bims-myxlip Biomed News
on Myxoid liposarcoma
Issue of 2024–12–15
two papers selected by
Laura Mannarino, Humanitas Research



  1. Ann Surg Oncol. 2024 Dec 08.
       BACKGROUND: In 2023 and 2024, a wide variety of new studies have been published in the field of soft tissue sarcomas, representing the enormous heterogeneity of sarcoma histotypes, anatomical location, treatment variability, and biological behavior.
    PATIENTS AND METHODS: This article summarizes the, in our view, seven most important publications in the field that will have an impact on the surgical practice and future treatment strategies of our patients.
    RESULTS: In the last year, we gained more insight in the genetic background of patients with sarcoma from a large Australian study, which will have an impact on future counseling and screening of our patients. Also, the role of radiotherapy in retroperitoneal liposarcoma became clearer in the combined STRASS-STREXIT study. Radiotherapy for extremity sarcomas can now also be done in a 3-week, hypofractioned manner, which is a major improvement for our patients. Furthermore, more evidence has been presented regarding the improved outcome of patients with retroperitoneal sarcoma when treated in high volume centers. Landmark studies with systemic treatment include the positive trial with nirogacestat in desmoid tumors, which have finally shifted the paradigm of desmoid treatment from surgery to systemic treatment. Also, the results of 2 phase 3 randomized controlled trials recently showed 1) that the combination of doxorubicin with trabectedin significantly improved progression free survival for patients with leiomyosarcoma when compared with doxorubicin alone and 2) that the combination of neoadjuvant pembrolizumab and radiotherapy followed by 1-yr adjuvant pembrolizumab significantly improved disease free survival for patients with primary localized undifferentiated pleomorphic sarcoma/myxofibrosarcoma or dedifferentiated/pleomorphic liposarcoma of the extremities or girdles.
    CONCLUSIONS: Key papers from the last year informs us that management of soft tissues sarcoma is constantly improving, since more data became available to guide us in defining the best treatment strategies.
    DOI:  https://doi.org/10.1245/s10434-024-16540-x
  2. Neurol Sci. 2024 Dec 14.
       BACKGROUND: Non-meningothelial intracranial mesenchymal tumors are a heterogeneous group of central nervous system neoplasms endowed with great variability clinically and histologically. For this precise reason, significant difficulties exist in specifically cataloguing tumor entities with such distant characteristics and such uncertain clinical course.
    CASE DESCRIPTION: In an attempt to increase the knowledge inherent in this type of central nervous system lesions we report a case of a rare and unusual myxoid mesenchymal tumor of difficult anatomopathological classification characterized by rapid progression and optimal therapeutic response after combined surgical and radiotherapy treatment, with histo-molecular definition and DNA methylation profile. In this case, multidisciplinary management led to timely surgical intervention based on the rapid clinical deterioration and radiological progression; after adjuvant therapy with hadron therapy, the patient has no signs of recurrence two years after the surgical procedure. No FET-CREB fusion was detected, and the DNA methylation profile suggested the presence of multiple chromosomal gains and losses.
    CONCLUSIONS: The molecular definition as well the optimal therapeutic regimen of these tumors is not clearly defined yet and analysis of larger series is strongly warranted.
    Keywords:  Angiomatoid fibrous histiocytoma (AFH); FET-CREB fusion; Intracranial myxoid mesenchymal tumor (IMMT); Mesenchymal non-meningothelial tumors; Myxoid tumor
    DOI:  https://doi.org/10.1007/s10072-024-07907-9