bluuebunny/crossref_metadata_2025_split huggingface dataset:
ODP335 Mutual Effects of Orexin and BMPs on Gonadotropin Expression by Mouse Gonadotrope LβT2 Cells
Authors: Hirata Shinichi , Nakayama Nanako , Soejima Yoshiaki , Iwata Nahoko , Nakano Yasuhiro , Yamamoto Koichiro , Suyama Atsuhito , Nada Takahiro , Fujisawa Satoshi , Otsuka Fumio 
Published: November 2022

Abstract
Orexins are neuropeptides that express primarily in the hypothalamus and are produced in two isoforms, orexin A and orexin B. There are two kinds of G protein coupled receptors, orexin type 1 (OX1R) and type 2 (OX2R) receptors. Orexin has been reported to have key roles on sleep-wake regulation and feeding behavior in the central nervous system, whereas its receptors are also expressed in peripheral tissues including the endocrine organs, and orexin affects the regulation of the endocrine system. In our previous experiments, we have revealed the impact of orexins on anterior pituitary functions. For instance, we reported that orexin A plays an inhibitory role in prolactin production through the suppression of endogenous bone morphogenetic protein (BMP) activity in rat pituitary lactotrope GH3 cells. It was also reported that orexin A enhances pro-opiomelanocortin (POMC) transcription by upregulating corticotropin-releasing hormone (CRH) receptor signaling and by downregulating BMP-Smad signaling in mouse corticotope AtT20 cells. However, the effects of orexin on the endocrine function regarding gonadotrope cells remain unclear. We have recently uncovered that core clock genes and BMPs have mutual effects on luteinizing hormone (LH) expression in a phase-dependent manner by mouse gonadotrope LβT2 cells. In the present study, we investigated the effect of orexin on pituitary gonadotropin expression using LβT2 cells, which express OX1R and OX2R, by focusing on the functional involvement of BMP system and clock genes. It was revealed that orexin A stimulation increased LHβ and FSHβ mRNA expression in a concentration-responsive manner in the absence of GnRH, and interestingly, GnRH co-treatment further upregulated LHβ mRNA expression in LβT2 cells. Regarding the interrelationships between the signalings of orexin and BMPs, it was also revealed that orexin A pretreatment enhanced the BMP receptor signaling detected as the Smad1/5/9 phosphorylation, indicating that orexin enables to upregulate the BMP actions in LβT2 cells. In our previous studies, we reported that several BMP ligands such as BMP-6, -7 and 15 expressed in LβT2 cells can promote gonadotropin transcription, in which BMP-6 regulates GnRH-stimulated LH expression by modulating the sensitivity to somatostatin analogs. Based on the present results, it was implied that endogenous orexin can be functionally involved in the underlying mechanisms of gonadotropin expression. Collectively, orexin enhances gonadotropin expression by regulating BMP signaling in gonadotrope cells. Here, we will also discuss functional involvement of clock genes in the regulatory system of gonadotropin secretion induced by orexin and BMPs.
Presentation: No date and time listed
ODP345 Pituitary Metastasis from Clear Cell Renal Cell Carcinoma
Authors: Cristiano Elizabeth , Pagadala Prathyusha , Crow Hanna 
Published: November 2022

Abstract
A 63-year-old female presented with a two month history of intermittent diplopia and headaches. Her past medical history was relevant for renal carcinoma status post right nephrectomy four years prior, she did not receive any adjuvant therapy. She underwent MRI brain which showed a 2.1×2. 0×1.6 cm sellar/suprasellar enhancing mass with mass effect on the optic chiasm and extending into the right cavernous sinus, consistent with pituitary macroadenoma. Visual field testing was also done showing mild bitemporal quadrantanopia. Lab evaluation did not show evidence of pituitary hormone excess or deficiency. Serum sodium was normal, IGF-1 117 ng/ml (reference range 35-201 ng/ml), GH 0.19 ng/ml (0. 01 -3.61 ng/ml), alpha subunit 0.4 ng/ml (&lt;1.8 in postmenopausal females), ACTH 15 pg/ml (7-63 pg/ml), prolactin 20.8 ng/ml (3.3 -26.7 ng/ml), TSH 2.36 MCU/ml (0.35-5 MCU/ml) and FT4 0.9 ng/dl (0.6 -1.6 ng/dl). Neurosurgery evaluated the patient and she underwent transsphenoidal surgery. Her postoperative period was unremarkable without evidence of diabetes insipidus or secondary adrenal insufficiency. Final pathology revealed clear cell lesion/carcinoma consistent with metastatic renal cell carcinoma. Transcription marker testing was positive for SF1, consistent with a gonadotroph pituitary adenoma.
Presentation: No date and time listed
ODP361 A Case of Panhypopituitarism in the Setting of Metastatic Large Cell Neuroendocrine Carcinoma
Authors: Engle Kelly , Hupfeld Chistopher 
Published: November 2022

Abstract

Introduction
Metastasis to the pituitary gland is an uncommon cause of panhypopituitarism. The pituitary gland is an infrequent site overall for metastasis, with breast and lung cancer as most often found primary sites. Large cell neuroendocrine carcinoma is a rare form of lung cancer, and while metastasis to brain is not uncommon is this form of cancer, metastasis to the pituitary gland has rarely been reported.

Clinical Case
Patient was a 64 year old male with history of homelessness and substance use who had repeated hospitalizations for right sided pneumonia. Eventually bronchoscopy was performed, and tissue obtained via biopsy revealed a high-grade large cell neuroendocrine carcinoma. Initial brain imaging noted extensive brain metastases with numerous enhancing lesions in the cortex, as well as a 13×9×8 mm suprasellar mass abutting the optic chiasm. Thickening of the infundibulum was also noted. Lab testing demonstrated hypopituitarism with free thyroxine 0.52 (reference 0.93-1.7) ng/dL, thyroid-stimulating hormone 0.11 (2.7-4.2) ulU/mL, sodium 139 (135-147) mEq/L, total testosterone &lt;0.1 (2.8-8) ng/ml, follicular-stimulating hormone 0.4 mlU/ml, lutenizing hormone &lt;0.10 mlU/ml, prolactin 25.7 (4-15.2) ng/ml, cortisol &lt;2 (3-12) ug/dL, and adrenocorticotropic hormone &lt;5 (6-50) pg/mL. Diabetes insipidus was not present. Metastatic cancer was treated with dexamethasone and atezolizumab plus carboplatin and etoposide. Panhypopituitarism was treated with levothyroxine, testosterone replacement, and glucocorticoid replacement. One month later, patient represented with shortness of breath and headache. Repeat brain imaging showed an enhancing sellar mass measuring 21×15×11 mm extending superiorly to involve the pituitary stalk and hypothalamus. The extending suprasellar mass was noted to have enhancement and characteristics akin to remainder of brain metastases. The patient passed away from complications of metastatic carcinoma about three months after initial biopsy proven diagnosis.

Conclusion
This patient developed extensive brain metastases from pulmonary large cell neuroendocrine carcinoma, which included a metastasis in the sella resulting in panhypopituitarism. While pituitary metastases from primary cancer are very rare events (with lung and breast carcinoma the most frequent sources), only a single case report exists describing a pituitary metastasis from a large cell neuroendocrine carcinoma. This case is also an important reminder to consider the pretreatment anatomy of the sella in patients subsequently exposed to immunomodulating chemotherapy, as a mass lesion seen during treatment may represent a preexisting metastasis rather than treatment-induced hypophysitis.
Presentation: No date and time listed
ODP362 An Unexpected Finding of Adamantinomatous Type Craniopharyngioma in the Late Seventh Decade of Life: a case report
Authors: Ahmed Ammar , Biswas Suman , Tan Zi , Jahshan Bishara , Meedani Hassan E 
Published: November 2022

Abstract

Background
Craniopharyngioma is a rare solid, mixed solid, and benign cystic neoplasm derived from remnants of Rathke's pouch from the sellar region and its surroundings parasellar area. It mainly has two major histopathological subtypes, Adamantinomatous and Papillary. The adamantinomatous subtype is more common in children, and the papillary subtype is more common in adults. Clinical case: A 66-year-old male with no known endocrinological disorder presented with progressive fatigue, decreased oral intake, lightheadedness, nausea, visual changes, low libido, myalgia, nausea, vomiting, polyurea, headache, visual field defects, and seizure-like activities requiring hospital admission. The endocrinological workup revealed a deranged HPA axis (panhypopituitarism). MRI revealed 3 cm macroadenoma within the pituitary gland. An endonasal transsphenoidal resection of pituitary mass resection with abdominal fat graft was done as definitive treatment along with correction of the multiple endocrinological derangements. The surgical tissue biopsy revealed a rare with adamantinomatous type Craniopharyngioma WHO Grade I. He was discharged on testosterone supplements, hydrocortisone, levothyroxine, and Desmopressin with significant improvement in his symptoms.

Conclusion
Craniopharyngioma (CP) is a rare benign intracranial mass (incidence 0.5-2/100000) that can present with extensive endocrinological dysfunction. However, due to the lack of patient-level data and reporting of adult-onset craniopharyngiomas, it can be easily missed during the initial presentation. Not considering craniopharyngioma in differential diagnoses may result in delayed diagnosis and adverse outcomes. Although the papillary CP type is more common among adults, occasionally, adamantinomatous type CP may occur in this group of patients.
Presentation: No date and time listed
ODP618 The profile of patients with ACTH-secreting pituitary adenomas and subclinical pituitary apoplexy
Authors: Tatsi Christina , Saidkhodjaeva Lola , Flippo Chelsi , Stratakis Constantine 
Published: November 2022

Abstract

Background
Subclinical hemorrhage or necrosis of pituitary adenomas (PAs), often defined as subclinical pituitary apoplexy (SPA), presents in up to 20% of all PAs and is more common in large prolactinomas. SPA in corticotropinomas in patients with Cushing disease (CD) is not common and the clinical and biochemical characteristics of patients with CD and SPA are not known. Subjects and methods: Pediatric patients with confirmed CD diagnosed between 2005-2021 with available MRI images before any surgical intervention were included in the study (n= 171). SPA diagnosis was based on MRI findings. Although MRI sequences and machines were not same for all patients and varied over time, the results were based on possible signs of hemorrhage on the available images, but smaller areas of bleeding or necrosis may have been missed. Statistical analyses were performed with Wilcoxon signed-rank test, chi-square or Fisher's exact test, and Cox proportional hazard as appropriate. Results are shown as median [Q1, Q3], count (percentage) and odds ratio (OR) (95% confidence intervals, 95%CI).

Results
Out of 171 patients, 12 patients (7%) were found to have imaging characteristics of possible hemorrhage or intratumor infarct. Patients with and without SPA were similar in age (median age: 12.5 years [10.6, 15.6] in SPA group vs 13.1 years [10.6, 15.4] in non-SPA group, p= 0.95) and gender distribution (n of female= 5 (41.7%) in SPA group vs 89 (56%) in non-SPA group, p= 0.51). Patients with SPA had shorter duration of disease as noted by changes in their growth chart parameters (median duration: 1. 0 year [1. 0, 2. 0] in SPA group vs 2.5 years [1.5, 3. 0] in non-SPA group, p= 0. 014). Morning and midnight cortisol and 24hour urinary cortisol levels were similar in both groups, but patients with SPA had higher levels of morning ACTH (60.8 pg/mL [43.5, 80.3]) compared to patients without SPA (39.8 pg/mL [28.5, 53.4], p=0. 017). Change of cortisol and ACTH levels during CRH stimulation test were similar between the two groups, but patients with SPA had lower suppression of cortisol after overnight high dose (8mg or weight-based equivalent) dexamethasone suppression test (58. 0% [-85.4, -49.7]) compared to patients without SPA (85.8 [-90.5, -76.8], p= 0. 035). Specifically, patients with SPA had lower chance of passing the threshold of 69% that it suggestive of pituitary source of hypercortisolemia compared to patients without SPA (OR: 0.18, 95%CI: 0. 03-0.95). The odds of remission after surgery and the risk of recurrence after initial remission did not differ based on the presence of SPA.

Conclusion
Patients with CD and sings of hemorrhage or necrosis in their tumor may present with different characteristics on their biochemical evaluation which should be considered during their diagnostic workup, especially in the setting of a discordant dexamethasone suppression test.
Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.
PMON107 Pineal Germinoma with Pituitary Involvement: Long-Term Survival following Treatment with Whole Ventricular Radiation
Authors: Khoury Brandon , Shin Terry , Vietor Nicole , Hoang Thanh , Shakir Mohamed 
Published: November 2022

Abstract

Introduction
Intracranial germinomas usually arise from midline structures such as the pineal gland or suprasellar region. Involvement of both structures occur in 6% of cases. We are reporting long-term follow up of a patient with pineal germinoma with pituitary involvement treated successfully with whole ventricular radiation.

Clinical Case
A 36-year-old man presented with polydipsia and polyuria 12 years ago. He also reported fatigue and cold intolerance. Vital signs: BP 130/75 mmHg, HR 69 bpm. Physical examination demonstrated moist mucous membranes and the rest of the examination was unremarkable. Labs: serum albumin 4.9 g/dL, calcium 9.8 mg/dL, glucose 91 mg/dL, potassium 4.2 mEq/L, sodium 145 mEq/L, and serum osmolality 294 mOsm/kg (reference 275-295). The patient also had multiple urinalyses showing a specific gravity of 1.005. Results of a water deprivation test: urine osmolality 114 mOsm/kg (50-1350) at 0805, 87 mOsm/kg at 0858, 73 mOsm/kg at 0955, 80 mOsm/kg at 1045, 88 mOsm/kg at 1150, and 102 mOsm/kg at 1253. His serum antidiuretic hormone during the water deprivation test was &lt;0.8 pg/mL (ref 0.0-4.7). Since these labs suggested central diabetes insipidus, a pituitary MRI was performed that demonstrated two mass lesions in the region of the cephalad portion of the pituitary stalk, hypothalamus, and optic chiasm as well as in the pituitary gland region. Biopsy of the mass lesions was consistent with germinoma. MRI of the spine revealed no evidence of disseminated disease. Additional labs: TSH 1.88 mIU/L, free thyroxine 0.46 ng/dL, IGF-1 62 ng/mL (109-284), prolactin 25 ng/mL, FSH 1.9 mIU/mL, LH 2.5 mIU/mL, total testosterone 183 ng/dL, and cortisol 3.2 ug/dL. An ACTH stimulation test confirmed central adrenal insufficiency. The patient was treated with radiation therapy to the whole ventricles with for a total dose of 45 Gray per cycle. Follow-up MRI in our patient following treatment demonstrated no evidence of persistent or recurrent disease. However, he continues desmopressin for diabetes insipidus and levothyroxine 88 mcg daily for central hypothyroidism whereas the adrenal insufficiency has completely resolved. The patient has been without disease recurrence for the last 12 years and is doing well with a good quality of life. Radiation therapy alone has been highly effective in the management of intracranial germinomas with an overall survival of up to 85%. However, recent studies have shown that chemotherapy followed by lower dose of complete ventricular radiation is as effective as high dose ventricular radiation for local or bifocal germinomas with lower risk of long-term consequences.

Conclusion
This case illustrates that long-term survival can be achieved in patients with germinomas following appropriate treatments.
Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.
PMON118 Cerebral Palsy Resulting in Hypothalamus Volume Loss and Temperature Dysregulation
Authors: Phan Van , Erenler Feyzer , Lechan Ronald 
Published: November 2022

Abstract

Background
Cerebral palsy is a syndrome caused by a non-progressive brain injury or malformation during early brain development, which can lead to severe physical disability. However, there is limited data on the consequences of cerebral palsy on other crucial brain functions such as hypothalamus and pituitary gland. We report a case of hypothalamic-pituitary involvement in a cerebral palsy patient.

Clinical case
A 28-year-old man with history of quadriplegic cerebral palsy, severe cognitive delay and seizure disorder presented to our clinic with temperature dysregulation for 2 years. He is wheel-chair-bound and non-verbal. His core temperature generally ranges from 96.8o to 97.2oF but occasionally he has had fluctuations as high as 102oF and as low as 88oF associated with extremes in surrounding temperature. At the time of initial evaluation, his temperature was 96.8oF (36°C) and his skin was cool and mottled. He was on levothyroxine replacement for hypothyroidism and thyroid hormone levels were within the normal range (Free T4 of 1.33 ng/dL and Total T3 of 153 ng/dL). Head CT revealed extensive encephalomalacia involving the frontal and temporal lobes, diffuse thinning of the parenchyma of the parietal and occipital lobes with secondary lateral ventricle enlargement, and volume loss in the deep gray nuclei including the hypothalamus. Further evaluation revealed a borderline response to Cortrosyn (16.9 ug/dL at 60 min) but associated with an adequate baseline cortisol of 13.7 ug/dL. IGF-1 and prolactin levels were normal (IGF-1 of 117 ng/mL and prolactin of 7.9 ng/mL), but testosterone was 175 ng/dL associated with an LH of 3.1 mIU/mL and FSH of 10.7 mIU/mL consistent with central hypogonadism. The family was advised to carefully regulate and monitor ambient temperature, and maintain dress appropriate for the surrounding temperature.

Conclusion
Although periventricular white matter lesions are most commonly associated with cerebral palsy resulting in motor dysfunction, deep gray matter lesions are also occasionally documented. These lesions should be recognized, as they could be detrimental to the body. The hypothalamus, with the preoptic area as its main thermoregulatory locus, plays an important role in temperature regulation. It integrates all information from the surface thermoreceptors and local brain temperature then initiates heat production or heat loss mechanisms to help maintain homeostasis. Therefore, volume loss of the hypothalamus can lead to serious consequences such as poikilothermia that can lead to life-threatening hypothermia or hyperthermia.
Keywords: Cerebral palsy, hypothalamus, temperature dysregulation, poikilothermia
Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.
PMON40 A Rare Etiology of Chronic Diarrhea: Zollinger-Ellison Syndrome
Authors: Rashid Hytham , McKinney Nathan , Jernigan Grant , Rhodes Alexander , Nix Emily , Morrissette Charles 
Published: November 2022

Abstract

Introduction
Zollinger-Ellison Syndrome (ZES) is a rare etiology of chronic diarrhea caused by a gastrin-secreting neuroendocrine tumor. Early suspicion can prevent multiple readmissions for recurrent diarrhea thus improving clinical outcomes through prompt diagnosis and treatment, as the following case highlights.

Case presentation
A 74-year-old African-American female with hypertension and chronic atrial fibrillation presented to the ED complaining of watery, non-bloody diarrhea for the past 3 weeks. She reports her symptoms worsened after completing a course of amoxicillin for pharyngitis one week prior. On further questioning, she reported intermittent diarrhea for over 20 years lasting approximately two days per episode. She denied recent travel, sick contacts, pet reptiles, and recent ingestion of undercooked meat. On arrival, she was afebrile and hemodynamically stable. She was admitted for an acute kidney injury with an elevated creatinine of 3.23 mg/dL (N: 0.6-1.1 mg/dL) due to dehydration. Her renal function improved with fluids, but she developed bloody diarrhea for which gastroenterology was consulted. Stool studies were negative for Shiga-toxin, C. difficile, lactoferrin, and parasites. Computed Tomography of the abdomen showed enteritis with fluid noted throughout the colon consistent with colitis. Unprepped colonoscopy revealed blood throughout the length of the colon, suggestive of an upper gastrointestinal bleed. Esophagogastroduodenoscopy (EGD) revealed multiple bleeding duodenal ulcers controlled with hemostasis. Her symptoms improved and she was discharged home on oral pantoprazole, and told to follow up outpatient. Two weeks later, she denied any new episodes of diarrhea, and a random serum gastrin level was elevated at 413 pg/mL (N: &lt;180 pg/mL). Repeat gastrin level off pantoprazole for 7 days was also elevated at 678 pg/mL, and a serum Chromogranin A level was elevated at 629 pg/mL (N: &lt;390 pg/mL). Repeat EGD found her Gastric pH to be 1 with multiple non-bleeding duodenal ulcers. Somatostatin receptor scintigraphy revealed a high intensity signal consistent with a duodenal gastrinoma causing ZES. She elected for surgical resection, and was discharged home on high dose pantoprazole. Three months later, she stated that her diarrhea had completely resolved.

Conclusion
This case confirms ZES as a rare cause of chronic diarrhea in the setting of multiple duodenal ulcers refractory to proton pump inhibitors. Diagnosis can be made with a fasting serum gastrin level &gt;1000 pg/mL or &gt;400 pg/mL with a gastric pH &lt;1. A Secretin Stimulation Testing can also be used. Treatment starts with high dose proton pump inhibitors, octreotide for hormonal regulation, and surgical resection if necessary.
Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.