Background : Post bariatric hypoglycemia (PBH) is an increasingly recognized complication of bariatric surgery. Onset typically occurs more than 1-year post-op with episodes typically occurring postprandially. In contrast, insulinoma causes fasting and postprandial hypoglycemia and has been described after bariatric surgery, with 9 reported cases. Case : A 69-year-old woman presented to the emergency department after syncope in the setting of hypoglycemia to 39 mg/dL. Medical history was notable for Roux-en-Y gastric bypass 9 years ago. She had been experiencing fatigue in the 8 months prior with episodes of diaphoresis, hunger and mental fogginess that occurred randomly throughout the day and resolved with hard candy. An inpatient supervised fast resulted in Whipple triad with blood glucose 32 mg/dL and insulin 28 uU/mL (N 2.6-24.4) within 3 hrs of starting the test, thus making it difficult to exclude PBH. Therefore, a repeat fast was performed after minimal carbohydrate intake. This test yielded similar results with hypoglycemia occurring within 2.5 hrs. Based on this, medical treatment with octreotide and diazoxide was initiated. CT abdomen showed atrophic pancreas with no mass and an enhancing liver mass suggestive of a hemangioma. However, MRI abdomen revealed a 2.9 cm pancreatic tail mass and 3 hypervascular liver lesions concerning for metastasis. Subsequently, biochemical results from the supervised fast confirmed hyperinsulinemic hypoglycemia likely due to metastatic insulinoma based on imaging studies: C-peptide 4.67 ng/mL (N 0.8-3.85), proinsulin >800 pmol/L (N <=18.8), ß hydroxy butyrate 0.04 mmol/L (N <= 0.28). Pancreatic mass FNA via endoscopic ultrasound was suspicious for neuroendocrine tumor (NET). Liver mass biopsy showed metastatic NET. Hypoglycemia worsened despite continuous IV dextrose infusion and medical therapy necessitating distal pancreatectomy, splenectomy and partial hepatectomy for tumor debulking. Pathology revealed a 1.4 cm grade 2 well- differentiated NET in the pancreatic tail (mitotic rate 1 per 10 hpfs, Ki67 5%) and 2 foci of metastasis in the liver (8 and 0.9 cm). Unfortunately, the patient developed unexplained refractory shock with multiorgan failure post-operatively and passed away after 7 days. Conclusion :To our knowledge, this is the first case reported of malignant insulinoma after bariatric surgery. Malignant metastatic insulinoma is very rare. It causes severe hypoglycemia that can occur in the very early PP period similar to PBH, which could delay diagnosis. Inconclusive initial imaging should not preclude comprehensive workup in severe persistent hypoglycemia. Reference : Mulla, CM et al. Insulinoma After Bariatric Surgery: Diagnostic Dilemma and Therapeutic Approaches. Obes Surg . 2016. 26:874-881 Unless otherwise noted, all abstracts presented at ENDO are embargoed until the date and time of presentation. For oral presentations, the a...
123I/131I-mIBG scan is an established tool for the localization and treatment of neuroendocrine tumors such as paragangliomas (PGL). To minimize thyroid irradiation by the radioactive iodine in the mIBG preparation, blockade of thyroidal iodine uptake with high doses of stable iodine used to be given routinely as part of all mIBG protocols. As 123I is now more frequently utilized than 131I, concern about thyroid radiation has lessened and thyroid blockade is often considered unnecessary. However, in certain situations, the lack of thyroid blockade can significantly impact treatment decisions. This report describes two patients who had anterior mediastinal masses incidentally discovered on CT scans, and on further evaluation were found to have symptoms suggesting catecholamine excess with mildly elevated plasma normetanephrine levels. 123I-mIBG scans were performed without thyroid blockade, which demonstrated accumulation of tracer in the masses that were therefore deemed positive for PGL. Both patients underwent surgical resection of the masses with their surgical pathology revealing ectopic thyroid tissue (ETT). These cases illustrate that if appropriate thyroid blockade is not performed, ETT concentrating radioiodine from mIBG can lead to falsely positive mIBG scans and unnecessary surgical procedures. We conclude that in the setting of a mass suspicious for PGL in a location potentially representing ETT, such as the mediastinum, thyroid blockade should be employed for mIBG protocols to avoid false positive scans caused by ETT.
The contiguous gene deletion syndrome of congenital adrenal hyperplasia and Ehlers-Danlos syndrome, named CAH-X, is a rare entity that occurs because of a deletion of a chromosomal area containing 2 neighboring genes, TNXB and CYP21A. Here, we describe a patient from a consanguineous family in which coincidentally MEN-1 syndrome is associated with CAH-X, causing particular challenges explaining the phenotypic features of the patient. A 33-year-old man with salt-wasting congenital adrenal hyperplasia and classic-like Ehlers-Danlos syndrome presented with an adrenal crisis with a history of recurrent hypoglycemia, abdominal pain, and vomiting. He was found to have primary hyperparathyroidism, hyperprolactinemia, and pancreatic neuroendocrine tumors, as well as primary hypogonadism, large adrenal myelolipomas, and low bone mineral density. A bladder diverticulum was incidentally found. Genetic analysis revealed a heterozygous previously well-described MEN1 mutation (c.784-9G > A), a homozygous complete deletion of CYP21A2 (c.1-?_1488+? del), as well as a large deletion of the neighboring TNXB gene (c.11381-?_11524+?). The deletion includes the complete CYP21A2 gene and exons 35 through 44 of the TNXB gene. CGH array found 12% homozygosity over the whole genome. This rare case illustrates a complex clinical scenario with some initial diagnostic challenges.
Objective: To describe the course and anatomo-pathologic findings in a patient with a cortisol-producing adrenocortical carcinoma (ACC) who presented with disseminated invasive aspergillosis (DIA). We also review the clinical characteristics of ACC and the pathogenesis of DIA in the setting of hypercortisolemia. Methods: We describe the case of a 47-year-old man with a recent diagnosis of ACC associated with hypercortisolism, who presented with a large right basal ganglia hematoma and lesions on multiple organs thought to be metastatic. The patient deteriorated rapidly and unfortunately 6 days after presentation he died. An autopsy was performed which revealed DIA as the cause of death. Results: DIA in ACC was reported for the first time in 1981. Since then, no other case has been reported in the literature associating these 2 conditions. The lesions this patient presented on images were difficult to differentiate from metastatic lesions. The lack of inflammatory features due to hypercortisolemia accounted for this. Both ACC and DIA have very high mortality rates, even when DIA is treated appropriately. Conclusion: Opportunistic fungal infections are common in hypercortisolemia, however disseminated and invasive forms are rare. Awareness of uncommon presentations of hypercortisolemia is important for early treatment of those patients with a chance of survival. (AACE Clinical Case Rep. 2019;5:e233-237) Abbreviations: ACC = adrenocortical carcinoma; CT = computed tomography; DIA = disseminated invasive aspergillosis e234 Aspergillosis in ACC, AACE Clinical Case Rep. 2019;5(No. 4)
Myriad questions regarding perioperative management of patients on glucocorticoids (GCs) continue to be debated including which patients are at risk for adrenal insufficiency (AI), what is the correct dose and duration of supplemental GCs, or are they necessary for everyone? These questions remain partly unanswered due to the heterogeneity and low quality of data, studies with small sample sizes, and the limited number of randomized trials. To date, we know that although all routes of GC administration can result in HPA axis suppression, perioperative adrenal crisis is rare. Correlation between biochemical testing for AI and clinical events is lacking. Some of the current perioperative management recommendations based on daily GC dose and duration of therapy may be difficult to follow in clinical practice. The prospective and retrospective studies consistently report that continuing the daily dose of GCs perioperatively is not associated with a higher risk for adrenal crises in patients with GC-induced AI. Considering that oral GC intake may be unreliable in the early postoperative period, providing the daily GC plus a short course of IV Hydrocortisone 25-100 mg per day based on the degree of surgical stress seems reasonable. In patients who have stopped GC therapy before surgery, careful assessment of the HPA axis is necessary to avoid an adrenal crisis. In conclusion, our literature review indicates that lower doses and shorter duration of supplemental GCs perioperatively are sufficient to maintain homeostasis. We emphasize the need for well-designed randomized studies on this frequently encountered clinical scenario.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.