Introduction Pheochromocytomas (PCCs) are rare neuroendocrine tumors that secrete catecholamines. They may be associated with familial syndromes such as Multiple Endocrine Neoplasia type 2 (MEN 2), von Hippel-Lindau disease (VHL), and Neurofibromatosis type 1 (NF 1). PCCs are a common cause of malignant hypertension in pregnancy and can lead to adverse maternal and fetal outcomes if left undiagnosed or untreated. Case Description A 39-year-old Latin American primigravid woman at 8 weeks 4 days gestation was referred to the endocrinology clinic for labile blood pressure (160/90 mmHg at recent ER visit) and a lab finding of elevated catecholamines (plasma norepinephrine 4720 pg/ml; normal 0-874 pg/ml). She reported chronic headaches and 2-lb unintentional weight loss during pregnancy. Past medical history was significant for right adrenal PCC diagnosed at age 13 (currently status post adrenalectomy), and a cerebrovascular accident at age 29. Family history was relevant for PCC in the patient's sister and mother. The patient's home medications were nifedipine 30 mg daily and prenatal vitamins. On physical exam, her blood pressure was 126/76 mm Hg, heart rate was 67/min and a scar of previous right adrenalectomy was noted. Doxazosin 1 mg daily was added to her regimen and nifedipine was discontinued. The patient was initially planned for an elective cesarean section at term. However, at 10 weeks 5 days of gestation, she had a missed abortion. Further genetic testing of syndromic PCC revealed a heterozygous mutation in the VHL gene. Abdominal MRI showed a left adrenal mass (3.6×2.6×3.6 cm3) and retroperitoneal lymphadenopathy. The patient underwent left adrenalectomy and is currently hemodynamically stable on maintenance hydrocortisone and fludrocortisone. Discussion We report a case of PCC complicating pregnancy in a 39-year-old primigravida who had a family history of PCC. In females of reproductive age with a known PCC diagnosis, we suggest early genetic testing as it can further advise family planning, antepartum monitoring, and management. Medically, these patients can be managed with α-adrenergic blockers and calcium-channel blockers, followed by β-blockers. This can improve maternal and fetal outcomes primarily by minimizing paroxysmal elevations in catecholamines. Definitive treatment of PCC is surgical excision, usually performed during the second trimester or postpartum. Management of patients with VHL requires lifelong clinical, laboratory, and imaging surveillance for other manifestations of the disease. References Bancos, Irina et al. Maternal and fetal outcomes in phaeochromocytoma and pregnancy: a multicentre retrospective cohort study and systematic review of literature. Lancet Diabetes Endocrinol 2021; 9: 13–21 Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

Introduction Insular thyroid cancer (ITC) is a rare form of thyroid cancer derived from follicular cells. ITC requires a histopathologic diagnosis and has an intermediate morphology between well differentiated thyroid cancers (follicular and papillary) and undifferentiated thyroid cancer (anaplastic). The rate of metastasis and mortality are higher compared with well-differentiated thyroid carcinomas. Case presentation A 74-year-old woman presented for a 1 year history of rapidly progressive neck mass associated with neck pain, limited neck motion, loss of appetite, weight loss, and dysphagia. Past medical history included hypertension, type 2 diabetes, dyslipidemia, and vitamin D deficiency. There was no history of neck radiation or family history of cancer. Physical examination revealed a 10×7 cm neck mass with cervical adenopathy. Vital signs were unremarkable. Laboratory studies showed TSH of 0.71 uIU/mL (0.270-4.200), free T4 of 0.57 ng/dL (0.93-1.70) and thyroglobulins of 213.616 ng/dL (1.6-59.9). Neck CT demonstrated a multilobulated mass in the left thyroid lobe measuring 12×5.2×5.6 cm with tracheal deviation, multiple nodules surrounding the carotids and bone metastasis. Fine needle aspiration showed a mixed pattern of thyroid follicles. An excisional biopsy showed nests and sheets of small uniform cells with scattered abortive follicles and mitosis consistent with ITC. There was no necrosis. The Ki67 index was 10-20%. On immunohistochemical analysis TTF1 and thyroglobulin were positive and P63, calcitonin, and PTH were negative. Tracheostomy and PEG tube placement were required due to mass obstructive effect. Radiation and steroids were initiated before starting lenvatinib. The patient was not a surgical candidate due to tumor extension to the carotids. She ultimately succumbed to the disease after a 45-day hospital course complicated by pneumonia. Discussion ITC is a rare type of thyroid tumor with a mortality rate of 12-75%. The diagnosis is based on histopathology, demonstrating solid nests (insulae) of small, uniform carcinoma cells, small follicles containing thyroglobulin, frequent necrotic foci and variable mitotic activity. The tumor cells in ITC are derived from poorly differentiated follicular cells expressing thyroglobulin. The immunostaining of thyroglobulin differentiates ITC from other poorly differentiated thyroid tumors. In this case the immunohistochemical analysis was positive for thyroglobulin and negative for calcitonin, which confirmed that the tumor cells were follicular in origin 1 . ITC behaves differently compared with other thyroid carcinomas, with more frequent metastasis and lower average 20-year post-diagnosis survival. Our patient had other characteristics that conferred a poor prognosis, including advanced age, high rate of mitosis, and multiple metastases. Although surgery and radioactive iodine are first line, this patient's tumor extension required alternative therapies. Radiotherapy and lenvatinib, a tyrosine kinase receptor inhibitor, were employed unsuccessfully. 1. Soza, J., et al. Insular Thyroid Cancer. Cancer, 3260-3267. Presentation: No date and time listed

Introduction Hyperosmolar crisis, which includes diabetic ketoacidosis (DKA) and hyperosmolar hyperglycemic state (HHS), is a severe and acute complication of diabetes. The estimated mortality rates of DKA and HHS are 6.8% and 10%, respectively (Benoit, Zhang, Geiss, Gregg, & Albright, 2018) (Pasquel & Umpierrez, 2014). There is limited evidence regarding the clinical characteristics, hospital complications, and mortality rate of patients with combined DKA and HHS. Therefore, we aim to compare hospital outcomes between patients with DKA, HHS, and combined DKA and HHS in a community hospital in Brooklyn, NY. Methodology We performed a retrospective analysis from the patients admitted for DKA and/or HHS at Health+Hospitals / Woodhull from 2019 to 2021. Descriptive statistics were used to determine the frequency of patients in each hyperglycemic group and their clinical characteristics. Using one-way ANOVA and Chi-square test, we compared the demographics and clinical outcomes between subgroups. Multivariate regression analysis and log-rank test were used to assess the influence of multiple variables on hospital mortality and the difference in survival between groups, respectively. Results We studied 263 patients admitted for a hyperglycemic crisis, DKA (58%), HHS (14%), and DKA+HHS (28%). Baseline characteristics of the population include male (60%), African American (42%), Hispanic (32%), and mean BMI of 28±8 kg/m2. No difference was found in mean admission HbA1c (DKA 12±2.6%, HHS 12.7±3%, DKA+HHS 12.7±2.5%, p: 0.08). Patients with HHS were older (mean age 56±15) compared with DKA (47±17) and DKA+HHS (49±18). Patients with DKA+HHS had higher mean admission blood sugar (893±330 mg/dL), compared with DKA (497±206 mg/dL) and HHS (808±346 mg/dL) (p <0.001). There was no difference in the frequency of hypokalemia, K+ <3.5 mEq/L (DKA 26.9%, HHS 6.3%, DKA+HHS 12.6%, p: 0.8); hypoglycemia (DKA 12%, HHS 4.6%, DKA+HHS 7%, p: 0.2); or cerebral edema (DKA 0.8%, HHS 0.4%, DKA+HHS 1.5%, p: 0.1) between the groups. However, the frequency of acute kidney injury (AKI) was higher in DKA (24.7%) than in HHS (7%) or DKA+HHS (16.7%) (p: 0.034). There was no difference in the mean days of hospital stay (DKA 8.5±28, HHS 7±8, DKA+HHS 8±10, p: 0.9) and in-hospital mortality (DKA 3.4%, HHS 3.5%, DKA+HHS 4%, p: 0.069) among the groups. In all subtypes of hyperglycemic crisis, the mortality rate was significantly higher with older age, presence of AKI, cerebral edema, and vasopressor requirement. Conclusions There was no significant difference in mortality, length of admission, prevalence of hypoglycemia, hypokalemia, and cerebral edema between the three groups. The prevalence of AKI was significantly higher in DKA compared with HHS and DKA+HHS. Older age, presence of AKI, cerebral edema, and vasopressor requirement were mortality predictors among hyperglycemic crisis. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.