TRAbectedin in adVanced rEtroperitoneal well differentiated/dedifferentiated Liposarcoma and Leiomyosarcoma (TRAVELL): results of a phase II study from the Italian Sarcoma Group.

Background: This is a multicentre, single-arm, phase II study aimed at further exploring the activity of trabectedin as second-/further-line treatment in retroperitoneal leiomyosarcoma (LMS) and well-differentiated/dedifferentiated liposarcoma (LPS).

Materials And Methods: The primary endpoint was the growth modulation index (GMI) defined as the ratio between PFS under trabectedin (PFS) and during previous chemotherapy treatment: time to progression (TTP-1). Secondary endpoints were objective response rate (ORR) and PFS.

[Medical Treatments in Oncology 2023].

Medical Oncology since the beginning of the new millennium has recognized a great positive evolution in the care of cancer. In fact, for more than 60 years the two classical pillars of the antineoplastic therapy were hormone therapies mainly applied in breast, prostate and thyroid cancer, and chemotherapy seldom curative and heavily toxic. Nowadays some new treatments are available thanks to the advances in genomics, proteomics and molecular biology of tumor cells either to the advances in immunology studies.

Guideline-Based Follow-Up Outcomes in Patients With Gastrointestinal Stromal Tumor With Low Risk of Recurrence: A Report From the Italian Sarcoma Group.

Importance: Gastrointestinal stromal tumor (GIST) follow-up is recommended by international guidelines, but data on the role of follow-up in patients with low relapse risk are missing. For these patients, the potential benefit of anticipating recurrence detection should be weighed against psychological burden and radiologic examination loads in terms of costs and radiation exposure.

Objective: To evaluate the outcomes of guideline-based follow-up in low-risk GIST.

The psychological impact of sarcoma on affected patients.

Objective: Sarcoma diagnosis and its treatment trajectory may deeply affect the somatopsychic balance of patients and their caregivers. This systematic review aimed at deepening the understanding of sarcoma's impact on the entire family unit involved in the illness experience on a physical (e.g.

The rare entity of gastrointestinal leiomyosarcomas: An Italian multicenter retrospective study in high-volume referral centers.

Background: After a huge efficacy of imatinib in treating patients with gastrointestinal stromal tumors (GISTs) was proven, a maximum effort was made to make a differential diagnosis between GISTs and gastrointestinal leiomyosarcomas (GI-LMS), showing the latter to be an extremely rare tumor entity. Limited data on GI-LMS biology, clinical behavior and drug-sensibility are available, and the clinical decision-making in this subgroup of patients is usually challenging.

Methods: We conducted a multicenter, retrospective observational study on patients with diagnosed GI-LMS from 2004 to 2020 within six high-volume referral centers in Italy.

Chemotherapy in patients with localized angiosarcoma of any site: A retrospective european study.

Background: We retrospectively investigated the role of (neo)adjuvant chemotherapy in patients with primary, localized angiosarcoma.

Methods: We selected all patients with primary, localized angiosarcoma, who had received radical surgery between January 2005 and December 2019 at 33 European sarcoma reference centers. The primary objective was to compare the outcome of patients who received (neo)adjuvant chemotherapy versus those who did not, in terms of overall survival (OS), disease-free survival (DFS) and distant metastasis-free survival (DMFS).

A Pharmacological Analysis of the Activity and Failure of the Medical Treatment of High-Grade Osteosarcoma.

Osteosarcomas (OSs) are a group of neoplasms originating from bone cells, usually presenting in three specific age groups: children, young adults, and the elderly. High-grade OS is an extremely malignant tumor mainly due to evolution into metastatic disease, usually in the lungs. Survival of these patients has improved since the 1980s thanks to close cooperation between oncologists, oncological surgeons and orthopedic surgeons.

The sacral chordoma margin.

Objective: Aim of the manuscript is to discuss how to improve margins in sacral chordoma.

Background: Chordoma is a rare neoplasm, arising in half cases from the sacrum, with reported local failure in >50% after surgery.

Methods: A multidisciplinary meeting of the "Chordoma Global Consensus Group" was held in Milan in 2017, focusing on challenges in defining and achieving optimal margins in chordoma with respect to surgery, definitive particle radiation therapy (RT) and medical therapies.

Salvage Therapy in Advanced Adult Soft Tissue Sarcoma: A Systematic Review and Meta-Analysis of Randomized Trials.

Background: Prognosis for patients with metastatic soft tissue sarcomas (STS) is dismal, with median overall survival (OS) of 8-12 months. The role of second-line therapy has been inconsistently investigated over the last 20 years. This systematic review and meta-analysis was performed to assess the efficacy of salvage treatment in pretreated adult type STS, gastrointestinal stromal tumor (GIST) excluded.

Long-term treatment with eribulin in heavily pretreated women with metastatic breast cancer: a case series.

During the last decades, much effort has been made to develop and test treatments for advanced/metastatic breast cancer (MBC) able to prolong survival and improve patients' quality of life. In this regard, eribulin represents one of the most recent and interesting discoveries. This tubulin-targeting chemotherapy demonstrated a survival benefit in MBC women who progressed after at least two prior lines of chemotherapy for the treatment of metastatic disease (prior therapies should have included an anthracycline and a taxane, in either adjuvant or metastatic setting).

[The importance of mutational status in prognosis and therapy of GIST].

GIST (gastrointestinal stromal tumor) are the most common mesenchymal tumors in gastrointestinal tract and are thought to derive from the cells of Cajal or their precursors that have a constitutional mutation in KIT and PDGFRA genes. There are KIT and PDGFRA genes mutations detected before the start of therapy that are believed to be related to GIST pathogenesis and some secondary mutations causing drug resistance and progression of disease. The most common KIT mutations are detected in exon 11 (66-71%), exon 9 (10-13%), exon 13,14,17 (1% each).

Phase II prospective study with sorafenib in advanced soft tissue sarcomas after anthracycline-based therapy.

Introduction: We investigated the activity and safety of sorafenib, a multitargeted tyrosine-kinase inhibitor, in patients with advanced soft tissue sarcomas (STS).

Patients And Methods: An open-label nonrandomised multicentre phase II study was conducted in advanced STS patients pre-treated with anthracycline-based chemotherapy. Patients received sorafenib 400 mg twice daily for 28 days.

A phase 2 trial of imatinib mesylate in patients with recurrent nonresectable chondrosarcomas expressing platelet-derived growth factor receptor-α or -β: An Italian Sarcoma Group study.

Background: Chondrosarcoma (CS) is a rare and heterogeneous sarcoma in which, after failure of surgery and radiotherapy, chemotherapy plays only a marginal role. Different molecular pathways have been shown to be activated in CS; in particular, both isoforms of platelet-derived growth factor receptor (PDGFR) are expressed and phosphorylated. These observations prompted investigation of the activity of imatinib mesylate (IM) in patients with advanced CS in a phase 2 trial.

Vinorelbine and fluorouracil plus leucovorin combination (ViFL) in patients with anthracycline and taxane-pretreated metastatic breast cancer: a phase II study.

Purpose: This phase II study was designed in order to evaluate efficacy and safety of the combination of vinorelbine (VNB), fluorouracil (FU) and leucovorin (LV) in patients with metastatic breast carcinoma (MBC) previously treated with anthracyclines and taxanes.

Methods: From 12/2003 to 12/2007, 51 women (median age 59) were treated. Performance status (PS) (ECOG) was 0-2 (median 0).

Weekly irinotecan plus protracted venous fluorouracil infusion (WI-FI) in advanced colorectal cancer: a phase II study.

Background: Irinotecan (IRI) is a topoisomerase I inhibitor active as first- or second-line chemotherapy in advanced colorectal cancer (ACRC). Its combination with fluorouracil (FU) increases the response rate and prolongs survival. In order to identify a new effective and less toxic schedule of administration, we planned this phase II study with weekly IRI and protracted venous infusion of FU (WI-FI regimen).

The retinoblastoma family member pRb2/p130 is an independent predictor of survival in human soft tissue sarcomas.

Purpose: pRb2/p130, a member of the Retinoblastoma gene family, has been shown to be a powerful prognostic factor in several malignancies. We sought to evaluate pRb2/p130 protein expression and its clinical effect in patients affected with soft tissue sarcomas (STS).

Experimental Design: Expression of pRb2/p130 was evaluated by immunohistochemistry on formalin-fixed, paraffin-embedded sections in 41 STSs.

New advances in biology and treatment of gastrointestinal stromal tumours (GISTs).

Gastrointestinal stromal tumours (GISTs) are a precise oncogenetic entity that has started the new era of targeted therapies in solid tumours. GISTs are now a model to understand the role of oncogenic kinase mutation in tumourigenesis, duplication, cell regulation, apoptosis and drug resistance. Ninety-five percent of GISTs have some activacting mutation of c-KIT or PDGFRA tyrosine kinase.

Biology, diagnosis and therapeutic options in gastrointestinal stromal tumours.

Gastrointestinal stromal tumours (GIST) are the most common form of mesenchymal tumour of the intestinal tract. The incidence in Italy is approximately 800-1,400 new cases/year; the most common localization is the stomach (50-60%), small bowel (20-30%), rectum (10%) and esophagus (5%). Extra-abdominal localizations are very rare.

High dose methotrexate in adult patients with osteosarcoma: clinical and pharmacokinetic results.

High dose methotrexate (HDMTX) with folinic acid rescue is widely used to treat osteosarcoma, which predominantly afflicts children; the study investigated HDMTX pharmacokinetics (pk) in adult subjects in neoadjuvant/adjuvant settings. Twenty five patients with advanced osteosarcoma (11 females--14 males, median age 26.0 years) were treated by 12 g/m2 HDMTX 4 hour iv infusion (64 total courses, range 1-7 courses).

Echocolor Power Doppler with contrast medium to evaluate vascularization in lesions of the soft tissues of the limbs.

Purpose: Echocolor Power Doppler with contrast medium forms a non-invasive vascular image; the purpose of the study is to evaluate the effectiveness in differentiating benign and malignant tumors in the soft tissues of the limbs.

Material And Method: Echocolor Power Doppler with contrast medium was used to study 80 patients with swelling in the soft tissues of the limbs: there were 54 benign lesions, 22 sarcomas, and 4 aggressive desmoid fibromatoses.

Results: Were identified 4 patterns of wash-in and wash-out curves that could be correlated to the histological diagnosis: type I was present in 85% of benign lesions, type III in 91% of malignant lesions and in 3.

Two episodes of ifosfamide-related neurotoxicity in the same patient following different schedules and doses of the drug. A case report.

We report the first case of recurrent ifosfamide-related neurotoxicity in the same patient following two distinct administrations of the drug at different doses and schedules and with a long interval between the two episodes. Remarkably, the first event was characterized by confusion and hallucinations, while the second, 29 months later, was characterized by partial and generalized seizures. Between the two episodes the patient had received high-dose cyclophosphamide, an oxazophoshorine agent closely related to ifosfamide, without any neurological side effects.

Treatment of advanced colorectal cancer (CRC) in daily practice: results of a survey in two Italian regions, Piemonte and Valle d'Aosta.

Aims And Background: Colorectal cancer (CRC) is one of the most important health problems in Western countries: it is the fourth cancer in terms of incidence and the second cause of cancer death. Surgery is the main therapeutic choice and there is broad consensus on the role of adjuvant chemotherapy (CT) after resection. Unfortunately, 50% of the patients will relapse and die of the disease.