bluuebunny/crossref_metadata_2025_split huggingface dataset:
SAT-272 Pituitary Macroadenoma Co-Secreting TSH and PRL Responsive to Cabergoline
Authors: Tirumalasetty Saritha , Galagan Robert , Lovre Dragana , David Julia 
Published: May 2020

Abstract
Background: Thyroid-stimulating hormone (TSH) secreting tumors (TSHoma) account for 0.5-2% of all pituitary adenomas with a prevalence of 1-2 cases per million, indicating that TSHomas are very rare. The majority of TSHomas solely secrete TSH however 9.7% co-secrete Prolactin (PRL). We are reporting a case of co-secreting TSH and PRL pituitary macroadenoma responsive to Cabergoline (CAB) Case: A 52 year old multiparous female presented with symptoms of galactorrhea and amenorrhea. Lab investigation revealed elevated PRL 73 ng/mL (n [normal] = 1-24), Total T3 305 ng/dL (n = 71-180), Free T4 (FT4) 2.37 ng/dL (n = 0.6-1.15), TSH 6.09 UIU/mL (n = 0.5-5.0), and α subunit 7.7 ng/mL. Estradiol was low at 16.9 pg/mL and FSH 6.3 MIU/mL LH 1.7 MIU/mL. Visual field testing showed a right nasal step. MRI imaging demonstrated a 21x24x32mm pituitary macroadenoma with optic chiasm distortion. Partial Transsphenoidal surgery (TSS) was performed and immunostaining of tumor tissue was positive for PRL and negative for other pituitary hormones. One month post-surgical MRI revealed 14x17x15mm residual tumor. One month post-op TFTs were normal: TSH 0.99 ng/dL, FT4 0.61 ng/dL; PRL decreased to 34.8 ng/mL. Six month post-op TSH increased to 5.86 UIU/mL, FT4 1.43 ng/dL, and PRL 44.7 ng/mL. Two years post-op TSH 8.06 ng/dL with elevated α subunit 3.4 ng/mL and PRL 56.8 ng/mL. Octreotide was then initiated for TSHoma treatment however she was unable to tolerate the medication due to diarrhea so was switched to CAB. After starting CAB at 0.5mg twice a week, residual sellar mass increased in size to 19.5x16x23mm with TFTs: TSH 5.66 UIU/mL, FT4 2.48 ng/dL. CAB dose was eventually uptitrated to 1mg twice a week. Repeat MRI showed slight decrease in pituitary lesion to 19x21x18mm and downtrending TFTs: TSH 2.28 UIU/mL, FT4 1.17 ng/dL. Discussion: In patients with pituitary tumors associated with elevated PRL and TSH, TSHoma should be part of the differential diagnosis. This patient’s initial lab evaluation with elevated PRL, TSH, FT4, Total T3, and α subunit confirm the diagnosis of a pituitary macroadenoma with co-secretion of PRL and TSH. Elevated PRL, TSH, FT4 and α subunit levels occurred 6 months after partial TSS resection with growing tumor size eventually requiring medical therapy. On CAB therapy, there were reductions in PRL, TSH, and FT4 levels as well as a decrease tumor size. This is the first reported case of a TSHoma responsive to CAB.
MON-234 síNdrome de InterrupcióN del Tallo Pituitario Que Se Presenta Como Amenorrea Primaria
Authors: Romero Fabiola , Arzamendia Sady Paola , Ferreira Dahiana , de Souza Claudia Neves , López Helen , Espinoza Grecia , Valinotti Elizabeth , Ayala Alejandro 
Published: May 2020

Abstract
Pituitary stalk interruption syndrome presenting as primary amenorrhea.
The pituitary stalk interruption syndrome is a rare congenital defect that is characterized by the absence or thinning of the pituitary stalk, an absent or ectopic posterior pituitary lobe, and hypoplasia or aplasia of the anterior lobe. The clinical presentation is variable.
It is usually diagnosed in the neonatal period due to the clinical manifestations that arise as a consequence of hormonal deficiencies. However some patients are diagnosed at a latter stage in life. Growth hormone deficiency it is observed in virtually all patients and can be associated to other anterior pituitary hormonal deficiencies. There is a high frequency of associated extra-pituitary malformations including those involving the central nervous system and the craniofacial structures.
We report the case of a 19-year-old woman with primary amenorrhea, lack of secondary sexual characteristics, short stature, low body mass index due to hypergonadotropic hypogonadism and growth hormone deficiency. The remaining pituitary hormones were normal. A pelvic ultrasound revealed orthotopic ovaries and uterus that were decreased in size. Her karyotype was normal. An MRI of the sellar region revealed an ectopic posterior pituitary lobe, and anterior pituitary hypoplasia with a thin pituitary stalk. The patient was treated with estradiol valerate and norgestrel as well as with growth hormone.
In conclusion, the pituitary stalk interruption syndrome is a rare form of congenital hypopituitarism that should be considered in cases of growth hormone deficiency and primary amenorrhea particularly in the presence of extra pituitary malformations involving the central nervous system.
OR28-06 Assessment of Long Term Quality of Life According to Treatment Options in Low Risk Papillary Thyroid Microcarcinoma Patients - Active Surveillance or Immediate Surgery, (A Follow up Interim Analysis of Maestro)
Authors: Shin Soo Myoung , Kong Sung Hye , Ryu Junsun , Kim Min Joo , Cho Sun Wook , Song Young Shin , Yi Ka Hee , Park Do Joon , Hwangbo Yul , Lee You Jin , Lee Kyu Eun , Kim Su-jin , Jeong Woo-Jin , Chung Eun-Jae , Hah Jeong Hun , Choi June Young , Ryu Chang Hwan , Jung Yuh-Seog , Lee Eun Kyung , Park Young Joo , Moon Jae Hoon 
Published: May 2020

Abstract
Background: The Multicenter Prospective Cohort Study of Active Surveillance on Papillary Thyroid Microcarcinoma (MAeSTro) started in June 2016. As a follow-up study of comparing the quality of life (QoL) of the active surveillance (AS) and immediate surgery (OP) groups at 8 months (9.3±4.8 and 7.1±4.2 months, respectively), here we aimed to compare the QoL between the AS and OP groups after 18 months (22.8±4.0 and 22.3±4.3 months, respectively) of follow-up.
Methods: QoL of 108 participants who chose AS, 101 who underwent OP, twelve who changed from AS to OP was evaluated using a thyroid-specific QoL questionnaire at diagnosis and during follow-up (median 23 months).
Results: The mean ages of the participants in the AS and OP groups were 47.7±11.0 and 45.1±10.0 years (p=0.075), respectively. At baseline, better physical (8.2±1.4 vs. 7.6±1.8, p=0.032), psychological (7.4±1.2 vs. 6.7±1.6, p=0.010), and total health (7.4±1.0 vs. 6.7±1.3, p=0.005) were observed in the AS group than in the OP group. After a mean follow up of 22.7±4.2 months, better physical (8.1±1.5 vs. 7.4±1.7, p=0.008), psychological (7.7±1.3 vs. 7.0±1.5, p=0.002), and total health (7.5±1.2 vs. 6.8±1.3, p=0.001) were observed in the AS group than in the OP group, whereas spiritual health was comparable between the two groups: compared with the AS group, the OP group experienced more alterations in appetite, sleep, menstrual cycle, voice, motor skill, weight, appearance, cold or heat tolerance, and body swelling. Furthermore, better QOL scores were observed in the AS group in self-concept, personal relationships, sexual life, work motivation, productivity and quality of work, feeling of isolation, driving, doing household chores, preparing meals and doing leisure activities after long term follow up.
Conclusion: Patients who underwent AS had better QOL even after long term follow up. Low risk papillary thyroid microcarcinomas do not influence survival, however surgery related deterioration of QOL lasted long and did not improve even in late post-operative stages when patients were fully recovered from surgery.
Keywords: Quality of life; papillary thyroid microcarcinoma; active surveillance; immediate surgery
SAT-LB52 A Case of Cushing’s Syndrome in a Patient With Addison’s Disease
Authors: Mungo Nicolas A 
Published: May 2020

Abstract
This is a case of a 56-year-old female with history of Addison’s disease, T1DM, hypothyroidism and Asthma. She was diagnosed with Addison’s disease after multiple hospital visits consistent with adrenal crises (Fatigue, weakness, salt craving, abdominal pain, hypotension, hyperpigmentation and hyponatremia). Multiple abdominal images including CT abdomen did not reveal any pathology. A random cortisol and ACTH were found to be abnormal at 0.5 and 3362, respectively. She was started on Hydrocortisone 10mg in the morning and 5mg in the evening. Her physical exam revealed diffuse hyperpigmentation including palmar creases and oral mucosa. There was no facial plethora or striae. She had no history of proximal myopathy, easy bruising, hypertension or osteoporosis. Upon follow up, exam revealed progressive worsening of her diffuse hyperpigmentation. A random cortisol and ACTH, without holding her Hydrocortisone, revealed 64.3 and &gt;2000, respectively. Concern arose for the possibility for a pituitary corticotrophin adenoma or ectopic secretion of ACTH driving her hyperpigmentation. MRI showed the pituitary gland had normal appearances. As there is a positive correlation between basal plasma ACTH values and the size of the pituitary adenoma in patients with Cushing’s disease the differential of a pituitary corticotrophin adenoma was originally thought to be dropped lower on the list of differentials. Further workup was pursued with an 8mg Dexamethasone suppression test to take advantage of the fact that ACTH secretion by the pituitary adenomas in Cushing’s disease are only relatively resistant to negative feedback regulation by glucocorticoids while most ectopic ACTH production from non-pituitary tumors are completely resistant to feedback inhibition. ACTH levels went from &gt;2000 down to 29 post 8mg Dexamethasone, which led to decreasing suspicion of an ectopic source of ACTH and further concern for a pituitary corticotrophin adenoma in addition to Addison’s disease. ACTH measurements are not widely used in documenting the adequacy of treatment of primary adrenocortical disease. The hormone is released in pulses, particularly in the early hours of the morning, and measurement of isolated samples is of limited value. Inappropriately normal to slightly elevated ACTH levels despite adequate glucocorticoid replacement in patient with adrenal insufficiency could be related to an altered pituitary sensitivity to cortisol suppression. Extremely elevated ACTH levels despite supra-physiological glucocorticoid replacement in a patient with Addison’s raises the question as to what is the source of ACTH?
SAT-LB77 Thyroid Radiofrequency Ablation: A New Office Based Procedure for Endocrinologists
Authors: Guttler Richard B 
Published: May 2020

Abstract
Recent FDA approval of thyroid RF has made it possible for endocrinologists in the USA to finally treat their own patients after obtaining training. I have 5 years experience working with these systems and have trained many endocrinologists in my practice. In 2019 I began a preliminary study of 12 patients with negative biopsies to see the feasibility of doing thyroid RF in my ultrasound room in my office without going to imaging centers or the hospital. The fee for office based RF is 3-6 times less expensive. RF system by RF Medical Korea was used in all cases. The results are promising. Skin and thyroid capsule local injection was all that was needed for pain control. Vital signs were monitored by my roving nurse. The maximal watts used was 20-40. There were no major complications and only one bruise in the neck area. No vocal symptoms. All 12 tolerated the procedure and after 30 minutes observation left with only a small band aid over the injection site. Two flew out of state that night.Conclusion: A preliminary assessment of in office thyroid RF without general of conscience sedation by trained endocrinologists suggests larger study with 80-100 cases is the next step.
MON-LB043 A Case of Renovascular Hypertension With Cortisol-Producing Adrenal Masses
Authors: Higashitani Takuya , Aono Daisuke , Kometani Mitsuhiro , Karashima Shigehiro , Demura Masashi , Yoneda Takashi , Takeda Yoshiyu 
Published: May 2020

Abstract
Renovascular hypertension (RVHT) is an important and potentially treatable form of resistant hypertension. Hypercortisolemia could also cause hypertension and diabetes mellitus. We experienced a case wherein adrenalectomy markedly improved blood pressure and plasma glucose levels in a patient with RVHT and subclinical Cushing’s syndrome. A 62-year-old Japanese man had been treated for hypertension and diabetes mellitus for 10 years. He was hospitalized because of disturbance in consciousness. His blood pressure (BP) was 236/118 mmHg; pulse rate, 132 beats/min; and plasma glucose level, 712 mg/dl. Abdominal computed tomography scanning revealed the presence of bilateral adrenal masses and left atrophic kidney. Abdominal magnetic resonance angiography demonstrated marked stenosis of the left main renal artery. The patient was subsequently diagnosed with atherosclerotic RVHT with left renal artery stenosis. Bilateral adrenal masses were immunohistologically identified as potential sites for cortisol overproduction. Therefore, laparoscopic left nephrectomy and adrenalectomy were simultaneously performed resulting in improved BP and glucose levels. Pathological studies revealed the presence of multiple cortisol-producing adrenal nodules and aldosterone-producing cell clusters in the adjacent left adrenal cortex. In the present case, activated renin-angiotensin-aldosterone system and cortisol overproduction resulted in severe hypertension, which was managed with simultaneous unilateral nephrectomy and adrenalectomy.
MON-LB112 Socioeconomic Status, Literacy, and Sex Differences in the Progression of Retinopathy in Patients With Type 2 Diabetes in Tokyo, Japan
Authors: Emoto Naoya , Soga Akimi , Fukuda Izumi , Tanimura-Inagaki Kyoko , Harada Taro , Sugihara Hitoshi , Goto Rei 
Published: May 2020

Abstract
Socioeconomic status has profound effects on glycemic control and diabetic complications in patients with type 2 diabetes. Sex differences are one of the most important factors in socioeconomic status and may vary among countries or areas. The study aim was to determine if sex differences are associated with glycemic control and diabetic complications in Tokyo, Japan, one of the most educated countries in the world. This study initially enrolled 3307 patients treated from 2017 to 2019 at the medical school hospital located in Tokyo. All enrolled patients were asked to complete behavioral and socioeconomic surveys. A total of 276 type 2 diabetic patients (175 males, age 64.1 ± 0.88 y, disease duration 15.2 ± 0.78 y, mean ± SE y; 101 females, age 64.0 ± 1.1 y, disease duration 15.6 ± 1.01 y) agreed to participate in the study. The survey questionnaire has been previously reported in detail (Patient Preference and Adherence, 10:2151-2162, 2016). The questionnaire attempted to determine estimations of risk preference regarding things like lottery gambling and accident insurance. After reviewing the patients’ answers, however, it became clear that some were illogical, which suggested that these patients did not understand the context of the questions, the hypothetical economical situations, or even the instructions, probably because of lower literacy skills. Thus, we labeled these patients as the unreasonable (UR) group (n = 81), and the patients who provided appropriate answers, even if extremely risk averse or risk loving, were labeled as the reasonable (R) group (n = 195). The prevalence of UR answers generally increased with age (&lt;50 y, 16%; 50-64 y, 10%; 65-74 y, 37%; ≥75 y, 64%) (p &lt; 0.0001). After age adjustment, there was a significant correlation between the UR answers and educational attainment in both sexes. The prevalence of UR answers was significantly higher in females (38.6%) than in males (24%) (p &lt; 0.01), which may be partly because the average number of educational years was lower for females than for males (males, 13.8 y; females, 12.9 y; p &lt; 0.05), but both averages are very high among all countries. The prevalence of retinopathy was significantly higher in the UR than the R group in males (p &lt; 0.05), but not in females. Job and economic status were not associated with prevalence of retinopathy. These results suggest that effects of literacy skills on progression of diabetic retinopathy may be sex dependent. Although the mechanism underlying this finding is unknown, sex may be an important biological factor beyond socioeconomic status in highly educated high-income countries.
SAT-LB304 A Rare Case of Atypical Teratoid/Rhabdoid Tumor of Sellar Region in an Adult
Authors: Madhavan Parvathy , Schwartz Paul , Kantorovich Vitaly 
Published: May 2020

Abstract
Introduction:
Atypical teratoid/rhabdoid tumor(ATRT) is an aggressive tumor of the central nervous system mostly seen in the pediatric age group. It is rare for these tumors to occur in adults, especially in the sellar region. Here we describe a case of ATRT of sellar region with rapidly progressive confusion and panhypopituitarism.
Case report: 58-year-old Caucasian woman presented with complaints of headache, generalized weakness, nausea and anorexia. Patient was also noted to be confused throughout her presentation and unable to provide detailed history. She also complained of blurring of vision and was noted to have visual field loss by her ophthalmologist. CT head without contrast revealed a sellar mass with suprasellar extension which is 2.5 x 2.4 x 3.5 cm with mass-effect on the base of anterior third ventricle. Blood work showed an A.M cortisol of 0.2 mcg/dL(6.2-19.4), prolactin of 62.6 ng/mL(4.8-22.3), TSH of 2 mIU/L(0.27-4.20) and free T4 of 0.36 ng/dL [0.80-1.90]. Cosyntropin stimulation test showed a 30-minute cortisol of 2.8 mcg/dL and a 60-minute cortisol of 4 mcg/dL. She was also noted to have hypernatremia with serum sodium upto 151 mmol/L(136-145). She was started on levothyroxine 88 mcg, DDAVP 0.1 mg PO twice daily and stress dose steroids which was weaned down to prednisone 7.5 mg daily eventually. MRI revealed 2.1 cm sellar mass with 3.1 cm suprasellar extension and rapidly progressive increase in size over several weeks period. Patient initially underwent left frontal midline craniotomy via transcallosal approach for resection of third ventricular and supra sellar tumor. For the residual tumor removal, she also underwent endoscopic resection of ATRT of brain via extended transnasal craniotomy with extensive nasoseptal flap repair of the skull base. Pathology revealed atypical teratoid/rhabdoid tumor of sellar region, WHO grade IV. The neoplastic cells characteristically show loss of INI-1 (SMARCB1), retained BRG1 (SMARCA4), focal weak positivity for EMA, Vimentin, Neurofilament (NF), CD56 and Cytokeratin AE1/AE3 is strongly positive. There is patchy non-specific immunoreactivity to PAX5 and PAX8. Hospital course was complicated by respiratory failure, NSTEMI, encephalopathy and patient is currently receiving supportive care for the same. Patient will be receiving radiotherapy and chemotherapy for further management of ATRT.
Conclusion:This case represents a rare occurrence of ATRT and suggests the need for suspicion in case of rapidly growing pituitary mass with progressive panhypopititarism.
SAT-LB44 WNT2B Regulates Adrenocortical Progenitor Cell Fate And Zona Glomerulosa Identity In Vivo
Authors: Little Donald W , Basham Kaitlin J , Hammer Gary D 
Published: May 2020

Abstract
Dysregulation of normal adrenal structure and function contributes to a spectrum of diseases from hypoplasia to cancer. Peripheral adrenocortical progenitor cells in the zona glomerulosa (zG) centripetally migrate and differentiate to replenish steroidogenic cells of the zG and the inner cortex over time (1). Both the fate of progenitor cells and aldosterone production by steroidogenic cells in the zG are regulated by Wnt signaling (2,3), but the cell-specific effects of individual Wnt ligands in the adrenal are not fully understood. To further characterize Wnt signaling components crucial for progenitor cell maintenance and zG identity, we analyzed mouse adrenals using single molecule in situ hybridization (smISH), which revealed the previously unknown expression of Wnt2b exclusively in the adrenal capsule. Wnt2b is co-expressed with the Wnt signaling potentiator Rspo3, the loss of which causes zG depletion and reduced adrenal size in mice (4). Therefore, we hypothesized that capsular WNT2B activates Wnt signaling in the underlying cortex to maintain the undifferentiated state of progenitor cells. To define the role of WNT2B in these processes, we generated Wnt2b conditional knockout (cKO) mice by crossing a capsule-specific Gli1-CreERT2 driver (5) and a floxed Wnt2b allele (6). We administered tamoxifen to 6-week-old male mice and assessed the effects of Wnt2b loss 4 weeks later. Gli1-CreERT2 activation significantly decreased Wnt2b expression (P&lt;0.001) and resulted in a lower adrenal to body weight ratio in Wnt2b cKOs compared to controls (P&lt;0.05). Adrenocortical proliferation (Ki67) was significantly decreased in Wnt2b cKO mice (P&lt;0.0001), suggesting that WNT2B may mediate progenitor cell self-renewal. To characterize the effect of WNT2B loss on Wnt signaling, we assessed activation of the primary Wnt effector β-catenin. High β-catenin activity in the zG observed in wild-type mice was disrupted in Wnt2b cKO mice, together with markedly reduced expression of adrenocortical Wnt target genes Axin2 and Wnt4. In addition, Wnt2b loss resulted in downregulation of steroidogenic genes Cyp11b2 (P=0.0139) and Hsd3b6 (P=0.0679). Together, these data suggest that capsule-derived WNT2B activates cortical Wnt signaling to maintain the identity of both undifferentiated progenitor cells and differentiated steroidogenic cells of the zG, which has important implications for adrenal homeostasis and disease, including both primary adrenal failure and neoplasia.
References: (1) King et al., PNAS, 2009 Dec 15;106(50):21185-90. (2) Berthon et al., Hum Mol Genet., 2010 Apr 15;19(8):1561-76. (3) Heikkila et al., Endocrinol., 2002 Nov;143(11):4358-65. (4) Vidal et al., Genes & Dev., 2016 Jun 15;30(12):1389-94. (5) Ahn & Joyner, Cell, 2004 Aug 20;118(4):505-16. (6) Tsukiyama & Yamaguchi, Neurosci Lett., 2012 Mar 14;512(1):48-52.