257 results match your criteria: "Osteoncology & Rare Tumors Center[Affiliation]"

Background: More accurate predictive biomarkers in patients with gastroenteropancreatic neuroendocrine tumours (GEP-NETs) are needed. This study aims to investigate radiomics-based tumour phenotypes as a surrogate biomarker of the tumour vasculature and response prediction to antiangiogenic targeted agents in patients with GEP-NETs.

Methods: In this retrospective study, a radiomics signature was developed in patients with GEP-NETs and liver metastases receiving lenvatinib.

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Background: Rare primary malignant bone sarcomas (RPMBS) account for 5%-10% of primary high-grade bone tumors and represent a major treatment challenge. The outcome of patients with RPMBS enrolled in the EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.

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Purpose: This study set out to evaluate the psychosocial effects of coronavirus disease 2019 (COVID-19) among adolescents with cancer, and whether these effects are significantly different among adolescents who were undergoing therapy or had completed it.

Materials And Methods: The AIEOP Adolescents Working Group and the AIEOP Psychosocial Working Group adapted a questionnaire, which was completed by 214 adolescent cancer patients ( Mage =16.3 y, range: 15 to 19 y old) treated at 16 AIEOP centers in the North (38%), South (31%), and Center (31%) of Italy.

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Aberrant splicing events are associated with colorectal cancer (CRC) and provide new opportunities for tumor diagnosis and treatment. The expression of the splice variants of NF-YA, the DNA binding subunit of the transcription factor NF-Y, is deregulated in multiple cancer types compared to healthy tissues. NF-YAs and NF-YAl isoforms differ in the transactivation domain, which may result in distinct transcriptional programs.

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The Potential Role of Adjuvant Chemoradiotherapy in Patients with Microscopically Positive (R1) Surgical Margins after Resection of Cholangiocarcinoma.

Curr Oncol

May 2023

Osteoncology, Soft Tissue and Bone Sarcomas, Innovative Therapy Unit, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.

(1) Background: Biliary tract cancers (BTCs) are a heterogeneous group of neoplasms with dismal prognosis and the role of adjuvant chemoradiotherapy in high-risk resected patients is unclear. (2) Methods: We retrospectively analyzed the outcomes of BTC patients who received curative intent surgery with microscopically positive resection margins (R1) and adjuvant chemoradioradiotherapy (CCRT) or chemotherapy (CHT) from January 2001 to December 201. (3) Results: Out of 65 patients who underwent R1 resection, 26 received adjuvant CHT and 39 adjuvant CCRT.

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Purpose: There is an increasing interest in the role of sex and gender in cancer patients. The impact of sex differences in oncological systemic therapies is still unknown, and there is a lack of evidence specially in uncommon neoplasms like neuroendocrine tumours (NET). In the present study, we combine the differential toxicities by sex in five published clinical trials with multikinase inhibitors (MKI) in gastroenteropancreatic (GEP) NET.

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Article Synopsis
  • Grade 3 gastro-entero-pancreatic neuroendocrine tumors (G3 GEP-NET) are not well understood regarding their molecular characteristics and treatment responses, prompting a study on their effectiveness with specific chemotherapy regimens.
  • Out of 49 patients in the chemotherapy cohort, those treated with FOLFOX/XELOX showed an objective response rate of 38.2% and median overall survival of 30.0 months, while a Ki67 index of 40% or higher was linked to poorer outcomes.
  • The next-generation sequencing (NGS) of 13 cases revealed that the mutation patterns in G3 NETs resemble those found in well-differentiated neuroendocrine tumors rather than neuro
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Unveiling the Genomic Basis of Chemosensitivity in Sarcomas of the Extremities: An Integrated Approach for an Unmet Clinical Need.

Int J Mol Sci

April 2023

Preclinic and Osteoncology Unit, Biosciences Laboratory, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", 47014 Meldola, Italy.

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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Combining preclinical tools and models to unravel tumor complexity: Jump into the next dimension.

Front Immunol

April 2023

Biosciences Laboratory, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", Meldola, Italy.

Tumors are complex and heterogeneous diseases characterized by an intricate milieu and dynamically in connection with surrounding and distant tissues. In the last decades, great efforts have been made to develop novel preclinical models able to recapitulate the original features of tumors. However, the development of an functional and realistic tumor organ is still utopic and represents one of the major challenges to reproduce the architecture of the tumor ecosystem.

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Nirogacestat, a γ-Secretase Inhibitor for Desmoid Tumors.

N Engl J Med

March 2023

From Sarcoma Medical Oncology, Department of Medicine, Memorial Sloan Kettering Cancer Center, and Weill Cornell Medical College, New York (M.G.), and Northwell Health Cancer Institute, New Hyde Park (T.P.) - all in New York; the Department of Sarcoma Medical Oncology, Division of Cancer Medicine, University of Texas M.D. Anderson Cancer Center, Houston (R.R.); the Department of Oncology, McGill University, Montreal (T.A.); the Department of General Medical Oncology, University Hospitals Leuven, KU Leuven, Leuven (P.S.), the Department of Medical Oncology, Ghent University Hospital, Ghent University, Ghent (L.L.), and King Albert II Cancer Institute, Cliniques Universitaires Saint-Luc, Catholic University of Louvain, Brussels (F.M.) - all in Belgium; the Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam (W.T.G.), and the Department of Medical Oncology, Leiden University Medical Center, Leiden (H.G.) - both in the Netherlands; the Department of Medicine, University of Colorado Cancer Center, Aurora (B.A.W.); Duke Cancer Institute, Duke University Medical Center (R.F.R.), and PharPoint Research (S.M.) - both in Durham, NC; SpringWorks Therapeutics, Stamford (A.L., L.M.S.), and Smilow Cancer Hospital, Yale Cancer Center, Yale School of Medicine, Yale University, New Haven (H.D.) - both in Connecticut; the Department of Hematology and Oncology, Mayo Clinic, Jacksonville (S.A.), and Sylvester Comprehensive Cancer Center, University of Miami Health System, Miami (G.D.) - both in Florida; the Sarcoma Oncology Center, Santa Monica (S.C.), the David Geffen School of Medicine, University of California, Los Angeles, Los Angeles (N.F.), and the Department of Medicine, Division of Oncology, Stanford Cancer Institute, Stanford (N.Q.B.) - all in California; the Sarcoma and Bone Cancer Treatment Center, Dana-Farber Cancer Institute and Harvard Medical School (P.M.), and the Henri and Belinda Termeer Center for Targeted Therapies, Massachusetts General Hospital Cancer Center (G.M.C.) - both in Boston; Washington University in St. Louis, St. Louis (B.A.V.T.); the Department of Medicine and Surgery, Università Campus Bio-Medico di Roma, and Fondazione Policlinico Universitario Campus Bio-Medico - both in Rome (B.V.), the Osteoncology, Bone and Soft Tissue Sarcoma, and Innovative Therapy Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna (E.P.), the Department of Medical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan (S.S.), and Medical Oncology, Candiolo Cancer Institute FPO-IRCCS, Candiolo (G.G.) - all in Italy; the Royal Marsden NHS Foundation Trust (C.B.) and the Department of Medical Oncology, University College London Hospital Foundation Trust (P.D.) - both in London; the University of Michigan Rogel Cancer Center, Ann Arbor (R.C.); the Ohio State University Comprehensive Cancer Center, Columbus (G.T.), and the Cancer and Blood Diseases Institute, Cincinnati Children's Hospital Medical Center, Cincinnati (J.G.P.); the Division of Hematology and Oncology, Department of Medicine, Medical College of Wisconsin, Milwaukee (J.C.), and the University of Wisconsin Carbone Cancer Center, Madison (H.H.B.); the Division of Hematology and Oncology, Department of Medicine, University of Pennsylvania, Philadelphia (L.H.), and the University of Pittsburgh Medical Center, Pittsburgh (M.A.B.); the Sarcoma Center Berlin-Brandenburg, Helios Klinikum Berlin-Buch, Berlin (P.R.), and the University of Heidelberg, Mannheim University Medical Center, Mannheim Cancer Center, Sarcoma Unit, Mannheim (B.K.) - both in Germany; the Knight Cancer Institute, Oregon Health and Science University, Portland (L.E.D., S.K.); and the Division of Medical Oncology, University of Washington, the Clinical Research Division, Fred Hutchinson Cancer Research Center, and Seattle Cancer Care Alliance, Seattle (E.L.).

Article Synopsis
  • * ! The trial showed that nirogacestat significantly improved progression-free survival (76% event-free at 2 years) compared to placebo (44%), with a higher rate of objective responses (41% vs. 8%).
  • * ! While nirogacestat had notable benefits, it also caused common side effects like diarrhea (84%) and nausea (54%), and 75% of women of childbearing potential experienced issues related to ovarian function.
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Systemic Inflammatory Indices in Second-Line Soft Tissue Sarcoma Patients: Focus on Lymphocyte/Monocyte Ratio and Trabectedin.

Cancers (Basel)

February 2023

Preclinic and Osteoncology Unit, Bioscience Laboratory, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", 47014 Meldola, Italy.

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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Purpose: The purpose of this study was to analyze the imaging features of extraskeletal osteosarcomas (ESOS) on computed tomography (CT) and magnetic resonance imaging (MRI) and to investigate their associations with overall survival (OS) using uni- and multivariable survival analyses.

Materials And Methods: This two-center retrospective study included all consecutive adult patients between 2008 and 2021 with histopathologically-proven ESOS who underwent pre-treatment CT and/or MRI. Clinical and histological characteristics, ESOS presentation on CT and MRI, treatment and outcomes were reported.

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Background: Undifferentiated small round cell sarcomas (URCSs) represent a diagnostic challenge, and their optimal treatment is unknown. We aimed to define the clinical characteristics, treatment, and outcome of URCS patients.

Methods: URCS patients treated from 1983 to 2019 at 21 worldwide sarcoma reference centres were retrospectively identified.

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HRAS overexpression predicts response to Lenvatinib treatment in gastroenteropancreatic neuroendocrine tumors.

Front Endocrinol (Lausanne)

February 2023

Bioscience Laboratory, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", Meldola, Italy.

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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Strategies to Overcome Resistance to Immune-Based Therapies in Osteosarcoma.

Int J Mol Sci

January 2023

Osteoncology, Bone and Soft Tissue Sarcomas and Innovative Therapies, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.

Improving the prognosis and cure rate of HGOSs (high-grade osteosarcomas) is an absolute need. Immune-based treatment approaches have been increasingly taken into consideration, in particular for metastatic, relapsed and refractory HGOS patients, to ameliorate the clinical results currently achieved. This review is intended to give an overview on the immunotherapeutic treatments targeting, counteracting or exploiting the different immune cell compartments that are present in the HGOS tumor microenvironment.

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Strontium-doped apatitic bone cements with tunable antibacterial and antibiofilm ability.

Front Bioeng Biotechnol

December 2022

Institute of Science, Technology and Sustainability for Ceramics (ISSMC) (Former ISTEC), National Research Council (CNR), Faenza, Italy.

Injectable calcium phosphate cements (CPCs) represent promising candidates for the regeneration of complex-shape bone defects, thanks to self-hardening ability, bioactive composition and nanostructure offering high specific surface area for cell attachment and conduction. Such features make CPCs also interesting for functionalization with various biomolecules, towards the generation of multifunctional devices with enhanced therapeutic ability. In particular, strontium-doped CPCs have been studied in the last years due to the intrinsic antiosteoporotic character of strontium.

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Article Synopsis
  • Ultra-rare sarcomas are rare types of cancer, happening less than once in a million people each year, and they make up about 20% of all soft tissue and bone sarcomas.
  • A study looked at the effects of a medicine called trabectedin on 36 patients who had these rare cancers, with some showing improvements after treatment.
  • The results showed that a few patients had their cancer shrink or even disappear, but some faced serious side effects from the treatment.
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Lu-DOTATATE Efficacy and Safety in Functioning Neuroendocrine Tumors: A Joint Analysis of Phase II Prospective Clinical Trials.

Cancers (Basel)

December 2022

Nuclear Medicine and Radiometabolic Unit, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", 47014 Meldola, Italy.

Article Synopsis
  • Neuroendocrine tumors (NETs) are rare cancers, with some patients experiencing severe symptoms due to functioning neuroendocrine tumors (F-NETs) that secrete bioactive peptides; existing treatment options are limited.
  • A study analyzed data from 68 patients with progressive metastatic F-NETs who were unresponsive to current treatments, finding that most experienced symptom relief and good disease control with 177 Lu-DOTATATE.
  • Results indicated a median progression-free survival of 33 months and a 2-year overall survival rate of 87.8%, with better outcomes for those who responded to treatment and manageable adverse effects reported.
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In recent years, BRAF inhibitors (BRAFi) and MEK inhibitors (MEKi), together with immune checkpoint inhibitors (ICIs), have changed the therapeutic strategy of cutaneous melanoma, both in adjuvant and metastatic settings. These inhibitors have significantly improved the clinical outcome for patients with melanoma, including in both BRAF-mutated and BRAF-wild type disease. Some preclinical and clinical studies have revealed that BRAFi and MEKi are able to influence T- and B-cell activation, and to modulate immune system activation within the tumor microenvironment.

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Article Synopsis
  • Tenosynovial giant cell tumour (TGCT) is a rare tumor that grows around joints and tendons, with two types: nodular (less aggressive) and diffuse (more aggressive).
  • TGCT can lead to chronic pain and negatively affect a person's quality of life, though it's not usually life-threatening.
  • A global meeting in June 2022 brought together experts and patients to establish better treatment guidelines for TGCT, given the challenges in making consistent treatment choices worldwide.
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Objectives: The ABC-06 and the NIFTY trials recently established the role of second-line chemotherapy (2L) in patients with advanced biliary tract cancer (BTC). Our real-world study aimed to explore 2L in BTC patients aged ≥ 70 years old and to compare their outcomes with younger subjects. Methods: Institutional registries across three academic medical centers were retrospectively reviewed.

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The emerging role of cancer nanotechnology in the panorama of sarcoma.

Front Bioeng Biotechnol

October 2022

Osteoncology Unit, Bioscience Laboratory, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", Meldola, Italy.

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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Pharmacogenomic Profiling of Cisplatin-Resistant and -Sensitive Human Osteosarcoma Cell Lines by Multimodal Targeted Next Generation Sequencing.

Int J Mol Sci

October 2022

Osteoncology, Bone and Soft Tissue Sarcomas and Innovative Therapies, IRCCS Istituto Ortopedico Rizzoli, 40136 Bologna, Italy.

Cisplatin (CDDP) is a drug for high-grade osteosarcoma (HGOS) treatment. Several germline pharmacogenetic studies have revealed associations between single nucleotide polymorphisms (SNPs) and CDDP-based therapy response or CDDP-related toxicity in patients with HGOS. Whether these variants could play a biological role in HGOS cells has not been studied so far.

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Reply to D.J. Benedetti et al.

J Clin Oncol

January 2023

Steven G. DuBois, MD, Dana-Farber/Boston Children's Cancer and Blood Disorders Center, Harvard Medical School, Boston, MA; Abha A. Gupta, MD, Department of Paediatrics, Hospital for Sick Children and Princess Margaret Cancer Center, University of Toronto, Toronto, Canada; and Emanuela Palmerini, MD, Osteoncology, Bone and Soft Tissue Sarcomas and Innovative Therapies Unit, IRCCS Istituto Ortopedico Rizzoli and Bologna University, Bologna, Italy.

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Introduction: Lanreotide autogel (LAN) and temozolomide (TMZ) are guidelines-recommended monotherapies for thoracic neuroendocrine tumors (carcinoids; T-NETs), but prospective data for both combined and monotherapies are lacking. ATLANT (NCT02698410) evaluated efficacy and safety of LAN/TMZ in progressive T-NETs.

Methods: ATLANT was a 12-month, Italian, phase 2, single-arm, open-label, multicenter pilot study.

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