Cushing's Syndrome Due to a Functional Thymic Neuroendocrine Tumor in Multiple Endocrine Neoplasia Type 1 Syndrome.

Multiple endocrine neoplasia type 1 (MEN 1) syndrome is characterized by endocrinopathies and could be associated with thymic neuroendocrine tumors (NET). On rare occasions, they can be functional adrenocorticotropic hormone-secreting thymic carcinoid leading to Cushing's syndrome. In this report, we describe a case of adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome due to a thymic NET associated with MEN type 1 syndrome. We highlight its aggressive clinical course, the premise for a high index of suspicion for an ectopic ACTH secretion, and the need for early surgical resection combined with medical therapy and alternative treatments.

A Rare Case of Steroid-Resistant Neurosarcoidosis of the Cavernous Sinus With Optic Neuropathy: A Case Report.

Sarcoidosis is an inflammatory disease that presents with nervous system involvement in 5-10% of cases, commonly known as neurosarcoidosis.1 While there are no randomized controlled trials for the treatment of neurosarcoidosis, expert opinion supports initial treatment with corticosteroids and the use of steroid-sparing or anti-TNF agents in refractory or severe cases. We report a case of a 48-year-old African American male with a past medical history of biopsy-proven hepatic and renal sarcoidosis and progressive headache, dizziness, and blurry vision for 5 months, presenting with an acute exacerbation of right-sided vision loss over one day. MRI of the brain revealed a dural-based mass extending into the right cavernous sinus and compressing the right optic nerve. Given the pathological confirmation of systemic granulomatous disease consistent with sarcoidosis, clinical manifestations, bilateral hilar and mediastinal lymphadenopathy, MRI findings typical of central nervous system inflammation, and exclusion of other possible etiologies, the patient was diagnosed with probable neurosarcoidosis. Corticosteroids initially resolved his symptoms; however, he suffered an acute relapse. Combination therapy with corticosteroids plus mycophenolate mofetil (MMF) led to the eventual resolution of his symptoms. Only 8 cases of neurosarcoidosis involving the cavernous sinus have been reported. Our patient represents the only reported case of probable neurosarcoidosis of the cavernous sinus with optic neuropathy successfully treated with corticosteroid plus MMF combination therapy. We highlight the need to consider early, aggressive treatment in cases of neurosarcoidosis with optic neuropathy and to develop criteria to guide treatment strategy based on neurolocalization and the degree of neurological disability.

Double valve involvement: papillary fibroelastoma in a patient with severe mitral and aortic valve regurgitation.

Cardiac papillary fibroelastoma is a benign neoplasm that arises in the endocardium. It commonly presents as an incidental finding on transthoracic echocardiography or as emboli to the coronary, cerebral or pulmonary vasculature. Clinical manifestations described in the literature have generally been related to a sequelae of the associated embolic phenomenon of these lesions. Valve regurgitation is less common with papillary fibroelastoma and when found, it is not known to cause severe regurgitation requiring valve replacement. We report a case of papillary fibroelastoma in a patient with severe mitral and aortic valve regurgitation in association with mobile masses requiring double valve replacement. This patient managed initially as infective endocarditis with severe double valve regurgitation, was found to have valvular masses concernng for papillary fibroelastoma and subsequently confirmed on pathology.

A Tale of Three Rarities: Secondary Amyloid A (AA) Amyloidosis Caused by Recurrent Sialadenitis and Complicated by Pulmonary Hypertension and Adrenal Insufficiency.

A 48-year-old lady presented with a parotid mass found to be secondary to recurrent sialadenitis. She was also found to have microcytic anemia, renal dysfunction, an elevated gamma gap, and an isolated alkaline phosphatase elevation. Later, she developed altered mental status and shock, and was found to have adrenal insufficiency, pulmonary hypertension, and pulmonary nodules. A liver biopsy was consistent with amyloid deposition. The constellation of findings was consistent with systemic amyloid A (AA) amyloidosis secondary to recurrent sialadenitis with hepatic, renal, pulmonary, and adrenal involvement. The patient later passed away due to acute hypoxic respiratory failure. This case demonstrates rare sequelae of systemic AA amyloidosis of pulmonary hypertension and adrenal insufficiency.

A Rare Case of Hemophagocytic Lymphohistiocytosis in an Adult with Diffuse Large B-Cell Lymphoma.

A 71-year-old Indian female presented with a 3-month history of weight loss and fatigue. Further review confirmed a histological diagnosis of diffuse large B-cell lymphoma. Although bone marrow analysis did not reveal hemophagocytosis, she had some clinical and laboratory pointers to hemophagocytic lymphohistiocytosis (HLH). Her clinical state deteriorated rapidly with development of acute respiratory distress syndrome, diffuse alveolar hemorrhage, and subsequently death.

A Unique Case of Complete Atrioventricular Block Rapidly Progressing to Diastolic and Systolic Dysfunction in Cardiac Sarcoidosis.

Sarcoidosis is a multisystem granulomatous disease that most commonly affects the lungs but can affect other organs including the heart due to granuloma infiltration. Atrioventricular block is a common manifestation of cardiac sarcoidosis which can progress to sudden cardiac death. We report a case of cardiac sarcoidosis presenting as complete heart block, progressing to diastolic and systolic dysfunction without extracardiac manifestations early in the disease. This case stresses the importance of having a high index of suspicion for cardiac sarcoidosis in patients presenting with atrioventricular block of unknown etiology.

Association Between Cannabis Use and Complications Related to Crohn's Disease: A Retrospective Cohort Study.

BACKGROUND: Crohn's disease is an idiopathic inflammatory process that is occasionally associated with complications, which cause significant morbidity and mortality. The anti-inflammatory effect of cannabis in intestinal inflammation has been shown in several experimental models; it is unknown whether this correlates with fewer complications in Crohn's disease patients. AIMS: To compare the prevalence of Crohn's disease-related complications among cannabis users and non-users in patients admitted with a primary diagnosis of Crohn's disease or a primary diagnosis of Crohn's related complication and a secondary diagnosis of Crohn's disease between 2012 and 2014. METHODS: We used data from the Healthcare Cost and Utilization Project-National Inpatient Sample. Cannabis users (615) were compared directly after propensity score match to non-users, in aspects of various complications and clinical end-points. RESULTS: Among matched cohorts, Cannabis users were less likely to have the following: active fistulizing disease and intra-abdominal abscess (11.5% vs. 15.9%; aOR 0.68 [0.49 to 0.94], p = 0.025), blood product transfusion (5.0% vs. 8.0%; aOR 0.48 [0.30 to 0.79], p = 0.037), colectomy (3.7% vs. 7.5%; aOR 0.48 [0.29-0.80], p = 0.004), and parenteral nutrition requirement (3.4% vs. 6.7%, aOR 0.39 [0.23 to 0.68], p = 0.009). CONCLUSION: Cannabis use may mitigate several of the well-described complications of Crohn's disease among hospital inpatients. These effects could possibly be through the effect of cannabis in the endocannabinoid system.

Suppressed without a Cause: A Case of Idiopathic Immune Deficiency.

We report a case of a 45-year-old male who presented with a headache, fever, vomiting, somnolence, and difficulty walking for 10 days. His cerebrospinal fluid studies revealed cryptococcal meningitis. Chest and abdominal computed tomography (CT) scans showed splenomegaly along with mediastinal, retroperitoneal and inguinal lymphadenopathy. CD4 count turned out to be 208 µL-1. Human immunodeficiency virus (HIV) testing, serum protein electrophoresis, serum light chains and quantitative immunoglobulins were non-diagnostic and CD4 lymphopenia was attributed to acute infection. However, a persistent CD4 lymphopenia was seen in subsequent outpatient testing, which prompted a detailed workup for secondary causes of immunodeficiency. Repeated lymph node biopsies with analytic cytometric immunophenotypic analysis were normal, as was the bone marrow biopsy with detailed immunophenotypic and cytogenetic studies. The patient was hence being treated as a case of idiopathic CD4 lymphocytopenia.

Radial endobronchial ultrasound for the diagnosis of peripheral pulmonary lesions: A systematic review and meta-analysis.

Tissue diagnosis of peripheral pulmonary lesions (PPLs) can be challenging. In the past, flexible bronchoscopy was commonly performed for this purpose but its diagnostic yield is suboptimal. This has led to the development of new bronchoscopic modalities such as radial endobronchial ultrasound (R-EBUS), electromagnetic navigation bronchoscopy (ENB) and virtual bronchoscopy (VB). We performed this meta-analysis using data from previously published R-EBUS studies, to determine its diagnostic yield and other performance characteristics. Ovid MEDLINE and PubMed databases were searched for R-EBUS studies in September 2016. Diagnostic yield was calculated by dividing the number of successful diagnoses by the total number of lesions. Meta-analysis was performed using MedCalc (Version 16.8). Inverse variance weighting was used to aggregate diagnostic yield proportions across studies. Publication bias was assessed using funnel plot and Duval and Tweedie's test. 57 studies with a total of 7872 lesions were included in the meta-analysis. These were published between October 2002 and August 2016. Overall weighted diagnostic yield for R-EBUS was 70.6% (95% CI: 68-73.1%). The diagnostic yield was significantly higher for lesions >2 cm in size, malignant in nature and those associated with a bronchus sign on computerized tomography (CT) scan. Diagnostic yield was also higher when R-EBUS probe was within the lesion as opposed to being adjacent to it. Overall complication rate was 2.8%. This is the largest meta-analysis performed to date, assessing the performance of R-EBUS for diagnosing PPLs. R-EBUS has a high diagnostic yield (70.6%) with a very low complication rate.

Giant cell myocarditis: a life-threatening disorder heralded by orbital myositis.

A 40-year-old man with a history of orbital myositis (OM) presented to the emergency department with ventricular tachycardia requiring electrical cardioversion. Postcardioversion ECG showed right bundle branch block, while an echocardiogram revealed an ejection fraction of 20% and a dilated right ventricle. Cardiac MRI produced suboptimal images because the patient was having frequent arrhythmias. The rest of the work up, including coronary angiography, was unremarkable. Given the dilated right ventricle, we suspected arrhythmogenic right ventricular cardiomyopathy and discharged the patient with an implantable cardioverter-defibrillator. 1 week later, he was readmitted with cardiogenic shock; endomyocardial biopsy revealed giant cell myocarditis (GCM). To the best of our knowledge, this is the seventh case report of GCM described in a patient with OM. We recommend that clinicians maintain a high degree of suspicion for GCM in patients with OM presenting with cardiac problems.

Fulminant hepatic failure secondary to primary hepatic angiosarcoma.

Background. Hepatic angiosarcoma is a rare and aggressive tumor that often presents at an advanced stage with nonspecific symptoms. Objective. To report a case of primary hepatic angiosarcoma in an otherwise healthy man with normal liver function tests two months prior to presenting with a short period of jaundice that progressed to fulminant hepatic failure. Methods. Case report and review of literature. Conclusion. This case illustrates the rapidity of progression to death after the onset of symptoms in a patient with hepatic angiosarcoma. Research on early diagnostic strategies and newer therapies are needed to improve prognosis in this rare and poorly understood malignancy with limited treatment options.

Diagnostic accuracy retrospectively of electrocardiographic findings and cancer history for tamponade in patients determined to have pericardial effusion by transthoracic echocardiogram.

Unexpected pericardial effusions are often found by frontline providers who perform computed tomography. To study the hypothesis that electrocardiographic findings and whether cancer is known or suspected importantly change the likelihood of tamponade for such providers, all unique patients with moderate or large pericardial effusions determined by transthoracic echocardiography during a 6-year period were retrospectively identified. Electrocardiograms were evaluated by blinded investigators for electrical alternans (total and QRS), low voltage (limb leads only, precordial leads only, and both), and tachycardia (>100 QRS complexes/min). Medical records were reviewed to determine whether cancer was known or suspected and whether tamponade was diagnosed. Tamponade was present in 66 patients (27% of 241) with moderate or large pericardial effusions. No tachycardia lowered the odds of tamponade the most (likelihood ratio 0.4, 95% confidence interval 0.3 to 0.6) but by a degree less than any single diagnostic element increased it when present. The combined presence of all 3 electrocardiographic findings and cancer increased the odds of tamponade 63-fold (likelihood ratio 63, 95% confidence interval 33 to 150), whereas their combined absence decreased the odds only fivefold (likelihood ratio 0.2, 95% confidence interval 0.2 to 0.3). In conclusion, electrocardiography findings and cancer rule in tamponade better than they rule it out. Combining these diagnostic elements improves their discriminatory power but not sufficiently enough to rule out tamponade in patients with moderate or large pericardial effusions.