Publications by authors named "Longhi A"

Background: Stereotactic body radiotherapy (SBRT) is increasingly used to treat lung metastasis (LM) in patients with soft tissue sarcoma (STS) and bone sarcoma (BS).

Methods: This retrospective study evaluated the outcomes of patients with BS and STS treated with SBRT for LM between 2010 and 2023.

Results: We enrolled 102 patients (51 each with STS and BS), of whom 71 were males and 31 were females (median age, 40 years; range, 11-81 years).

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Purpose: The longest diameter (LD) is a strong prognostic factor for patients with soft-tissue sarcoma (STS). Other dimensional assessments, such as the sum of diameters (SoD), product of diameters (PoD), and volume (3D-COG - proposed by the Children Oncology Group), can be rapidly performed; however, their prognostic values have never been compared to LD. Our goal was to investigate their performance in improving patients' prognostication for STS of the lower limbs.

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Importance: The combination of ifosfamide and etoposide (IE) is commonly used to treat relapsed or refractory osteosarcoma; however, second-line treatment recommendations vary across guidelines.

Objective: To evaluate whether the addition of lenvatinib to IE (LEN-IE) improves outcomes in children and young adults with relapsed or refractory osteosarcoma.

Design, Setting, And Participants: The OLIE phase II, open-label, randomized clinical trial was conducted globally across Europe, Asia and the Pacific, and North America.

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Article Synopsis
  • Osteosarcomas are rare bone tumors that make up less than 1% of all cancers and are most commonly found in children, second only to multiple myeloma among primary bone tumors.
  • The imaging characteristics of osteosarcomas vary by histological subtype, with conventional types showing mixed radiological patterns and aggressive features, while telangiectatic forms are typically purely lytic.
  • The review emphasizes the relationship between imaging features and patient outcomes, including treatment response and survival, highlighting important parameters for medical professionals involved in the management of these tumors.
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Background: The prognosis of patients with Relapsed/Refractory Osteosarcoma (R/R OS) remains dismal without an agreement on systemic therapy. The use of High-Dose Ifosfamide (14 g/sqm) with an external pump in outpatient setting (14-IFO) in R/R OS patients is limited. This study represents the first retrospective cohort analysis focused on evaluating the activity and toxicity of 14-IFO in this setting.

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Article Synopsis
  • The study aimed to analyze the histological and imaging characteristics of myoepithelial carcinoma in bone and soft tissue, examining data from 22 patients over time.
  • Results showed that a significant number of patients experienced local recurrence (36.3%) and distant metastasis (50.0%), with severe cytological changes and vascular invasion being noted in many cases.
  • The findings suggest that understanding these histological and imaging features could help identify the aggressive nature of myoepithelial carcinoma, highlighting its high risk for recurrence and metastasis.
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Neurotrophic tyrosine receptor kinase (NTRK) gene-fusion targeted molecules revolutionized the paradigm of treatment of a limited subgroup of cancers of various histologies. Entrectinib and larotrectinib obtained unprecedented response rates in patients with cancer harboring NTRK rearrangements. This evidence recently led to the agnostic approval of these drugs, and evidence (confirmation) of their activity in a broader disease setting is emerging.

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Article Synopsis
  • The study investigates the treatment outcomes and factors influencing myoepithelial carcinoma of soft tissue and bone based on 33 patients treated between 1998 and 2015.
  • The research found that a significant number of patients experienced local recurrences and metastases, with a five-year survival rate of 62.6% for those with localized tumors.
  • The authors emphasize the need for multidisciplinary treatment approaches, as current chemotherapy options do not significantly improve outcomes for patients with metastatic disease.
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Background: Heterozygous isocitrate dehydrogenase (IDH) mutations occur in about half of conventional central bone chondrosarcomas (CCBC). Aim of this study was to assess the frequency and prognostic impact of IDH mutations in high grade CCBC patients.

Methods: 64 patients with G2 and G3 CCBC were included.

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This review focuses on the role of magnetic resonance imaging (MRI) in the evaluation of the gastrointestinal tract (GI MRI), analyzing the major technical advances achieved in this field, such as diffusion-weighted imaging, molecular imaging, motility studies, and artificial intelligence. Today, MRI performed with the more advanced imaging techniques allows accurate assessment of many bowel diseases, particularly inflammatory bowel disease and rectal cancer; in most of these diseases, MRI is invaluable for diagnosis, staging, and disease monitoring under treatment. Several MRI parameters are currently considered activity biomarkers for inflammation and neoplastic disease.

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Fibular fractures are the third most common stress fractures in children and adolescents. Proximal fibular location is a very rare finding, with few reports in the literature and, frequently, careful investigations before a definitive diagnosis could be necessary. The authors report a case of an adolescent 13 years old soccer player with a proximal fibular fracture that was initially underestimated and misdiagnosed and ultimately confirmed as a stress lesion by MRI.

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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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Purpose: The relevance of the subfamily A members of ATP-binding cassette (ABCA) transporters as biomarkers of risk and response is emerging in different tumors, but their mechanisms of action have only been partially defined. In this work, we investigated their role in Ewing sarcoma (EWS), a pediatric cancer with unmet clinical issues.

Methods: The expression of ABC members was evaluated by RT-qPCR in patients with localized EWS.

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Background: Giant cell tumors of bone (GCTB) are osteolytic tumors. Denosumab, a RANK-L inhibitor, is approved for GCTB. Data on serum bone turnover marker (sBTM) changes are lacking.

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Article Synopsis
  • Lurbinectedin effectively targets the EWS-FLI1 oncogenic factor, showing promise in slowing tumor growth in Ewing sarcoma, leading to its clinical evaluation in patients with relapsed disease.
  • The phase II trial involved 28 adult patients who received lurbinectedin, with the main goal of assessing the overall response rate alongside secondary outcomes like progression-free survival and safety.
  • Results indicated a 14.3% response rate, a 2.7-month median progression-free survival, and manageable adverse effects, suggesting that lurbinectedin could be a beneficial option for Ewing sarcoma treatment moving forward.
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Background: According to retrospective osteosarcoma series, ABCB1/P-glycoprotein (Pgp) overexpression predicts for poor outcomes. A prospective trial to assess a risk-adapted treatment strategy using mifamurtide in Pgp+ patients was performed.

Methods: This was a phase 2, multicenter, uncontrolled trial including patients 40 years old or younger with nonmetastatic extremity high-grade osteosarcoma stratified according to Pgp expression.

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Synovial sarcoma is a relatively chemosensitive type of soft tissue sarcoma and it often metastasizes to the lung. We investigated the role of adjuvant chemotherapy in patients with high-grade synovial sarcoma at their first lung metastasectomy (LMTS). Forty-six HGSS patients had their first LMTS at our institute (Rizzoli Orthopedic Hospital, Bologna, Italy) between 2000 and 2020.

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Osteosarcoma is the most frequent primary malignant bone tumour with an impressive tendency to metastasise. Highly proliferative tumour cells release a remarkable amount of protons into the extracellular space that activates the NF-kB inflammatory pathway in adjacent stromal cells. In this study, we further validated the correlation between tumour glycolysis/acidosis and its role in metastases.

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Background: We report results from the phase I dose-finding and phase II expansion part of a multicenter, open-label study of single-agent lenvatinib in pediatric and young adult patients with relapsed/refractory solid tumors, including osteosarcoma and radioiodine-refractory differentiated thyroid cancer (RR-DTC) (NCT02432274).

Patients And Methods: The primary endpoint of phase I was to determine the recommended phase II dose (RP2D) of lenvatinib in children with relapsed/refractory solid malignant tumors. Phase II primary endpoints were progression-free survival rate at 4 months (PFS-4) for patients with relapsed/refractory osteosarcoma; and objective response rate/best overall response for patients with RR-DTC at the RP2D.

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Article Synopsis
  • This study investigates the effectiveness of a treatment combining lenvatinib, etoposide, and ifosfamide in young patients with relapsed or refractory osteosarcoma, aiming to find the optimal dosage and assess its anti-tumor activity.
  • Conducted in 17 hospitals across six countries, the trial focused on patients aged 2-25 with measurable disease and a life expectancy of at least 3 months, evaluating safety and progression-free survival.
  • Thirty patients were screened for the initial phase, with 22 participants advancing to the second phase, assessing how well the combination therapy works over a maximum of five treatment cycles followed by lenvatinib alone.
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Purpose: The main objective was to evaluate the activity and tolerability of TEMIRI as a front-line treatment in primary disseminated Ewing sarcoma (PDMES) using the RECIST 1.1 criteria. The secondary objectives included the assessment of toxicity and the performance status/symptom changes.

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Purpose: To analyze toxicity and outcome predictors in Ewing sarcoma patients with lung metastases treated with busulfan and melphalan (BU-MEL) followed by whole-lung irradiation (WLI).

Methods: This retrospective study included 68 lung metastatic Ewing Sarcoma patients who underwent WLI after BU-MEL with autologous stem cell transplantation, as part of two prospective and consecutive treatment protocols. WLI 12 Gy for <14 years old and 15 Gy for ≥14 years old patients were applied at least eight weeks after BU-MEL.

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Background: Bone and soft-tissue sarcoma are rare tumors. Complementary and alternative medicine (CAM) is often used in cancer patients however limited data are available in sarcoma patients. The aim of the present study is to explore the use of CAM in patients with bone and soft-tissue sarcoma.

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A stable and precise articulation of the distal tibiofibular syndesmosis maintains the tibiofibular relationship, and it is essential for normal motion of the ankle joint. The disruption of this joint is frequently accompanied by rotational ankle fracture, such as pronation-external rotation, and rarely occurs without ankle fracture. The diagnosis is not simple, and ideal management of the various presentations of syndesmotic injury remains controversial to this day.

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