FAP-α in soft tissue and bone sarcomas: A new potential diagnostic and therapeutic biomarker in the era of precision medicine.

Fibroblast activation protein alpha (FAP-α) is a membrane-bound serine protease expressed by mesenchymal cells within the tumor microenvironment. It has garnered significant attention due to its involvement in tumorigenesis in several neoplasms, with a specific attention in sarcomas due to their mesenchymal origin. In this review, the authors explore the biological functions of FAP-α and its implications in sarcoma pathophysiology.

Enhancing patient-centered care with AI: a study of responses to neuroendocrine neoplasms queries.

Introduction: Large Language Models (LLMs) are increasingly used in oncology, but their application in neuroendocrine neoplasms (NENs) is still unexplored.

Aim: To evaluate the accuracy, clarity, and completeness of LLM responses to clinically relevant NEN management questions.

Material And Methods: A study was conducted from October to December 2024, during which a team of experts posed nine key NEN management questions to three LLMs: ChatGPT Plus, Microsoft Copilot, and Perplexity.

Locoregional Non-Surgical Treatments for Unresectable or Oligometastatic Sarcomas: A Literature Review.

Sarcomas are rare neoplasms, whose complex management is a consequence of their heterogeneity. Due to their variegate histology and characteristics, prospective trials are challenging to design. Thus, diagnostic and therapeutic guidelines are often based on limited evidence available, and only few and dated systemic treatment regimens are included in our current practice.

A Rare Malignant Case of a Primary Pseudomyogenic Haemangioendothelioma of the Bone.

Pseudomyogenic haemangioendotheliomas (PMH) are exceedingly rare, mostly occurring in soft tissue, with malignant cases even more uncommon. In this report, we present a case of a 28-year-old male initially suspected of having a fibroblastic osteosarcoma of the right femur, which was then correctly diagnosed as a primary pseudomyogenic hemangioendothelioma of the bone with synchronous metastases to other skeletal segments. Molecular analysis through targeted RNA sequencing confirmed the correct diagnosis, revealing a fusion transcript .

The efficacy of streptozotocin in managing pancreatic neuroendocrine neoplasms - A systematic review.

Pancreatic neuroendocrine tumors (pan-NETs) represent a highly heterogeneous and complex pathology, with therapeutic management and prognosis influenced by several biological and clinical characteristics. Chemotherapy, including regimens based on capecitabine and temozolomide (CAPTEM) or the combination of streptozotocin and 5-fluorouracil (STZ-5FU), is indicated for rapidly growing, symptomatic, or high-burden disease requiring swift cytoreduction. Historical studies provide scientific evidence for the STZ-5FU regimen, often retrospective and frequently analyzing small series.

SaLudo: a randomized phase IIb/III study of lurbinectedin plus doxorubicin as first-line treatment in leiomyosarcoma.

Previous phase I/II trials indicate promising activity of lurbinectedin plus doxorubicin (DOX) in leiomyosarcoma (LMS). We describe here the rationale and design of SaLuDo, an open label, randomized, multicenter, seamless phase IIb/III study to evaluate the antitumor activity and safety of lurbinectedin plus DOX DOX alone in the first-line setting of metastatic LMS. The phase IIb stage will evaluate two schedules of the combination for the phase III stage given every 3 weeks (q3wk): DOX 50 mg/m plus lurbinectedin 2.

Assessing Urinary Para-Hydroxyphenylacetic Acid as a Biomarker Candidate in Neuroendocrine Neoplasms.

The management of neuroendocrine neoplasms (NENs) involves the measurement of serum chromogranin A (s-CGA), serum neuro-specific enolase (s-NSE), and urinary 5-hydroxindolacetic acid (5-HIAA). Urinary para-hydroxyphenylacetic acid (u-pHPAA), a metabolite of tyrosine, has been proposed as a potential biomarker for these diseases. This study aims to evaluate the effectiveness of u-pHPAA and tyrosine as biomarkers.

Sunitinib for the treatment of patients with advanced pheochromocytomas or paragangliomas: The phase 2 non-randomized SUTNET clinical trial.

Background: Metastatic Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors characterized by high morbidity and limited systemic treatment options, mainly based on radiometabolic treatments or chemotherapy. Based on the preclinical rationale that PGGLs carcinogenesis relies on angiogenesis, treatment with tyrosine kinase inhibitors (TKI) may represent another viable therapeutic option.

Methods: We conducted a prospective phase II study in patients with metastatic or unresectable PGGLs.

Plexiform Fibromyxoma in the Stomach: Immunohistochemical Profile and Comprehensive Genetic Characterization.

Plexiform fibromyxoma (PF), also referred to as plexiform angiomyxoid myofibroblast tumor, is an exceedingly rare mesenchymal neoplasm primarily affecting the stomach. Herein, we present a case of PF diagnosed in a 71-year-old male with a history of lung cancer, initially suspected to have a gastrointestinal stromal tumor (GIST) of the stomach, who subsequently underwent subtotal gastrectomy. The histopathological and molecular features of the tumor, including mutations in , , , , , and MALAT1-GLI1 fusion, are elucidated and discussed in the context of diagnostic, prognostic, and therapeutic considerations.

Clinical recommendations for treatment of localized angiosarcoma: A consensus paper by the Italian Sarcoma Group.

Angiosarcoma (AS) represents a rare and aggressive vascular sarcoma, posing distinct challenges in clinical management compared to other sarcomas. While the current European Society of Medical Oncology (ESMO) clinical practice guidelines for sarcoma treatment are applicable to AS, its unique aggressiveness and diverse tumor presentations necessitate dedicated and detailed clinical recommendations, which are currently lacking. Notably, considerations regarding surgical extent, radiation therapy (RT), and neoadjuvant/adjuvant chemotherapy vary significantly in localized disease, depending on each different site of onset.

The Management of Phaeochromocytomas and Paragangliomas in the Era of Precision Medicine: Where Are We Now? Evidence-Based Systemic Treatment Options and Future Cluster Oriented Perspectives.

Pheochromocytomas (PCCs) and Paragangliomas (PGLs), commonly known as PPGLs to include both entities, are rare neuroendocrine tumors that may arise in the context of hereditary syndromes or be sporadic. However, even among sporadic PPGLs, identifiable somatic alterations in at least one of the known susceptibility genes can be detected. Therefore, about 3/4 of all PPGL patients can be assigned to one of the three molecular clusters that have been identified in the last years with difference in the underlying pathogenetic mechanisms, biochemical phenotype, metastatic potential, and prognosis.

Metabolomic profile of neuroendocrine tumors identifies methionine, porphyrin, and tryptophan metabolisms as key dysregulated pathways associated with patient survival.

Objective: Metabolic profiling is a valuable tool to characterize tumor biology but remains largely unexplored in neuroendocrine tumors (NETs). Our aim was to comprehensively assess the metabolomic profile of NETs and identify novel prognostic biomarkers and dysregulated molecular pathways.

Design And Methods: Multiplatform untargeted metabolomic profiling (GC-MS, CE-MS, and LC-MS) was performed in plasma from 77 patients with G1-2 extra-pancreatic NETs enrolled in the AXINET trial (NCT01744249) (study cohort) and from 68 non-cancer individuals (control).

Prognostic Assessment of Gastropancreatic Neuroendocrine Neoplasm: Prospects and Limits of Radiomics.

Neuroendocrine neoplasms (NENs) are a group of lesions originating from cells of the diffuse neuroendocrine system. NENs may involve different sites, including the gastrointestinal tract (GEP-NENs). The incidence and prevalence of GEP-NENs has been constantly rising thanks to the increased diagnostic power of imaging and immuno-histochemistry.

One-way endobronchial valves in the management of complex persistent air leaks in a soft tissue sarcoma patient.

Complex persistent air leak (PAL) is a clinical condition which is difficult to treat. Herein, we report the clinical case of an 18-year-old woman with lung and bone metastases due an ultrarare sarcoma: "round cell sarcoma non-Ewing". She developed persistent air leaks due to an alveolopleural fistula which developed following two cycles of chemotherapy with doxorubicin.

Radiomics and outcome prediction to antiangiogenic treatment in advanced gastroenteropancreatic neuroendocrine tumours: findings from the phase II TALENT trial.

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.

The Impact of O6-Methylguanine-DNA Methyltransferase () Promoter Methylation on the Outcomes of Patients with Leiomyosarcoma Treated with Dacarbazine.

Dacarbazine is an important drug in the therapeutic landscape of leiomyosarcoma (LMS). Alkylating agents are subjected to resistance mechanisms based on anti-apoptotic pathways and repair mechanisms, including the DNA repair enzyme O6-methylguanine-DNA methyltransferase (). In this retrospective study, the methylation status of the promoter in histological tumor samples from patients with LMS, dacarbazine-based regimens-treated, was measured and correlated with clinical outcomes aimed at optimizing the use of dacarbazine in soft tissue sarcomas.

Sex differences on multikinase inhibitors toxicity in patients with advanced gastroenteropancreatic neuroendocrine tumours.

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.

COVID-19 in patients with neuroendocrine neoplasms: 2-year results of the INTENSIVE study.

We conducted a retrospective/prospective worldwide study on patients with neuroendocrine neoplasms (NENs) and a molecularly proven SARS-CoV-2 positivity. Preliminary results regarding 85 patients of the INTENSIVE study have been published in 2021. Now we are reporting the 2-year analysis.