Ewing’s Sarcoma of the Breast in a Young Woman: A Case Report and Review of the Literature

Ewing’s Sarcoma Family Tumors (ESFT) include classic Ewing’s sarcoma of bone, extra-skeletal Ewing’s sarcoma (EES), malignant small cell tumor of the chest wall (Askin tumor), and soft tissue-based Peripheral Primitive Neuroectodermal tumors (pPNET). The t(11;22)(q24;q12) translocation is associated with 85% of tumors and leads to EWS-FLI-1 (Ewing’s Sarcoma–Friend Leukemia Integration-1) formation. This is a potent transforming gene that encodes a chimeric protein that plays a role in the genesis of Ewing’s Sarcoma and Primitive Neuroectodermal Tumors. The breast location of ESFT remains exceptional. The prognosis is among the poorest of all subtypes of breast cancer and even poorer than other extraosseous Ewing’s sarcomas. We describe the case report of a 23-year-old patient with a growing breast lump, who required an accurate and challenging diagnostic estimation and who ultimately resulted in a peripheral primary neuroectodermal tumor (pPNET). Through this case description and a brief narrative review of the literature, we aim to highlight the rarity of ESFT located in the breast. Histopathological confirmation is mandatory for all growing masses of the breast to reach a conclusive diagnosis and plan the correct treatment. Patients with rare diagnoses should always be centralized in breast units, conducting multidisciplinary meetings and, when necessary, the diagnosis should be shared through wider national or international registries

Carcinoid Crisis: A Misunderstood and Unrecognized Oncological Emergency

Carcinoid Crisis represents a rare and extremely dangerous manifestation that can occur in patients with Neuroendocrine Tumors (NETs). It is characterized by a sudden onset of hemodynamic instability, sometimes associated with the classical symptoms of carcinoid syndrome, such as bronchospasm and flushing. Carcinoid Crisis seems to be caused by a massive release of vasoactive substances, typically produced by neuroendocrine cells, and can emerge after abdominal procedures, but also spontaneously in rare instances. To date, there are no empirically derived guidelines for the management of this cancer-related medical emergency, and the available evidence essentially comes from single-case reports or dated small retrospective series. A transfer to the Intensive Care Unit may be necessary during the acute setting, when the severe hypotension becomes unresponsive to standard practices, such as volemic filling and the infusion of vasopressor therapy. The only effective strategy is represented by prevention. The administration of octreotide, anxiolytic and antihistaminic agents represents the current treatment approach to avoid hormone release and prevent major complications. However, no standard protocols are available, resulting in great variability in terms of schedules, doses, ways of administration and timing of prophylactic treatments

Poly(Vinylalcohol-Co-Vinyloleate) for the Preparation of Micelles Enhancing Retinyl Palmitate Transcutaneous Permeation

The amphiphilic properties of poly(vinylalcohol) substituted with oleic acid was evaluated to assess the possibility to prepare polymeric micelles in an aqueous phase containing a hydrophobic core able to host lipophilic drugs such as retinyl palmitate and thereby enhance its transcutaneous absorption in the stratum corneum. The effect of the increased drug absorption suggests the possibility of interaction between the substituted polymer and the components present in the intercorneocyte spaces. Correlations between the drug concentration in the preparative mixture, micelle size, and drug permeation were evaluated to establish the best functional properties of the micellar systems enhancing retinyl palmitate absorption. Transcutaneous absorption increased with decreasing micelle size, and micelle size decreased on decreasing the drug concentration in the preparative mixture

Role of Notch2 pathway in mature B cell malignancies

In recent decades, the Notch pathway has been characterized as a key regulatory signaling of cell-fate decisions evolutionarily conserved in many organisms and different tissues during lifespan. At the same time, many studies suggest a link between alterations of this signaling and tumor genesis or progression. In lymphopoiesis, the Notch pathway plays a fundamental role in the correct differentiation of T and B cells, but its deregulated activity leads to leukemic onset and evolution. Notch and its ligands Delta/Jagged exhibit a pivotal role in the crosstalk between leukemic cells and their environment. This review is focused in particular on Notch2 receptor activity. Members of Notch2 pathway have been reported to be mutated in Chronic Lymphocytic Leukemia (CLL), Splenic Marginal Zone Lymphoma (SMZL) and Nodal Marginal Zone Lymphoma (NMZL). CLL is a B cell malignancy in which leukemic clones establish supportive crosstalk with non-malignant cells of the tumor microenvironment to grow, survive, and resist even the new generation of drugs. SMZL and NMZL are indolent B cell neoplasms distinguished by a distinct pattern of dissemination. In SMZL leukemic cells affect mainly the spleen, bone marrow, and peripheral blood, while NMZL has a leading nodal distribution. Since Notch2 is involved in the commitment of leukemic cells to the marginal zone as a major regulator of B cell physiological differentiation, it is predominantly affected by the molecular lesions found in both SMZL and NMZL. In light of these findings, a better understanding of the Notch receptor family pathogenic role, in particular Notch2, is desirable because it is still incomplete, not only in the physiological development of B lymphocytes but also in leukemia progression and resistance. Several therapeutic strategies capable of interfering with Notch signaling, such as monoclonal antibodies, enzyme or complex inhibitors, are being analyzed. To avoid the unwanted multiple “on target” toxicity encountered during the systemic inhibition of Notch signaling, the study of an appropriate pharmaceutical formulation is a pressing need. This is why, to date, there are still no Notch-targeted therapies approved. An accurate analysis of the Notch pathway could be useful to drive the discovery of new therapeutic targets and the development of more effective therapies

Evaluation of carcass quality, body and pulmonary lesions detected at the abattoir in heavy pigs subjected or not to tail docking

BackgroundNowadays, body and tail lesions and respiratory disease are some of the greatest problems affecting the health and welfare of pigs. The aim of the study was to measure the prevalence of pleurisy, bronchopneumonia (enzootic pneumonia like lesions) and lesions on tail and body of heavy pigs subjected or not to tail docking through the inspection in Italian abattoirs. Additionally, the effect of tail docking and season was investigated on carcass quality (weight, % of lean meat, and Protected Designation of Origin (PDO) classification). For this purpose, a total 17.256 carcasses belonging to 171 batches from 103 farms were inspected in an Italian abattoir between 2019 and 2022. Enzootic pneumonia (EP) like lesions were scored according to the Madec and Derrien method, while pleurisy was scored using the Italian Slaughterhouse pleuritic evaluation system (SPES). For the tail and body, the lesions were scored according to Welfare Quality. The lesion score index (LSI) was calculated for each area. Data were analysed using a general linear model (GLM) including tail caudectomy, season and distance of the farm from the abattoir.ResultsThe warm season increased the percentage of lesions in carcasses in all parts of the body observed (P < 0.0001). The presence of undocked tail increased the LSI of the tail (P < 0.0001). The percentage of limbs lesions with score 2 and limbs LSI increase with increasing duration of transport (coef. = 0.003, P < 0.001; coef. = 0.008, P < 0.001; respectively). The hot carcass weight and the percentage of carcasses included in the PDO were higher in batches with docked tails (P = 0.027; P < 0.001, respectively), while the percentage of lean meat was higher in batches with undocked tails (P < 0.001). There was a negative correlation between the percentage of carcasses included in PDO and the LSI of tail (r = - 0.422; P < 0.001).ConclusionsIn conclusion, the presence of the undocked tail and the warm season can be considered risk factors for the prevalence of tail lesions, while long transport can increase limb lesions. Furthermore, the carcass weight and meat quality were negatively influenced by tail lesions

Best supportive care for idiopathic pulmonary fibrosis: Current gaps and future directions

Best supportive care (BSC) is generally defined as all the interventions and the multiprofessional approach aimed to improve and optimise quality of life (QoL) in patients affected by progressive diseases. In this sense, it excludes and might be complementary to other interventions directly targeting the disease. BSC improves survival in patients with different types of cancer. Patients with idiopathic pulmonary fibrosis (IPF) experience a vast range of symptoms during the natural history of the disease and might have a beneficial effect of BSC interventions. This review highlights the current evidence on interventions targeting QoL and gaps for the clinical assessment of BSC in the treatment of IPF patients. Very few interventions to improve QoL or improve symptom control are currently supported by well-designed studies. Sound methodology is paramount in evaluating BSC in IPF, as well as the use of validated tools to measure QoL and symptom control in this specific group of patients