A Case of Primary Insular Ovarian Carcinoid Tumor with Hyperandrogenism and Carcinoid Heart Disease.

BACKGROUND Carcinoid heart disease typically occurs in the presence of metastatic carcinoid tumor deposits in the liver, as vasoactive substances access the systemic circulation through the hepatic vein. Primary ovarian carcinoid tumors are rare neuroendocrine tumors, and can be associated with carcinoid syndrome and carcinoid heart disease. CASE REPORT We describe the case of a 40-year-old woman who presented with secondary amenorrhea, acne, hirsutism, and diarrhea. She was found to have a heart murmur on exam in the absence of severe symptoms of heart failure. Her investigations demonstrated elevated urinary 5-hydroxyindoleacetic acid (5-HIAA), chromogranin A, and free testosterone. Abdominal computed tomography enterography showed a large and hypervascular pelvic mass. Octreotide scintigraphy confirmed the diagnosis of primary ovarian carcinoid tumor in the setting of an intensely octreotide-avid mass with no evidence of distant metastases. Transesophageal echocardiography showed severe tricuspid regurgitation with severe dilation of the right heart chambers. She underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. The pathology demonstrated a 14-cm carcinoid tumor of 'insular' type confined to the ovary, pT1apNX, grade 1, positive for chromogranin and synaptophysin (neuroendocrine markers) and positive mib-1 (Ki-67). Postoperatively, clinical and biochemical parameters improved significantly but her cardiac function regressed over time, resulting in a tricuspid valve replacement 6 years later. CONCLUSIONS Primary ovarian carcinoid tumors can result in carcinoid heart disease, even in the absence of liver metastases. Early diagnosis and treatment contribute to favorable outcomes.

Implementing a Diabetic Algorithm for Ophthalmology Surgery Patients: A Quality Improvement Initiative.

Introduction: The objective of this quality improvement, interventional study regarding patients with diabetes undergoing diabetic ophthalmology outpatient surgery aimed to develop, implement, and evaluate a new diabetic algorithm to improve safety, operating room efficiency, and decrease supply cost. Methods: A multidisciplinary study team was assembled, including ophthalmologists, endocrinologists, anesthesiologists, management, and nurses to review the current diabetic protocol. From August 2016 to July 2017, 13 patient safety concerns or incident reports were reviewed that identified two serious cases of hypoglycemia. Using the concerns data, frontline perspectives, and reviewing best practice guidelines, a new diabetic algorithm was developed and trialed for 24 months. The new algorithm limited the use of an existing preoperative insulin protocol and reduced the number of nurses required. The number of adverse events, nursing setup process steps, setup time, and preoperative insulin infusion protocols used were collected. An evaluation of the supply costs was performed. Results: After implementing the new diabetic algorithm, zero safety incidents were reported, and a 97.5% reduction in the use of preoperative insulin protocol resulted. Nursing staff perceived that the new diabetic algorithm was easier to configure, 23 minutes faster to set up, and required one nursing staff member. Supply cost was reduced by $30.63 (Canadian Dollars, CAD) per patient. Conclusion: Perioperative glucose irregularities may threaten patient safety and surgical outcomes. Healthcare professionals must improve patient safety, decrease healthcare expenditure, and prevent unnecessary delays. Multidisciplinary frontline staff experiential knowledge aided in the recognition of potential problems and comprehensive solutions to optimize patient care.

Evaluation of Thyroid Ultrasound Report Quality and Assessing Effect of Adherence to Risk Stratification Criteria on Referral for Thyroid Nodule Biopsy.

PURPOSE: This study aims to evaluate the quality of diagnostic thyroid ultrasound reports and determine the impact of consistent adherence to 2015 American Thyroid Association (ATA) and 2017 American College of Radiology (ACR) Thyroid Imaging Reporting and Data System (TI-RADS) on reducing unnecessary referrals for thyroid nodule biopsy. MATERIALS AND METHODS: Reports from 291 referrals for thyroid nodule biopsy were included for retrospective report evaluation (males: 42; mean age: 56) according to 2015 ATA and ACR TI-RADS lexicon. Cytology results were collected for each patient. Two radiologists blinded to cytology results independently, retrospectively reviewed imaging of the referrals, and rescored them according to 2015 ATA and 2017 ACR TI-RADS risk stratification systems. Statistical analysis was completed using chi-square analysis and calculation of κ statistic for interobserver variability. RESULTS: No report completely addressed all features associated with malignancy. Over half of the reports did not include descriptors on echogenicity, shape, margin, or echogenic foci. In all, 9.3% of biopsies showed malignant histology. Rescoring of referrals demonstrated decrease in biopsy referrals by 55% as per 2017 ACR TI-RADS and 14% as per 2015 ATA (P < .0001). There was no impact on detection of malignancy with adherence to ATA or ACR criteria and less interobserver variability with application of 2017 ACR TI-RADS compared to 2015 ATA. CONCLUSION: Thyroid ultrasound report quality was found variable with respect to nodule description. Reports recommended biopsy based on nodule size with no detailed description of other imaging features. Adherence to risk stratification system would have resulted in significant reduction in the number of unnecessary biopsy referrals.

Development of the Functional Assessment of Cancer Therapy-Immune Checkpoint Modulator (FACT-ICM): A toxicity subscale to measure quality of life in patients with cancer who are treated with ICMs.

BACKGROUND: Patients with cancer who are treated with immune checkpoint modulators (ICMs) have their health-related quality of life (HRQOL) measured using general patient-reported outcome (PRO) tools. To the authors' knowledge, no instrument has been developed to date specifically for patients treated with ICMs. The objective of the current study was to develop a toxicity subscale PRO instrument for patients treated with ICMs to assess HRQOL. METHODS: Input was collected from a systematic review as well as patients and physicians experienced with ICM treatment. Descriptive thematic analysis was used to evaluate the qualitative data obtained from patient focus groups and interviews, which informed an initial list of items that described ICM side effects and their impact on HRQOL. These inputs informed item generation and/or reduction to develop a toxicity subscale. RESULTS: Focus groups and individual interviews with 37 ICM-treated patients generated an initial list of 176 items. After a first round of item reduction that produced a shortened list of 76 items, 16 physicians who care for patients who are treated with ICMs were surveyed with a list of 49 patient-reported side effects and 11 physicians participated in follow-up interviews. A second round of item reduction was informed by the physician responses to produce a list of 25 items. CONCLUSIONS: To the authors' knowledge, this 25-item list is the first HRQOL-focused toxicity subscale for patients treated with ICMs and was developed in accordance with US Food and Drug Administration guidelines, which prioritize patient input in developing PRO tools. The subscale will be combined with the Functional Assessment of Cancer Therapy-General (FACT-G) to form the FACT-ICM. Prior to recommending the formal use of this PRO instrument, the authors will evaluate its validity and reliability in longitudinal studies involving substantially more patients.

Glucose control during labor and delivery.

Neonatal hypoglycemia is an important consequence for the infant of the mother with diabetes. We have reviewed 24 published papers of various protocols for control of glucose in pregnant diabetic women during labor and delivery including our own published work. A relationship of maternal glucose during labor and neonatal hypoglycemia was sought in 19 of these studies. A significant inverse relationship was found in 10 reports with 3 others showing a similar trend. In all but 1 of these 13 studies the participants had pregestational diabetes. Three of the 6 studies not reporting an inverse relationship involved women with GDM. From this review it appears that the maternal glucose should be maintained between 4.0 and 6.0-7.0 mmol/L during labor. Most women with gestational diabetes, especially if they require <1.0 units/kg/d of insulin, can simply be monitored without intravenous insulin. Our own results demonstrate that a target glucose of 4.0-6.0 mmol/L can be used safely and results in a low rate of neonatal hypoglycemia using an iterative glucose insulin infusion protocol for women with pregestational diabetes and when needed for women with gestational diabetes.

Metabolic glucose status and pituitary pathology portend therapeutic outcomes in acromegaly.

INTRODUCTION: Acromegaly is frequently associated with impaired glucose tolerance and/or diabetes. To evaluate the relationship between glucose metabolism and acromegaly disease, we evaluated 269 consecutive patients from two referral centres. METHODS: Clinical presentation, pituitary tumor size and invasiveness, and pituitary pathology were captured in a dedicated database. RESULTS: 131 women and 138 men with a mean age of 53.8 years were included. Of these, 201 (74.7%) presented with a macroadenoma and 18 (6.7%) with a microadenoma. Radiographic invasion was present in 91 cases (33.8%). Mean tumor diameter was 1.86 cm (0.2-4.6). Pituitary histopathologic findings revealed pure GH-producing somatotroph adenomas (SA) in 147 patients, prolactin-production by mixed lactotroph (LA) and SA or mammosomatotroph adenoma (MSA) in 46 [22.4%], acidophil stem cell adenoma in 6 [2.9%], and other diagnoses in 6 [2.9%]. Medical treatment included octreotide in 96 [36.9%] and in combination with pegvisomant or dopamine agonists in 63 [24.2%]. Nearly 80% of patients achieved IGF-1 normalization. Importantly, patients with pure somatotroph adenomas were significantly more likely to present with abnormal glucose metabolism [48.7%] than those with mixed adenomas [9.7%] [p<0.001] independent of GH/IGF-1 levels or tumor invasiveness. Abnormal glucose metabolism and pituitary pathology also remained linked following IGF-1 normalization. Moreover patients with pure SA and abnormal glucose metabolism were significantly (p<0.001) less likely to achieve disease remission despite the same therapeutic strategies. Conversely, patients with mixed adenomas were more likely (OR: 2.766 (95% CI: 1.490-5.136) to achieve disease remission. CONCLUSIONS: Patients with pure somatotroph adenomas are more likely than those with mixed adenomas to exhibit abnormal glucose metabolism.

Pregnancy in acromegaly: experience from two referral centers and systematic review of the literature.

BACKGROUND: Acromegaly results from increased growth hormone and its target insulin-like growth factor-1, most commonly due to a pituitary tumour. As it is frequently accompanied by infertility, little is known about the course of this disease in pregnancy. OBJECTIVE: We describe 13 new pregnancies in acromegalic women and compare their outcomes in a systematic review of the literature. METHODS: We collected clinical, biochemical, imaging, and outcomes data during and following pregnancy and performed a systematic review for a total of 47 pregnancies. An extended analysis of 106 pregnancies was also performed. RESULTS: In 13 newly described cases, pregnancy was un-complicated without need for additional surgical intervention. In these pregnancies, adjunctive medical therapy was required in three patients. This was in the form of somatostatin analogs (SA) (3/13) as well as pegvisomant in 1/13 to control symptomatic and biochemical progression. One 37-year-old female succeeded in having two separate pregnancies 2 years apart both without need for any form of medical therapy. Review of an additional 34 published reports allowed for an analysis of outcomes in 47 pregnancies. Adjunctive medical therapy during pregnancy was required in 15 of these cases where 12 received SA and an additional three received dopamine agonists. None of these patients developed endocrine or neurologic complications during pregnancy. In an extended analysis of 106 pregnancies, treatment during pregnancy appears to be associated with good disease control but increased risk of microsomic or macrosomic newborns depending on the medical agent used. CONCLUSIONS: In 13 newly described pregnancies along with systematic review of an additional 34 cases indicate that pregnancy in treated acromegalic women can proceed without significant complications or teratogenicity. Medical treatment during pregnancy with DA or SA appears to be associated with altered neonatal weight. Nevertheless, gestation may have a beneficial impact on acromegaly control both during and following pregnancy.

Caesarean section and macrosomia increase transient tachypnoea of the newborn in type 1 diabetes pregnancies.

We determined whether transient tachypnoea of the newborn (TTN) is more common in macrosomic versus normal weight infants and in those delivered by caesarean section versus vaginally, in a retrospective cohort analysis of 212 type 1 diabetes pregnancies. Caesarean section and macrosomia were both associated with higher TTN rates.