Comprehensive evaluation of adrenal cortical cancer: Single-center 22-year experience

Background: Adrenocortical carcinoma (ACC) is a rare malignancy with a reported incidence of 0.5–2 cases per million population per year. Literature is scarce on this malignancy with poor prognosis and limited treatment options. The objective of this report was to report a single-center experience of this rare malignancy. Methods: A retrospective chart review (1997–2019) was performed on patients diagnosed with ACC via surgical pathology. Data were subsequently split into two time periods defined by the date of initial presentation: 1996–2007 and 2008–2019. Results: 52 patients were identified, 55.8 % were female, and the mean age at the time of surgery was 53 years. Most common presentations (25 % each) were: incidentalomas, abdominal pain, or hormonal hypersecretion. Laboratory evidence of hormonal hypersecretion was identified in 40.4 %. Among those patients, elevated 24-hour urinary free cortisol was the most common finding in both time periods.The overall 5-year survival rate was 53.1 % and showed significant improvement in recent years when compared between the two time periods, 85.7 % vs 40.0 %. P = 0.004. Conclusion: Overall, the most common presentation of ACC was found to be equally distributed among incidentaloma, hormonal hypersecretion, and abdominal pain. Hypercortisolism, a recognized poor prognosticator, was the most frequent form of hormonal hypersecretion. The 5-year survival rate for ACC was noted to be poor in general; however, it was observed to be substantially higher in more recent years. More research is necessary to further understand this malignancy in order to optimize management and improve outcomes. Clinical relevance: This report provides a valuable contribution and expands the knowledge base for this important yet rare malignancy. The objective of this study was to report a single-center experience of ACC by characterizing patients diagnosed with this rare disease and managed at our institution and assessing their outcomes

Surgical Management of Giant Prolactinomas: A Descriptive Study

Introduction. Giant prolactinoma (GP) is a rare pituitary lactotropic cell tumor larger than 4 cm in its widest dimension, and is less likely than a smaller prolactinoma to achieve prolactin normalization on dopamine agonist (DA) monotherapy. There is a paucity of data on the circumstances and outcomes of second-line management of GP with surgery. Herein, our institution’s experience with the surgical management of GPs is described. Methods. A single-center retrospective analysis was conducted of patients who underwent surgery for giant prolactinoma from 2003 to 2018. A chart review was conducted for demographic data, clinical features, laboratory and radiographic findings, operative and pathology reports, perioperative management, and clinical outcomes in follow-up. Descriptive statistics were used. Results. Of 79 prolactinoma cases, 8 patients had GP with a median age of 38 years (range 20–53), 75% (6/8) were male, with a median largest tumor dimension of 6 cm (range 4.6–7.7), and a median prolactin level of 2,500 μg/L (range 100–>13,000). Six patients had transsphenoidal surgery for dopamine agonist (DA) resistance or intolerance. Two patients had a craniotomy for a missed diagnosis; one was due to the hook effect. No tumor resections were complete by either surgical approach; all had persistent hyperprolactinemia requiring postoperative DA therapy, and two patients had an additional craniotomy procedure for further tumor debulking. There was no recovery of pituitary axes and postoperative deficits were common. Remission as defined by prolactin normalization occurred in 63% (5/8) at a median time of 36 months (range 14–63 months) on DA therapy after surgery with a follow-up of 3–13 years. Conclusions. GPs infrequently require surgical resection, which is generally incomplete and requires adjuvant therapy. Given the rarity of surgery for GPs, multi-institutional or registry studies would yield clearer guidance on optimal management

Duration of Type 2 Diabetes and Very Low Density Lipoprotein Levels Are Associated with Cognitive Dysfunction in Metabolic Syndrome

Type 2 diabetes (T2D) is now recognized as an independent risk factor for accelerated cognitive decline and neurological conditions like Alzheimer’s disease. Less is known about the neurocognitive function of T2D patients with comorbid metabolic syndrome, despite their elevated risk for impairment. Computerized testing in 47 adults with T2D that met criteria for NCEP metabolic syndrome revealed that cognitive impairment was prevalent, including 13% in tests of memory, 50% in attention, and 35% in executive function. Partial correlations showed that longer duration of diabetes was associated with poorer performance on tests of basic attention (r=-0.43), working memory (r=0.43), and executive function (r=0.42). Strong associations between very low density lipoprotein and poor cognitive function also emerged, including tests of set shifting (r=0.47) and cognitive inhibition (r=-0.51). Findings suggest that patients with T2D that meet criteria for metabolic syndrome are at high risk for cognitive impairment. Prospective studies should look to replicate these findings and examine the possible neuroprotective effects of lipid-lowering medication in this population