60 A systematic review on intraabdominal and retroperitoneal leiomyosarcomas has highlighted significant gaps in the research surrounding this rare form of cancer. Despite being a life-threatening condition, there is a noted lack of high-quality evidence guiding its diagnosis and treatment. The findings, published in the journal Frontiers in Surgery, underscore the need for more robust studies and clinical trials to better understand and treat the disease. Leiomyosarcomas are a type of soft tissue sarcoma originating in smooth muscles, with about 40% of cases occurring in the abdomen or retroperitoneal space. Though these tumours are relatively rare, accounting for less than 1% of all cancers, they pose a significant threat due to their aggressive nature and the challenges in treatment. A team of researchers from Denmark led the systematic review, which aimed to compile and summarise the information available on these particular leiomyosarcomas. But it discovered a startling lack of data. One of the most alarming revelations from the review was the dearth of randomised clinical trials for abdominal and retroperitoneal leiomyosarcomas. Of the 1,983 studies screened, only one randomised controlled trial and one prospective study were deemed relevant for the systematic review. This limited number of high-quality studies means that much of the available guidance on treatment is based on retrospective data, expert consensus, or experiences with similar soft tissue sarcomas. This shortage of evidence is particularly troubling given the complexity of leiomyosarcomas and their varied presentation. Depending on the tumour’s location, symptoms can range from painless, gradually enlarging masses to more severe issues like intestinal obstruction or internal bleeding. The aggressive nature of the cancer, combined with these vague symptoms, means that the disease is often diagnosed at a more advanced stage, further complicating treatment. For those diagnosed with leiomyosarcoma, surgery is currently the only consistently recommended treatment option. Complete surgical removal with negative margins is considered the gold standard, particularly for those with localised tumours. But the review notes that the outcomes of surgery can vary significantly depending on the tumour’s size, location, and the surgeon’s ability to achieve clear margins. In some cases, surgeons face challenges in removing the tumour without damaging surrounding organs, particularly in retroperitoneal cases where the tumour may press against vital structures like the kidneys, pancreas, or major blood vessels. Despite these difficulties, the review emphasises the importance of surgery in achieving better survival outcomes. While surgery is the preferred first-line treatment, the role of radiotherapy and chemotherapy remains controversial. According to the review, the use of neoadjuvant radiotherapy – administered before surgery – does not appear to significantly impact survival outcomes, particularly in retroperitoneal leiomyosarcomas. Some studies suggest that radiotherapy may help reduce the risk of local recurrence, but this benefit is not consistent across all cases. Similarly, the efficacy of chemotherapy remains inconclusive. Most chemotherapy regimens are reserved for patients with metastatic disease, and the available studies do not offer definitive evidence on whether chemotherapy improves survival in earlier-stage leiomyosarcomas. As a result, the decision to administer chemotherapy often depends on individual patient factors and the specific characteristics of the tumour. The review makes it clear that more research is urgently needed to improve outcomes for patients with abdominal and retroperitoneal leiomyosarcomas. The authors argue that international, multicentre trials could be key to addressing the current gaps in knowledge. Given the rarity of this cancer, collaboration between research institutions across the globe is essential to gather enough data to inform treatment decisions. In the meantime, the review encourages clinicians to continue using a multidisciplinary approach when treating patients with leiomyosarcomas. Surgeons, oncologists, and radiologists should work together to tailor treatment plans to the individual, ensuring that patients receive the best possible care despite the limited evidence available.