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Título

Brain Metastasis due to Retroperitoneal Leiomyosarcoma: a case report

RESUMO

CASE PRESENTATION: We present the case of a 53-year-old man with biopsy-proven retroperitoneal leiomyosarcoma (LMS) and pulmonary metastasis. Chemotherapy was started, with stability of the abdominal lesion and regression of the lung metastasis. Nine months after the diagnosis, he presented with progressive headache and an episode of epileptic seizure. Head MRI showed metastatic brain lesion in the right parietal lobe. He underwent surgical resection followed by stereotactic radiosurgery (total dose of 2000 cGy) and pathological examination confirmed LMS metastasis. Control head MRI was performed 1 month after radiosurgery and showed a new right frontal lesion, which was also managed with stereotactic radiosurgery (total dose 3000 cGy). After a period of 6 months of clinical and radiological stability, a new lesion close to the 3rd ventricle appeared, the previous brain lesions increased, and the systemic disease progressed. He evolved with global functional decline, and palliative care was then instituted. DISCUSSION: Sarcoma refers to a cancer that arises from cells of mesenchymal origin while LMS is a rare histological subtype that originates from smooth muscle. It is the second most common soft-tissue sarcoma to affect the retroperitoneum. Sarcoma metastasis to the central nervous system is uncommon, occurring in 1-8% of the cases, with LMS being the most common subtype. Among LMS of all sites, the retroperitoneal LMS has the worst prognosis and about 80% - 87% of these patients die within 5 years. LMS responds poorly to chemotherapy and radiation, being considered a resistant cancer; thus the best results come from early surgery, when feasible. Retroperitoneal LMS is often fatal owing to local recurrence and distant metastases. As our knowledge of retroperitoneal LMS brain metastasis is based on a few cases reports, the clinical management and adjuvant therapy of theses tumors remain based on individualized decision-making. COMMENTS: This is one of the few cases described in the literature of brain metastasis from retroperitoneal LMS treated with surgical resection and stereotactic radiosurgery and, to the best of our knowledge, the only case described with documentation of the treatment of the disease progression. Reports like this aim to contribute to the development of a body of evidence that will guide the management of similar cases.

Palavras Chave

leiomyosarcoma retroperitoneal metastasis neurooncology