Background: Primary intracranial-extracranial communicating leiomyosarcomas, capable of invading both the intracranial and extracranial regions and involving complex anatomical structures, are exceedingly rare neoplasms.
Case presentation: A 37-year-old male patient who initially presented with a subcutaneous mass on the left frontal vertex. Following surgical intervention, a recurrent lump appeared on the left frontotemporal vertex. Symptoms, computed tomography (CT), and magnetic resonance imaging (MRI) revealed a lump on the left frontal vertex accompanied by an irregular abnormal lesion. On both occasions, the diagnosis of leiomyosarcoma was confirmed. The patient underwent leiomyosarcoma excision under general anesthesia. Recurrence of the leiomyosarcoma occurred 2 years and 4 months post-surgery, necessitating an expanded excision of the lesion. After 2 years of follow-up, no significant complications were observed, and the patient's condition remains stable.
Conclusion: Primary extracranial communicating leiomyosarcoma is an exceptionally rare entity, with surgery currently serving as the primary treatment modality. The decision to excise the lesion should be based on the patient’s age, tumor location, pathological features, and the presence of distant metastases.