Intracranial metastases in sarcoma: a (not so) rare localization, experience of a sarcoma reference center

Résumé fourni par la source

Intracranial metastases (IM) in patients with soft tissue sarcomas (STS) and bone sarcomas (BS) are uncommon. The reported incidence ranges from 1% to 3%, although it varies according to histological subtype, with alveolar soft part sarcoma and Ewing sarcoma showing the highest propensity for central nervous system (CNS) involvement. The development of IM is associated with a poor prognosis. We conducted a single-center retrospective study including patients with STS and BS treated at the Veneto Institute of Oncology between 2010 and 2024. Data collected included age at diagnosis, sex, primary tumor characteristics, date and site of first metastasis, time to IM, and radiological characteristics of IM (number, location, and presence of peritumoral edema). Information on neurological symptoms, treatment strategies, and survival outcomes was also retrieved. Overall survival (OS) was estimated using the Kaplan–Meier method. Among 1,870 patients, including 801 with advanced disease, 30 developed IM, corresponding to an overall incidence of 1.6% and 3.7% among patients with advanced disease. The most frequent histological subtypes were Ewing sarcoma (16.7%), undifferentiated pleomorphic sarcoma (16.7%), and chondrosarcoma (13.3%). The median time from sarcoma diagnosis to IM was 30 months, while the median interval from the onset of metastatic disease to IM was 7 months. Most IM were diagnosed following the onset of neurological symptoms (70%), which were more commonly related to lesion location than to intracranial hypertension. The cerebrum was the most frequent site of involvement (66.7%), followed by the meninges (20.0%) and cerebellum (13.3%). Overall, 23 patients (76.7%) received at least one treatment for IM: 40.0% underwent local treatment alone, 20.0% received systemic therapy alone, and 30.0% received a combination of local and systemic treatments. Among patients receiving local treatment, radiotherapy alone was administered in 57.2% of cases, surgery alone in 21.4%, and combined surgery and radiotherapy in 21.4%. At the time of analysis, 25 of the 30 patients had died. Median OS after IM diagnosis was 2 months (95% CI, 1.14–2.85). In this single-center series of patients with STS and BS, the overall incidence of IM was low, although certain histological subtypes showed a higher risk of CNS involvement. Most IM were solitary and potentially amenable to local treatment. Despite multimodal management, IM remained associated with a dismal prognosis. Further studies are needed to better understand the biological mechanisms underlying CNS dissemination and to identify more effective therapeutic strategies.

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