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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. Results: Thirty‐three patients had diagnosis of KIT‐negative GI‐LMS confirmed by sarcoma‐expert pathologist. The most common site of origin was the intestine. Twenty‐two patients had localized disease and underwent surgery: with a median follow‐up of 72 months, median disease‐free survival was 42 months. Overall survival (OS)‐rate at 5 years was 73% and median OS was 193 months. Five out of 10 patients with local relapse received a salvage surgery, and 2/5 remained with no evidence of disease. Thirteen patients received neoadjuvant (6) or adjuvant (7) chemotherapy, and 2/13 patients remained free from relapse. The median OS for patients with metastatic LMS was 16.4 months. Conclusion: GI‐LMS is very rare and extremely aggressive subgroup of sarcomas with a high tendency to systemic spread. Localized GI‐LMS at diagnosis may be cured if treated with adequate surgery with or without (neo) adjuvant chemotherapy, while de‐novo metastatic disease appeared to have a poor prognosis. Clinical effort to understand GI‐LMS biology and clinical behavior and to develop active treatment strategy, especially for metastatic‐disease, is warranted. [ABSTRACT FROM AUTHOR]

• Angiosarcoma presents unique challenges in clinical management of localized disease, due to its rarity and aggressiveness. • Dedicated clinical recommendations for angiosarcoma are currently lacking despite the applicability of ESMO guidelines. • The Italian Sarcoma Group, together with patient's advocates from "Sofia nel Cuore Onlus", convened to develop specific recommendations, aiming to standardize and harmonize clinical practices for localized angiosarcoma management. 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. Indeed, AS are one of the sarcoma types most sensitive to cytotoxic chemotherapy. Despite this, uncertainties persist regarding optimal management across different clinical presentations, highlighting the need for further investigation through clinical trials. The Italian Sarcoma Group (ISG) organized a consensus meeting on April 1st, 2023, in Castel San Pietro, Italy, bringing together Italian sarcoma experts from several disciplines and patient representatives from "Sofia nel Cuore Onlus" and the ISG patient advocacy working group. The objective was to develop specific clinical recommendations for managing localized AS within the existing framework of sarcoma clinical practice guidelines, accounting for potential practice variations among ISG institutions. The aim was to try to standardize and harmonize clinical practices, or at least highlight the open questions in the local management of the disease, to define the best evidence-based practice for the optimal approach of localized AS and generate the recommendations presented herein. [ABSTRACT FROM AUTHOR]