Dados do Trabalho

Título

Primary sarcoma of the central nervous system - Case Report

RESUMO

A 28-years-old woman, presented with headache in the right frontotemporal region starting in July 2019, evolving with visual acuity changes, dizziness and paresthesia in the left upper limb (LLE). Neurological examination showed only bilateral papilledema. Magnetic Resonance Imaging (MRI) of the skull showed a right frontoparietal dural lesion measuring 5.1x4.4x3.8cm, heterogeneous with T1 hyposignal, intense contrast enhancement and increased rCBV inside. She underwent surgical resection in September 2019 with no residual lesion. Histopathological examination identified features suggestive of high-grade fusocellular sarcoma, with immunohistochemistry demonstrating a Ki-67: Positive 50%. During the planning of radiotherapy, the patient progressed with recurrence of complaints, neuroimaging showed disease progression, and a new surgical resection was performed in January 2020. The patient underwent adjuvant three-dimensional conformal radiotherapy (dose 60Gy). It evolves with recurrence of headache and paresis in LLE, in addition to focal motor perceptual onset crisis. Cranial MRI showed progression of the lesion in the same topography. She underwent a third procedure in June 2021, with wide resection, but with a residual lesion. Stereotactic radiosurgery was performed in a residual lesion (dose 25Gy) in September 2021. During clinical follow-up, a protuberance in the surgical wound was identified and MRI of the skull confirmed the progression of the disease with exteriorization through the frontal bone. A new surgical approach was performed in January 2022 with lesion resection, in addition to subdural empyema drainage and surgical wound cleaning. Antibiotic therapy was started, but the patient evolved unfavorably and died in March 2022. Discussion and conclusions: primary sarcoma of the central nervous system (CNS) is a rare neoplasm. It has been hypothesized that these neoplasms originate from a pluripotent mesenchymal lineage within the dura or leptomeninges, extending into the CNS parenchyma. Surgical resection along with chemotherapy and radiation have historically been the cornerstone treatment strategy for CNS sarcoma. Future studies should be focused to understand the histopathological subtypes of primary CNS sarcoma to use different chemotherapeutic agents which be helpful to give patients long survival and best choice of treatment.