Sporadic, Nontrauma-Related, Desmoid Tumor of the Pancreas: A Rare Disease—Case Report and Literature Review

Desmoid tumors (DTs) are neoplasms of fibroblastic origin characterized by lack of a capsule. They are nonmetastatic and locally aggressive. Intraabdominal DTs are often observed in familial adenomatous polyposis and Gardner syndrome or subsequent to localized traumatic injury. Sporadic forms are defined as nontrauma- or nongenetic-related DTs. Isolated, sporadic pancreatic DTs have been considered anecdotal, with only 9 cases described in the literature. We report the case of a 68-year-old man with a case of sporadic cystic DT localized to the pancreatic tail. The tumor was discovered incidentally during computerized tomography performed for an unrelated condition. The patient was asymptomatic; however, biopsy was performed on the clinical suspicion of cystic cancer of the pancreas. Pathology analysis showed fibroblastic proliferation, and the diagnosis of DT was confirmed by immunohistochemical staining for beta-catenin. The patient underwent resection with no further treatment and remain disease-free 60 months after surgery

Circulating dendritic cells are severely decreased in idiopathic pulmonary fibrosis with a potential value for prognosis prediction

Dendritic cells (DCs) accumulate in the lung of patients affected by idiopathic pulmonary fibrosis (IPF). We measured the frequencies of circulating conventional CD1c + and CD141+ cells (namely, cDC2 and cDC1) and of plasmacytoid CD303+ DCs in a cohort of 60 therapy naive IPF patients by flow cytometry. Peripheral levels of reactive oxygen species (ROS) and of pro-inflammatory and Th1/Th2 polarizing cytokines were also analyzed. All blood DC subtypes were significantly reduced in IPF patients in comparison to age- and sex-matched controls, while ROS and interleukin (IL-6) levels were augmented. IL-6 expression increased along with disease severity, according to the gender-age-physiology index, and correlated with the frequency of cDC2. IL-6 and cDC2 were not influenced by anti-fibrotic therapies but were associated with a reduced survival, the latter being an independent predictive biomarker of worse prognosis. Deciphering the role of DCs in IPF might provide information on disease pathogenesis and clinical behavior

Severe depletion of peripheral blood dendritic cell subsets in obstructive sleep apnea patients: A new link with cancer?

Recent evidence suggests that alterations of the immune responses are associated with the inflammatory nature of obstructive sleep apnea (OSA) and of its related co-morbidities. In this scenario, we asked whether circulating dendritic cell (DC) subsets may be possible players as their role has not yet been detailed. The frequency distribution of peripheral blood myeloid (mDC1 and mDC2) and plasmacytoid (p) DCs was investigated by mean of multi-parametric flow cytometry in 45 OSA patients (mean age: 53 yrs; M = 29) at the time of the first diagnosis and compared to 30 age- and sex-matched healthy controls. Oxidative burst (OB) and serum levels of tumor necrosis factor (TNF)-α, (interleukin) (IL)-6, interferon (INF)-γ, IL-2, IL-4, IL-10 and vascular endothelial growth factor (VEGF) were also analyzed. All subsets of circulating DCs were significantly depleted in OSA patients as compared to healthy subjects (p 30) and were inversely correlated with both mDC2 (r = −0.802, p < 0.007) and pDC (r = −0.317, p = 0.04) subsets. We first provide evidence for a constitutive reduction of all circulating DC subsets in OSA patients. Perturbation of DCs coexists with an inflammatory milieu and is negatively correlated with the expression of IL-6, which is actually recognized as a pivotal inhibitor of DC maturation. Future studies exploring the contribution of DCs in the pathogenesis of OSA and of its complications should be encouraged

First description of the hyperpnea–hypopnea periodic breathing in patients with interstitial lung disease-obstructive sleep apnea: Treatment implications in a real-life setting

There is evidence that hypopneas are more common than apneas in obstructive sleep apnea (OSA) related to idiopathic pulmonary fibrosis (IPF). We investigated the frequency distribution of hypopneas in 100 patients with interstitial lung diseases (ILDs) (mean age 69 yrs ± 7.8; 70% males), including 54 IPF cases, screened for OSA by home sleep testing. Fifty age-and sex-matched pure OSA patients were included as controls. In ILD-OSA patients the sleep breathing pattern was characterized by a high prevalence of hypopneas that were preceded by hyperpnea events configuring a sort of periodic pattern. This finding, we arbitrarily defined hyperpnea–hypopnea periodic breathing (HHPB), was likely reflecting a central event and was completely absent in control OSA. Also, the HHPB was highly responsive to oxygen but not to the continuous positive pressure support. Thirty-three ILD-OSA patients (42%) with a HHPB associated with a hypopnea/apnea ratio ≥3 had the best response to oxygen with a median residual AHI of 2.6 (1.8–5.6) vs. 28.3 (20.7–37.8) at baseline (p &lt; 0.0001). ILD-OSA patients with these characteristics were similarly distributed in IPF (54.5%) and no-IPF cases (45.5%), the most of them being affected by moderate–severe OSA (p = 0.027). Future studies addressing the pathogenesis and therapy management of the HHPB should be encouraged in ILD-OSA patients

Subclinical impairment of dynamic left ventricular systolic and diastolic function in patients with obstructive sleep apnea and preserved left ventricular ejection fraction

Background: Hypoxia affects myocardial oxygen supply resulting in subclinical cardiac dysfunction in obstructive sleep apnea (OSA) patients, with cardiovascular complications being associated with increased oxidative burst (OB). The aims of our study were to assess left ventricular (LV) dynamic myocardial deformation and diastolic reserve at rest and upon exercise, along with OB determination in this patients subset. Methods: Conventional echocardiography, Doppler myocardial imaging and LV 2D speckle tracking echocardiography were performed in 55 OSA patients with preserved LV ejection fraction (EF) and 35 age and sex-comparable healthy controls. Peripheral OB levels were evaluated by flow cytometry. Results: Despite comparable LVEF, LV global longitudinal strain (GLS) was significantly reduced in OSA at rest (-13.4 ± 3.8 vs-18.4 ± 3.3 in controls, P < 0.001) and at peak exercise (-15.8 ± 2.6 vs-23.4 ± 4.3, P < 0.001). Systolic pulmonary artery pressure (sPAP) and E/E′ ratios increase during effort were higher in OSA than in controls (ΔsPAP 44.3% ± 6.4 vs 32.3% ± 5.5, P < 0.0001, and ΔE/E' 87.5% ± 3.5 vs 25.4% ± 3.3, P < 0.0001, respectively). The best correlate of E/E′ at peak stress was peak exertion capacity (r =-0.50, P < 0.001). OB was also increased in OSA patients (P = 0.001) but, unlike OSA severity, was not associated with LV diastolic dysfunction. Conclusions: Evaluation of diastolic function and myocardial deformation during exercise is feasible through stress echocardiography. OSA patients with preserved LVEF show subclinical LV systolic dysfunction, impaired LV systolic and diastolic reserve, reduced exercise tolerance, and increased peripheral levels of OB. Therapy aimed at increasing LV diastolic function reserve might improve the quality of life and exercise tolerability in OSA patients