Publications by authors named "Silvia Vanni"

Objectives: Gastrointestinal stromal tumors, the most prevalent mesenchymal tumors (80 %) of the gastrointestinal tract, comprise less than 1 % of all gastrointestinal neoplasms and about 5 % of all sarcomas. Despite their rarity, Gastrointestinal stromal tumors present diverse clinical manifestations, anatomic locations, histological subtypes, and prognostic outcomes.

Methods: This scoping review comprehensively explores the epidemiology, clinical characteristics, diagnostic and prognostic modalities, as well as new therapeutic options for Gastrointestinal stromal tumors.

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  • Adipose tissue, or body fat, is more than just a fat store; it helps in cancer growth by providing energy and sending signals that promote tumor activity.
  • Cancer can make fat cells act differently, which creates a cycle that helps the tumor grow even more.
  • The review also talks about how being obese can increase cancer risks and how different diets can influence cancer's development and new treatments for it.
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  • Soft tissue sarcomas make up a rare group of tumors accounting for just 1% of solid cancers, with liposarcoma being a notable subtype.
  • The study focused on 21 patients with atypical lipomatous tumors/well differentiated liposarcoma (ALT/WDLPS) and dedifferentiated liposarcoma (DDLPS), investigating the role of biomarkers CDK4 and MDM2.
  • Results indicated that CDK4 was more highly expressed in DDLPS than in ALT/WDLPS, and the CDK4 inhibitor palbociclib showed promise in a sequential treatment approach when combined with lenvatinib, suggesting a new management strategy for DDLPS.
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Background: Neuroendocrine Carcinomas (NECs) prognosis is poor.No standard second-line therapy is currently recognized after failure of platinum-based first-line treatment. FOLFIRI and CAPTEM regimens have shown promising activity in preliminary studies.

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  • Immunotherapy is becoming an important treatment for sarcomas, which are tumors that can act very differently from each other.
  • Researchers found that understanding the different immune environments in these tumors can help improve treatment results.
  • The presence of specific immune cells called Tumor Infiltrating Lymphocytes (TIL) may help patients live longer and respond better to immunotherapy, along with certain genes related to the immune system.
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  • Myxofibrosarcoma is a rare and aggressive soft tissue cancer that often recurs and becomes more severe with each recurrence, posing challenges for patients and clinicians alike.
  • The study introduces a new cell line (MF-R 3) derived from a myxofibrosarcoma patient, which was thoroughly characterized using various biological tests to evaluate its tumor properties.
  • The MF-R 3 cell line exhibits similar characteristics to the original tumor and has shown promising sensitivity to anthracycline drugs, making it a valuable model for further research and drug testing.
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Myxofibrosarcoma (MFS) is a malignant soft tissue sarcoma (STS) that originates in the body's connective tissues. It is characterized by the presence of myxoid (gel-like) and fibrous components and typically affects patients after the fifth decade of life. Considering the ongoing trend of increasing lifespans across many nations, MFS is likely to become the most common musculoskeletal sarcoma in the future.

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Background: Polymorphous adenocarcinoma (PAC) represents the second most widespread neoplasm of the minor salivary glands. These tumors rarely develop a histological progression from low-grade to high-grade malignancy, named "high-grade transformation" (HGT). Only nine cases are described in literature.

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Myxofibrosarcoma (MFS) and undifferentiated pleomorphic sarcoma (UPS) can be considered as a spectrum of the same disease entity, representing one of the most common adult soft tissue sarcoma (STS) of the extremities. While MFS is rarely metastasizing, it shows an extremely high rate of multiple frequent local recurrences (50-60% of cases). On the other hand, UPS is an aggressive sarcoma prone to distant recurrence, which is correlated to a poor prognosis.

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A second-line standard of treatment has not yet been identified in patients with soft tissue sarcomas (STS), so identifying predictive markers could be a valuable tool. Recent studies have shown that the intratumoral and inflammatory systems significantly influence tumor aggressiveness. We aimed to investigate prognostic values of pre-therapy neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), lymphocyte-to-monocyte ratio (LMR), systemic inflammatory index (SII), progression-free survival (PFS), and overall survival (OS) of STS patients receiving second-line treatment.

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Introduction: Neuroendocrine neoplasms (NENs) are a rare group of tumors exceptionally heterogeneous, with clinical presentation ranging from well differentiated more indolent tumors to poorly differentiated very aggressive forms. Both are often diagnosed after the metastatic spread and require appropriate medical treatment. A high priority need in the management of this disease is the identification of effective therapeutic strategies for advanced and metastatic patients.

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Prion diseases are characterized by the self-assembly of pathogenic misfolded scrapie isoforms (PrP) of the cellular prion protein (PrP). In an effort to achieve a theranostic profile, symmetrical bifunctional carbazole derivatives were designed as fluorescent rigid analogues of GN8, a pharmacological chaperone that stabilizes the native PrP conformation and prevents its pathogenic conversion. A focused library was synthesized via a four-step route, and a representative member was confirmed to have native fluorescence, including a band in the near-infrared region.

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In the field of nanomedicine a multitude of nanovectors have been developed for cancer application. In this regard, a less exploited target is represented by connective tissue. Sarcoma lesions encompass a wide range of rare entities of mesenchymal origin affecting connective tissues.

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Myxofibrosarcoma (MFS) is a common entity of adult soft tissue sarcomas (STS) characterized by a predilection of the extremities and a high local recurrence rate. Originally classified as a myxoid variant of malignant fibrous histiocytoma, this musculoskeletal tumor has been recognized since 2002 as a distinct histotype showing a spectrum of malignant fibroblastic lesions with myxoid stroma, pleomorphism and curvilinear vessels. Currently, the molecular pathogenesis of MFS is still poorly understood and its genomic profile exhibits a complex karyotype with a number of aberrations including amplifications, deletions and loss of function.

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  • Prion diseases are brain disorders caused by misfolded proteins called prion proteins (PrP) that build up in the brain.
  • Scientists have been trying to stop this misfolding, but a new drug strategy is being tested that aims to remove prions without affecting the prion proteins directly.
  • A small molecule named compound 5 (ARN1468) has been found to help reduce prion levels in infected cells, showing promise for treating prion diseases even though it needs more testing to work with mice.
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Head and neck cancers (HNCs) represent the sixth most widespread malignancy worldwide. Surgery, radiotherapy, chemotherapeutic and immunotherapeutic drugs represent the main clinical approaches for HNC patients. Moreover, HNCs are characterised by an elevated mutational load; however, specific genetic mutations or biomarkers have not yet been found.

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Serpins represent the most broadly distributed superfamily of proteases inhibitors. They contribute to a variety of physiological functions and any alteration of the serpin-protease equilibrium can lead to severe consequences. SERPINA3 dysregulation has been associated with Alzheimer's disease (AD) and prion diseases.

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Introduction: Bone metastases (BM) are still the main cause of morbidity and mortality in cancer patients, not only because of their complications, defined as skeletal-related events (SREs), but also because of the negative impact bone pain has on quality of life (QoL) and survival, especially when opioid analgesics and locoregional treatments fail.

Materials And Methods: A single-center prospective study was carried out on 12 patients with symptomatic BM treated with MRI-guided focused ultrasound (MR-HIFU). The primary endpoint was the effectiveness of MR-HIFU in reducing current and breakthrough cancer pain (BTCP) scores.

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Giant cell tumor of bone (GCTB) and desmoplastic fibroma (DF) are bone sarcomas with intermediate malignant behavior and unpredictable prognosis. These locally aggressive neoplasms exhibit a predilection for the long bone or mandible of young adults, causing a severe bone resorption. In particular, the tumor stromal cells of these lesions are responsible for the recruiting of multinucleated giant cells which ultimately lead to bone disruption.

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Background: NTRK (neurotrophic tyrosine receptor kinase)-rearranged spindle cell neoplasms are a new group of tumors included in the new 5 edition of the World Health Organization (WHO) classification of soft Tissue and Bone Sarcomas. These tumors are characterized by NTRK gene fusions and show a wide spectrum of histologies and clinical behavior. Several targeted therapies have recently been approved for tumors harboring NTRK fusions, including STS.

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  • - Adult rhabdomyosarcoma (RMS) is a rare type of soft tissue sarcoma, making up less than 3% of cases, which leads to limited understanding of its clinical management and natural history.
  • - Researchers studied the molecular biology of adult RMS, focusing on gene expression related to cell transition and drug resistance, utilizing patient-derived cultures and 3D models to assess drug response.
  • - Key findings include the identification of potential biomarkers for RMS through RT-PCR, the highest drug sensitivity in anthracycline-based treatments, and significant genetic mutations linked to poor prognosis, contributing to a better understanding of this rare cancer.
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Aims: Perilipins are conserved proteins that decorate intracellular lipid droplets and are essential for lipid metabolism. To date, there is limited knowledge on their expression in human brain or their involvement in brain aging and neurodegeneration. The aim of this study was to characterise the expression levels of perilipins (Plin1-Plin5) in different cerebral areas from subjects of different age, with or without signs of neurodegeneration.

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Rationale: Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor that is prevalent among children and adolescents. Surgery is the most important therapeutic approach for IMT and complete resection is recommended. Although 50% of IMTs show anaplastic lymphoma kinase (ALK) rearrangements, crizotinib has proven an effective therapeutic approach.

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  • Soft tissue sarcomas (STS) are rare types of tumors, with treatment starting with drugs called anthracyclines and possibly a drug named trabectedin next.
  • Researchers studied how trabectedin works using special 3D cell cultures from patients and found it worked better in these 3D setups than in flat ones.
  • The results suggest that trabectedin is effective against certain sarcomas and may work better because it interacts with the cells' surrounding environment.
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