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Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome is believed to result from an autosomal dominant mutation in the fumarate hydratase (FH) gene on chromosome 1. It is characterized by leiomyomas, mainly uterine or cutaneous, and renal cell carcinoma (RCC). The most common type of RCC associated with HLRCC is type II papillary RCC although other types are seen. Of note, chromophobe RCC has not been described in previously documented cases of HLRCC. HLRCC is typically associated with germline mutations with occasional somatic mutations reported, however, to the best of our knowledge, none have yielded the full phenotype until now. Herein, we report a case of a 45-year-old woman who underwent a hysterectomy following a year of heavy vaginal bleeding, yielding a diagnosis of uterine leiomyomas. Eight months later, the patient presented with hematuria and was subsequently found to have a left renal mass. Following a left radical nephrectomy, histologic exam revealed a chromophobe RCC with FH deficiency.

Effusive-constrictive pericarditis (ECP) is a rare clinical entity resulting from accumulating pericardial fluid within a stiff, non-compliant pericardium. There are a number of etiologies for ECP, which include malignancy, radiation, post-surgical causes, infectious, and collagen disorders. Clinically, ECP often presents as right-sided heart failure, or in advanced cases, cardiac tamponade. Symptoms may persist despite treatment with pericardiocentesis, and may warrant consideration for pericardiectomy for more definitive management. Invasive hemodynamic evaluation with cardiac catheterization remains the gold standard for diagnosis of ECP; however, echocardiography can provide a definitive diagnosis with high sensitivity and specificity. Echocardiographic features suggestive of ECP include ventricular septal motion abnormalities, such as interdependence, accentuated longitudinal motion of the heart, and altered respirophasic ventricular filling. While these features have been well established and can lead to the diagnosis of ECP, they are rarely observed in clinical practice. We present a case of ECP in a 25-year-old active duty male with a history of chest wall myoepithelial carcinoma who clearly demonstrated such echocardiographic findings of ECP.Effusive-constrictive pericarditis (ECP) is a rare clinical entity resulting from accumulating pericardial fluid within a stiff, non-compliant pericardium. There are a number of etiologies for ECP, which include malignancy, radiation, post-surgical causes, infectious, and collagen disorders. Clinically, ECP often presents as right-sided heart failure, or in advanced cases, cardiac tamponade. Symptoms may persist despite treatment with pericardiocentesis, and may warrant consideration for pericardiectomy for more definitive management. Invasive hemodynamic evaluation with cardiac catheterization remains the gold standard for diagnosis of ECP; however, echocardiography can provide a definitive diagnosis with high sensitivity and specificity. Echocardiographic features suggestive of ECP include ventricular septal motion abnormalities, such as interdependence, accentuated longitudinal motion of the heart, and altered respirophasic ventricular filling. While these features have been well established and can lead to the diagnosis of ECP, they are rarely observed in clinical practice. We present a case of ECP in a 25-year-old active duty male with a history of chest wall myoepithelial carcinoma who clearly demonstrated such echocardiographic findings of ECP.

In the appropriate clinical context, ST-segment elevation on electrocardiogram (ECG) necessitates prompt evaluation for coronary artery occlusion requiring reperfusion with percutaneous coronary intervention. Conversely, the etiology of ST-segment elevation may be representative of an alternative diagnosis other than myocardial infarction. We report the case of a patient with a history of primary bone sarcoma who presented for further evaluation of a large pericardial effusion identified on an outpatient echocardiogram and was found to have ST-segment elevation on ECG in the absence of any cardiopulmonary symptoms. The ECG abnormalities were attributed to a likely persistent current of injury resulting from a mass in the interventricular septum, likely representative of a metastatic lesion of his known malignancy. This case highlights the importance of maintaining a broad differential for ST-segment elevation, particularly in patients without symptoms of angina and those with a history of aggressive or relapsing cancer to minimize the morbidity and mortality of invasive procedures.

Objectives Performance status (PS) scales such as the Eastern Cooperative Oncology Group (ECOG) PS and the Karnofsky Performance Index have limited utility in selecting therapies and predicting related adverse events in older patients with cancer. In July 2016, medical oncologists at our institution adopted the Cancer and Aging Research Group toxicity prediction score (CARG), a toxicity prediction tool, to identify patients who are \"fit\" for chemotherapy versus those who are \"frail\" and may experience severe complications. Methods Our retrospective review included referrals of beneficiaries 75 years of age and older who received standard systemic therapy and patients of the same age whose treatment was modified due to CARG. We compared the score's utilization six months before and after its incorporation and then assessed how its application impacted admissions, emergency department (ED) visits, and medical management. Results Thirty-eight patients with a mean age of 81 years met the inclusion criteria. Their diagnoses included gastrointestinal (37%), lung (21%), hematologic (18%), breast (10.5%), genitourinary (3%), and other (10.5%) malignancies. CARG was documented for 12.5% of systemic therapy recipients before its adoption and 41% of recipients after adoption. Its use was limited by the reliance on physicians to perform scoring during time-constrained patient encounters. Patients had fewer mean inpatient admissions (0.7 versus 2.3), admission days (4.3 versus 8), and ED visits (1.1 versus 2.5) when management was modified based on the score. Conclusion CARG assessment may facilitate a safer and more tailored approach to cancer care in older patients than conventional PS scales alone. Its integration into patient screening would increase its application and better define its potential predictive capacity to decrease risks for hospitalization.

Introduction Longitudinal data on management and progression of type 2 diabetes mellitus (T2DM) in India are scarce. LANDMARC (CTRI/2017/05/008452), first‐of‐its‐kind, pan‐India, prospective, observational study aimed to evaluate real‐world patterns and management of T2DM over 3 years. Methods Adults (≥25 to ≤60 years old at T2DM diagnosis; diabetes duration ≥2 years at enrolment; controlled/uncontrolled on ≥2 anti‐diabetic agents) were enrolled. The first‐year trends for glycaemic control, therapy and diabetic complications, including those from metropolitan and non‐metropolitan cities are reported here. Results Of 6236 enrolled participants, 5654 completed 1 year in the study. Although the overall mean glycated haemoglobin (HbA1c) improved by 0.5% (baseline: 8.1%) at 1 year, only 20% of the participants achieved HbA1c <7%. Participants from metropolitan and non‐ metropolitan cities showed similar decrease in glycaemic levels (mean change in HbA1c: −0.5% vs. −0.5%; p = .8613). Among diabetic complications, neuropathy was the predominant complication (815/6236, 13.1% participants). Microvascular complications (neuropathy, nephropathy and retinopathy) were significantly (p < .0001) higher in non‐metropolitan than metropolitan cities. Hypertension (2623/6236, 78.2%) and dyslipidaemia (1696/6236, 50.6%) continued to be the most commonly reported cardiovascular risks at 1 year. After 1 year, majority of the participants were taking only oral anti‐diabetic drugs (OADs) (baseline: 4642/6236 [74.4%]; 1 year: 4045/6013 [67.3%]), while the proportion of those taking insulin along with OADs increased (baseline: 1498/6236 [24.0%] vs. 1 year: 1844/6013 [30.7%]). Biguanides and sulfonylureas were the most used OADs. The highest increase in use was seen for dipeptidyl peptidase‐IV inhibitors (baseline: 3047/6236 [48.9%]; 1 year: 3529/6013 [58.7%]). Improvement in all glycaemic parameters was significantly (p < .0001) higher in the insulin vs. the insulin‐naïve subgroups; in the insulin‐naïve subgroup, no statistical difference was noted in those who received >3 vs. ≤3 OADs. Conclusions First‐year trends of the LANDMARC study offer insights into real‐world disease progression, suggesting the need for controlling risk factors and timely treatment intensification in people with T2DM. The literature describing the burden of T2DM in India are sparse. The present study reports 1‐year trend from LANDMARC study, a nationwide, prospective, longitudinal study planned for 3 years. This study highlights some important observations in terms of diabetes complications, treatment patterns and glycaemic control in T2DM patients from India and also emphasizes the disease burden in metropolitan and non‐metropolitan cities. This study revealed that the burden of uncontrolled T2DM is high in India with only 20% of participants achieving glycaemic control in 1 year (HbA1c <7%; 53  mmol/mol).

Introduction Longitudinal data on progression, complications, and management of type 2 diabetes mellitus (T2DM) across India are scarce. LANDMARC (CTRI/2017/05/008452), the first pan‐India, longitudinal, prospective, observational study, aims to understand the management and real‐world outcomes of T2DM over 3 years. Methods Adults (≥25 to ≤60 years old at T2DM diagnosis; diabetes duration ≥2 years at enrollment; controlled/uncontrolled on ≥2 anti‐diabetic agents) were enrolled. Baseline characteristics were analyzed using descriptive statistics. Results Of the 6279 recruited participants, 6236 were eligible for baseline assessment (56.6% [n/N = 3528/6236] men; mean ± SD age: 52.1 ± 9.2 years, diabetes duration: 8.6 ± 5.6 years). mean ± SD HbA1c, fasting plasma glucose, and postprandial glucose values were 64 ± 17 mmol/mol (8.1 ± 1.6%), 142.8 ± 50.4 mg/dl, and 205.7 ± 72.3 mg/dl, respectively. Only 25.1% (n/N = 1122/6236) participants had controlled glycemia (HbA1c < 53 mmol/mol, <7%). Macrovascular and microvascular complications were prevalent in 2.3% (n/N = 145/6236) and 14.5% (n/N = 902/6236) participants, respectively. Among those with complications, non‐fatal myocardial infarction (n/N = 74/145, 51.0%) and neuropathy (n/N = 737/902, 81.7%) were the most reported macrovascular and microvascular complication, respectively. Hypertension (n/N = 2566/3281, 78.2%) and dyslipidemia (n/N = 1635/3281, 49.8%) were the most reported cardiovascular risks. Majority (74.5%; n/N = 4643/6236) were taking oral anti‐diabetic drugs (OADs) only, while 24.4% (n/N = 1522/6236) participants were taking OADs+insulin. Biguanides (n/N = 5796/6236, 92.9%) and sulfonylureas (n/N = 4757/6236, 76.3%) were the most reported OADs. Basal (n/N = 837/6236, 13.4%) and premix (n/N = 684/6236, 11.0%) insulins were the most reported insulins. Conclusions Baseline data from LANDMARC help understand the clinical/medical profile of study participants and underscore the extent of suboptimal glycemic control and prevalence of associated complications in a vast majority of Indians with T2DM. People with long‐standing diabetes are at an increased risk of diabetes complications and cardiovascular events. In India, progression of diabetes and its complications over a long time has not been studied extensively. The LongitudinAl Nationwide stuDy on Management And Real‐world outComes of diabetes (LANDMARC) study has enrolled 6279 people with type 2 diabetes across India and will prospectively gather data on disease control, treatment, concomitant complications, and risks in these participants over 3years. LANDMARC baseline data represent the real‐world snapshot of a type 2 diabetic profile and underscore poor glycemic control and a considerable burden of complications prevalent in India.