the Pediatric Intensive Care Unit (PICU) and intubated given her waxing and waning mental status and complex history. Since she was anuric, fluid resuscitation was carefully managed. She received a total of 14 mL/kg normal saline boluses, which lowered blood glucose by about 240 mg/dL. Multidisciplinary conversations between PICU, Endocrinology, and Nephrology culminated with a plan to attempt to slowly reduce blood glucose levels via PD prior to using regular insulin infusion. 24-hour PD was restarted with lower dextrose in the dialysate (1.5%). Due to persistent hypotension, she required norepinephrine infusion and stress dose hydrocortisone. With PD, blood glucose levels decreased by 20-30 mL/kg/hr in the first day. Regular insulin infusion was started, while continuing 24-hour PD, after blood glucoses stagnated at 1400-1500 mg/dL. After 4-5 days of PD and insulin infusion, the child’s blood glucoses decreased to 300-400 mg/dL. After tolerating her g-tube feeds, she was transitioned to an insulin pump as she was requiring high doses of insulin (1.8 units/kg/hr). Conclusion: We present a child with complex medical history, including history of steroid induced hyperglycemia, end stage renal disease status post renal transplant, anuria, currently on PD, who developed hyperglycemic hyperosmolar state (HHS). PD helped to lower her significant hyperglycemia, initially without insulin. There are few case reports on the use of PD or hemodialysis to improve hyperglycemia or hypernatremia in children with renal failure. This case demonstrates dialysis can be an effective strategy to attempt for children in a hyperosmolar state in whom aggressive fluid management can be detrimental.
Presentation: Thursday, June 15, 2023
THU189 Complex Treatment Of Recurrent Gynecomastia In An Affirmed Male With 46XX Ovotesticular Difference Of Sexual Differentiation (DSD).
Authors: Jumani Sanjay , Shimy Kim , Casella Daniel , Long Jessica , Gomez-Lobo Veronica 
Published: October 2023

Abstract
Disclosure: S. Jumani: None. K. Shimy: None. D. Casella: None. J. Long: None. V. Gomez-Lobo: None.
Introduction: Identifying gonads with ovarian and testicular components (ovotestis) can be difficult, despite multimodal imaging, biopsy, and surgical visualization. Here, we describe an unexpected identification of an ovotestis following the initiation of tamoxifen in a patient with 46XX Ovotesticular Difference of Sexual Differentiation (DSD). Case: A 14YO affirmed male with 46XX Ovotesticular DSD presented to our clinic for management of recurrent gynecomastia. At birth, he had ambiguous genitalia consistent with a under-virilized phallus, dorsally hooded foreskin, bifid scrotum, proximal hypospadias, palpable left gonad and non-palpable right gonad. He had a “normal” testosterone (i.e. presumably in male range for mini-puberty, though specific values predate available records). Limited genetic testing showed a karyotype of 46XX and negative SRY. He underwent a laparoscopy during which a right ovary, Müllerian tissue, and a small amount of vaginal tissue were identified and removed. A biopsy of the left gonad showed Sertoli cells without germinal epithelium. In adolescence, he developed progressive gynecomastia, with tanner 3 breasts by 12y6m. Treatment with anastrozole was unsuccessful, and he underwent a reduction mammaplasty at 13y6m. Unfortunately, the gynecomastia recurred shortly thereafter, and he was referred to our institution. At 14y7m bloodwork revealed a robust testosterone (543ng/dL), elevated estradiol (106 pg/mL, ref: 10-36), and estrone (71pg/mL, ref: 15-45). A trial of 20 mg tamoxifen daily was initiated. After 1 month, he developed scrotal pain suspicious for torsion and was taken to the operating room. Scrotal exploration revealed a hemorrhagic cyst at the superior pole of the gonad and an external appearance consistent with an ovo-testis. The hemorrhagic cyst was drained, and the remainder of the gonad left in situ. His pain resolved post-operatively however he returned 3 weeks later with scrotal pain and a recurrent hemorrhagic cyst on ultrasound. Tamoxifen was discontinued, and treatment with a Gonadotropin Releasing Hormone agonist (GnRHa) and concomitant testosterone replacement was initiated. This medication change led to resolution of the gonadal cysts. Conclusion: Initiation of tamoxifen as treatment for gynecomastia resulted in recurrent hemorrhagic cysts and the identification of ovarian tissue in a descended gonad which was presumed to be a biopsy proven testis. Recurrent hemorrhagic cysts in the ovotestis were likely due to physiologic stimulation and tamoxifen1 as well as elevated estradiol. Treatment with a GnRHa and testosterone offered a reversible, non-surgical method to suppress endogenous sex hormones contributing to gynecomastia while continuing male pubertal development. 1.PMID: 16254040.
Presentation: Thursday, June 15, 2023
THU619 Atypical Clinical Presentation Of Adrenal Hemorrhage
Authors: Madsen-Barbosa Thayse Lozovoy , Antoni Fidini Hayati , Ghazi Adline 
Published: October 2023

Abstract
Disclosure: T.L. Madsen-Barbosa: None. F.H. Antoni: None. A. Ghazi: None.
Introduction: Adrenal hemorrhage (AH) is an uncommon condition that is challenging to diagnose due to its nonspecific findings. We illustrate a case of AH that presented with acute clinical deterioration and had catecholamine overproduction. Case: A 58-year-old woman with a history of migraine headaches and prediabetes presented to the emergency department for “the worst headache of her life” and palpitations of 4 hours duration. On admission, her blood pressure was 224/139 mmHg and heart rate was 102 bpm. Her physical examination was significant for diaphoresis and active vomiting. Initial laboratory tests showed hyperglycemia and leukocytosis. Her ECG, non-contrast CT scan of the head and CT angiography of the head and neck were unremarkable. Her hospital course was complicated by recurrent hypertensive episodes, arrhythmias including Torsades de pointes, and left flank pain. A CT chest, abdomen, and pelvis revealed a large left retroperitoneal hemorrhagic collection which seemed to arise from the inferior aspect of the left adrenal gland. She subsequently underwent successful IR embolization of the left inferior suprarenal artery. Further workup showed elevated urinary fractionated metanephrine and normetanephrine levels, as well as elevated levels of plasma free metanephrine and normetanephrine. Given concern for pheochromocytoma, phenoxybenzamine was started. A repeat CT abdomen after six days demonstrated shrinkage in hematoma and a 7 mm hypoattenuating nodule arising from the medial limb of the left adrenal gland. Outpatient follow-up CT abdomen showed left adrenal thickening with a 2.3 cm nodular area with 78.9 Hounsfield units and absolute washout of 60.9% suggestive of adenoma. After one month, repeat catecholamine levels normalized. Discussion: The clinical manifestation of AH varies widely. Acute adrenal insufficiency is seen if bilateral glands and 90% of the cortices are compromised. However, unilateral AH is most often asymptomatic - found incidentally on imaging. Having elevated catecholamines is described in 30% of patients in one case series. Differentiating spontaneous AH from hemorrhagic adrenal tumors is also a dilemma. Among the hemorrhagic adrenal masses, pheochromocytoma is the most common. Conclusion: This case highlights that clinicians should be aware of clinical manifestations of catecholamine excess when evaluating patients with AH. A catecholamine producing adrenal mass (pheochromocytoma) should also be ruled out in these situations. Reference:1- Elhassan YS, Ronchi C, Wijewickrama P, Balvedeweg SE. Approach to the patient with adrenal hemorrhage. J Clin Endocrinol Metab. 2023, 108 (4):995-1006.2- Karwacka IM, Obolonczyk L, Sworczak K. Adrenal hemorrhage: a single center experience and literature review. Adv Clin Exp Med. 2018; 27 (5): 681-687.
Presentation: Thursday, June 15, 2023
FRI601 Unique Considerations For Workup And Management Of An 11 Year Old Male With Concern For A Neuroendocrine Tumor
Authors: Gunaratne Naiomi C , Deplewski Dianne 
Published: October 2023

Abstract
Disclosure: N.C. Gunaratne: None. D. Deplewski: None.
Background: Pheochromocytoma/paragangliomas are very rare in children. In patients who are undergoing work up for paragangliomas and pheochromocytomas, special considerations need to be made to distinguish neuroendocrine tumor findings from medication and other symptoms. Clinical Case: An 11 yo male with past medical history of migraines presented with new onset seizure, hypertension and tachycardia. Initial exam was remarkable for severe lower back pain, which eventually progressed to generalized pain. His tachycardia and hypertension were initially thought to be related to his pain, but were not improved with escalating pain and intermittent anti-hypertensive medications – leading to admission to the intensive care unit. He was started on lisinopril and metoprolol, but continued to have persistent tachycardia and hypertension.Initial work up included an echocardiogram revealing concentric left ventricular hypertrophy suggesting long-standing hypertension, MRI brain significant for Posterior Reversible Encephalopathy Syndrome, MRI abdomen and spine with no significant findings, and elevated urine metanephrines. Urine and serum catecholamines and serum metanephrines were also elevated, but not at levels typical of neuroendocrine tumors. Due to concern for possible pheochromocytoma/paraganglioma, patient was started on alpha-blocker phenoxybenzamine, and had marked improvement in hypertension. Patient was also started on scheduled gabapentin one day after initiation of phenoxybenzamine and noted to have some improvement in pain – making cause of acute improvement in blood pressure less clear. Patient underwent Ga-68 DOTATATE imaging that was significant for “mild to moderately increased activity within the left adrenal gland,” but no distinct lesion was appreciated on scan. After discussion with endocrine surgery, plan was made to monitor the patient in outpatient setting with serial plasma free metanephrines and chromogranin A while continuing phenoxybenzamine. Conclusion: Pheochromocytoma/paragangliomas are very rare in children. In patients who are undergoing work up for paragangliomas and pheochromocytomas, special considerations need to be made when patient has concomitant pain or is on beta-blockers. Ability to interpret urine/plasma metanephrines and catecholamine levels while on beta-blockers and other pharmacologic agents is difficult due to known iatrogenic alterations of these levels. Management for patients with elevated metanephrines without a localized neuroendocrine tumor can consist of pharmacologic intervention with alpha-blockers and close monitoring with serial labs.
Presentation: Friday, June 16, 2023
OR15-06 Measuring Hepatic Steatosis With Transient Elastography In Adolescents With Obesity
Authors: Sundararajan Divya , Lo Jaclyn S , Morelli Nazeen , Garcia-Reyes Yesenia , Ware Meredith A , Rahat Haseeb , Sundaram Shikha , Severn Cameron , Pyle Laura L , Tas Emir , Cree Melanie G 
Published: October 2023

Abstract
Disclosure: D. Sundararajan: None. J.S. Lo: None. N. Morelli: None. Y. Garcia-Reyes: None. M.A. Ware: None. H. Rahat: None. S. Sundaram: None. C. Severn: None. L.L. Pyle: None. E. Tas: None. M.G. Cree: Consulting Fee; Self; Pollie, Inc. Research Investigator; Self; Amino Corp.
Obesity affects nearly 20% of adolescents in the United States. Nonalcoholic fatty liver disease (NAFLD) is a common comorbidity, affecting between 40-70% of youth with obesity, thus approximately 10% of United States youth. Traditional diagnostic methods for NAFLD are expensive or invasive, and recently, transient elastography has been used to diagnose hepatic steatosis (HS). We sought to determine if transient elastography accurately diagnosed HS as compared to MRI in adolescents with obesity. Baseline data from adolescents (age 12-18 years) with obesity studied in 3 clinical trials were included (NCT03919929, NCT03717935, NCT04342390). Liver fat was assessed with 2 methods: MRI proton density fat fraction and transient elastography controlled attenuation parameter (CAP) score. Demographics, anthropometrics, liver scans and fasting serum laboratory measures were collected. HS was defined as MRI fat fraction ≥ 5.0%. Group data means, standard deviation, and ranges were calculated, and CAP for those with and without HS by MRI were compared with Mann Whitney U tests. Receiver operator curves (ROC) for CAP were generated based on MRI-determined HS. Data from 84 adolescents (age 15.6 ± 1.4 years, BMI 36.5 ± 5.96 kg/m2, 81% female) were included. Fifty-one had HS by MRI, with liver fat % different by HS status [9.3 (6.7, 14.0)% HS vs 3.1 (2.2, 3.9); p &lt; 0.001]. CAP scores were different by MRI based on HS status [293 (267, 325) HS vs 267 (248, 282); p = 0.012]. With the recommended CAP cutoff for HS of 241, there was only a 59% concordance of HS status between the MRI and CAP. For CAP, ROC for HS was significant (r = 0.66, p = 0.012) and the Youden index had a sensitivity of 70.0% (56.3-80.9) and a specificity of 62% (42.3-74.5) at a CAP score of &gt; 270. Additionally, BMI was not significantly related to hepatic fat (by MRI), AUC (0.44, 0.69, p-value = 0.323), or to CAP-determined HS, AUC 0.613 (0.425-0.802, p-value = 0.2686). In adolescents with significant obesity, the transient elastography CAP is not an accurate measure of HS compared to MRI. A CAP score of 270 to identify HS is higher than previously described in pediatrics, but may be needed in youth with obesity to improve accuracy.
Presentation: Friday, June 16, 2023
THU649 PCOS Phenotype In Unselected Populations (P-PUP) Study: Defining And Comparing Hirsutism Across Various Ethnic Groups In Unselected Populations
Authors: Kiconco Sylvia , Joham Anju Elizabeth , Suturina Larisa V , Zhao Xiaomiao , Gambineri Alessandra , Tehrani Fahimeh Ramezani , Yildiz Bulent Okan , Kim Jinju , Teede Helena Jane , Azziz Ricardo 
Published: October 2023

Abstract
Disclosure: S. Kiconco: None. A.E. Joham: None. L.V. Suturina: None. X. Zhao: None. A. Gambineri: None. F.R. Tehrani: None. B.O. Yildiz: None. J. Kim: None. H.J. Teede: None. R. Azziz: None.
Introduction Hirsutism is defined as the male pattern distribution of terminal hair in a female. It is one of the diagnostic features of polycystic ovary syndrome (PCOS), reflecting clinical hyperandrogenism, as measured by the modified Ferriman-Gallwey score (mFG). However, whether the mFG scores and the normative cut-off varies by ethnicity is unclear. We aimed to describe and compare the distribution of mFG score in different ethnic groups from eight countries using data from unselected (medically unbiased) populations to determine and compare the normative cut-off values between the different ethnic groups and the prevalence of hirsutism thereby defined. Methods We utilised data of 10,919 participants aged 15 to 80 years. Data were obtained from epidemiologic studies assessing unselected women conducted in different countries including China, Iran, Italy, Nigeria, Russia, South Korea, Turkey, and the United States of America. The distribution of mFG between ethnic groups was determined descriptively as medians. Cluster analysis (k means, k=2) and the receiver operating characteristics curve were used to determine the normative cut-offs for the mFG score. All medians, cut-offs and percentile values were compared between ethnic groups using the Kruskal-Wallis test at a 95% level of significance. Results Our data consisted of 11 ethnic groups from 8 countries. The Persian (4.8±4.7) and Italian (4.8±4.7) populations had higher median mFG scores than the rest of the ethnic groups, whilst Asian populations (Chinese, Korean and Buryat) had the lowest scores. Overall, the median distribution of mFG differed significantly by ethnicity. Using cluster analysis, the mFG cut offs range from 4 to 9, with the Persian ethnic group having the highest, followed by the Italian group, with cut-offs differing by ethnicity overall; however cut offs are similar for Black American, Turkish, Asian and Russian groups. Conclusion The distribution and normal cut off values for hirsutism differ significantly by ethnicity, with the Persian population having the highest cut offs. Ethnicity needs to be considered when assessing women for clinical hyperandrogenism.
Presentation: Thursday, June 15, 2023