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Background Neuroendocrine tumors (NETs) are rare, can be found in any part of the body including the gastrointestinal tract, lung, and pancreas and have potential for metastasis. Clinical Case A 39-year-old male with stage IV high grade neuroendocrine tumor of the pancreas with metastasis to the liver and lymph nodes whose disease progressed despite treatment with systemic therapy who presented with severe hypoglycemia. Diagnosis and management of his hypoglycemia was difficult. For the diagnosis, patient's serum blood glucose did not always correlate with his capillary blood glucose via finger sticks, which lead to a delay in his diagnosis. Also, his diagnosis was not straight forward as he did not respond adequately to the glucagon following a serum glucose of less than 55 mg/dL (normal 70-110 mg/dL) during a diagnostic fast. Once critical labs were obtained, it took days for the results. In the interim, his hypoglycemia was managed with dextrose infusions at 10%, 20% and as high as 50%, frequent meals, and high dose steroids, yet he still had daily symptomatic hypoglycemia. A diagnosis of insulinoma was made with the following critical lab results: insulin 70.4 uIU/mL (2.6-24.9), C-peptide 12.6 ng/mL (1.1-4.4), proinsulin 407.6 pmol/L (0. 0-10. 0), beta-hydroxybutyrate <0. 050 mmol/L (0. 02-0.27), insulin antibodies <5. 0 uU/mL, negative sulfonylurea screen, insulin-growth factor-1 (IGF-1) 77 ng/mL (90-278), and insulin-growth factor binding protein three (IGF-BP3) 2133 ug/L (2571-5982). Patient was treated with both diazoxide and hepatic artery embolization to reduce tumor burden. His dose of diazoxide was titrated to maintain normoglycemia. He had mild tumor lysis syndrome that did not require dialysis. His hypoglycemia resolved within a few days of treatment and was discharged from the hospital with a continuous glucose monitor (CGM), diazoxide and hydrocortisone. It is our belief that transformation of the liver lesions to insulinoma was the cause of his hypoglycemia. Conclusion We recommend that patients with metastases from their neuroendocrine tumor who develop hypoglycemia be evaluated for insulinoma and if found should be treated with diazoxide and if not surgical candidates to consider hepatic artery embolization. Presentation: No date and time listed

Background Despite improvement in many metabolic outcomes, bone loss remains a concern with weight loss (WL). In this prospective study, we compared the effects of WL from lifestyle intervention (LI) and bariatric surgery (BS) on WL-associated bone loss. We hypothesize that the higher WL from bariatric surgery will result in a greater decline in bone mineral density (BMD) and bone quality. Methods Twenty-nine obese subjects (BMI > 30 kg/m2; weight: 107±19.3 kg) were included in the study; 13 participated in a LI study of WL (supervised diet+exercise) and 16 underwent BS. Assessments were done at 10% WL and at 6 months. Areal BMD and body composition were evaluated by DXA; bone micro-architecture and bone strength by microfinite element analysis-derived parameters as failure load (f.load) and stiffness at the distal radius and distal tibia were evaluated by high resolution peripheral quantitative computed tomography. Serum bone turnover markers, adipokines and cytokines were measured by Elisa. Results Participants in the LI arm were significantly older (71.3±4 vs 48.2±10 y.o., p < 0.001) and lighter (93.4 ± 8 vs. 118.5±18 kg, P=0.001) compared to the BS arm. Analysis adjusted for baseline age and weight showed no significant differences in areal BMD at all sites, volumetric BMD and bone microarchitectural features of the radius and tibia, except for higher cortical porosity (Ct.Po) at the tibia in the LI arm compared to the BS arm (3.0±0.3 vs 1.7±0.3%, p=0.04, respectively). The average WL at 6 months were -11.87±4.7 vs.-15.96±5.1%, p=0.07, for LI and BS, respectively. At 10%WL, the LI arm had a reduction in trabecular volumetric BMD (tb.vBMD) at the radius (-2.2±1.2 vs. 3.1±4.8% P=0.05) and tibia (-2.2±1.4 vs. 2.2±3.9 % P=0.02, respectively) compared to BS arm which had increases in this parameter. There was also a trend for reduced radius trabecular number and thickness in the LI arm at 10% WL. Meanwhile, there was a trend for reduction in total hip BMD, f.load and stiffness at the radius in the BS arm only. At 6 months, tb.vBMD at the radius was reduced in LI (-2.7±0.9%) relative to the increase in BS group (5.7±2.0%), p=0.008. There was a reduction in F.load (-5.3±2.4 vs 0.6±1.2%, p=0.09) and stiffness (-6.1±2.7 vs. 0.8±1.4%, p=0.08) of borderline significance at the tibia in the BS compared to no change in LI arm. BS arm showed a greater increase in serum C- telopeptide (28.1±48 vs. 81.3±30%, P=<0.05), an index of bone resorption, and in adiponectin (-0.7±8 vs.36.2±22.1%, P=0.01) compared to LI at 6 months. There were no significant differences in changes in lean and fat mass at 6 months in both arms. Conclusion: Although WL from LI resulted in reduced radial tb.vBMD, BS was associated with a greater increase in bone resorption and a trend for reduction in bone strength at the weight-bearing tibia at 6 months compared to LI. Results from this pilot project need confirmation in a larger study with longer duration of follow-up.

Introduction Patients with untreated congenital adrenal hyperplasia (CAH) can present early with severe symptoms of salt wasting, adrenal insufficiency and hyperandrogenism. Late consequences as a result of long term untreated CAH are rarely seen nowadays. We present a patient who presented with a massive uterine leiomyoma and bilateral adrenal myelolipomas due to longstanding treatment noncompliance. Clinical Case A female was born with ambiguous genitalia and diagnosed with CAH at birth. She was raised as a female and received steroids until age 29 when she stopped taking steroids on her own with the intention of identifying as a male. At age 37, he presented with abdominal distension, vomiting, and hypotension. Physical exam was notable for hypotension, significantly distended abdomen, hirsutism, gynecomastia and clitoromegaly. Labs revealed sodium 126 meq/L (136–145) cortisol 78.5 ug/dL (3.7–19.4), ACTH 166 pg/mL (6–50), 17-hydroxyprogesterone 4356 ng/dL (≤285), androstenedione 7188 ng/dL (35–250), total testosterone 737 ng/dL (2–45), estradiol 142 pg/mL (48–440), aldosterone <1 ng/dL (3–16), renin 0.45 ng/mL/hr (0.25–5.82), metanephrines 56 pg/mL (≤205), normetanephrines 56 pg/mL (≤148). CT abdomen and pelvis revealed a large 31 x 35 x 31 cm pelvic mass, a 5.9 x 2.4 cm right adrenal mass and an 11.8 x 8.8 cm left adrenal mass. The patient underwent total abdominal hysterectomy and bilateral adrenalectomy. Pathology of the pelvic mass was consistent with uterine leiomyoma (gross tumor was 12.4 kg) and pathology of the bilateral adrenal masses were consistent with bilateral adrenal myelolipomas. Discussion Glucocorticoids and mineralocorticoids are the mainstays of treatment in CAH, with the goal of providing adequate replacement while reducing levels of ACTH and adrenal androgens. Persistently elevated levels of ACTH and androgens can lead to many serious sequela, even outside of adrenal insufficiency and virilization. Due to the conversion of androgens to estrogens, untreated females with CAH have significantly elevated levels of both hormones. These high levels of androgens and estrogen can then stimulate growth of estrogen-dependent organs as exemplified by our patient. Chronic ACTH stimulation can cause adrenal hyperplasia, but has also been associated with the development of other adrenal masses including adrenal myelolipomas. Adrenal myelolipomas can become hormonally functional or cause mass effect, hemorrhage, necrosis when reaching a large enough size. Conclusion This case demonstrates the importance of CAH treatment compliance as there are many serious sequela outside of the expected adrenal insufficiency and virilization. Even when the desired effect is virilization with physical male features, other means of hormonal therapy should be considered as there remains the risks of abnormal growth of certain organs sensitive to the excessive hormones.