Primary Intracranial Leiomyosarcoma Secondary to Glioblastoma: Case Report and Literature Review

Abstract

BackgroundLeiomyosarcoma is a highly malignant soft-tissue sarcoma with a poor prognosis. In recent years, treatment for leiomyosarcoma has not shown much progress. Primary intracranial leiomyosarcoma (PILMS) is a much rarer type of neoplasm, which occurs more frequently in immunocompromised patients. PILMS cases reported in the literature are scarce and treatment strategy and prognosis are still under debate. In this study, a case of PILMS secondary to the total resection of giant cell glioblastoma is reported.Case DescriptionA 38-year-old male was hospitalized with a three-month history of a temporal opisthotic bump. His medical history included a total resection of a tumor located in the right temporal lobe performed 4 years earlier. Pathological examination led to a diagnosis of giant cell glioblastoma, and the patient underwent postoperative chemotherapy with temozolomide for 6 weeks plus simultaneous radiotherapy with 63.66 Gary. Four years later, during regular follow-up, a preoperative MRI brain scan resulted in a well-defined signal pointing out two nodule-like features located at the right temporal lobe and subcutaneous soft tissue, respectively, and near the area where the previous giant cell glioblastoma was located. The mass was completely removed by a transtemporal approach and postoperative pathology revealed that the mass was a leiomyosarcoma. The patient underwent postoperative radiotherapy and no recurrence occurred until now.ConclusionsTo date, research on soft-tissue sarcoma, especially PILMS, has not made much progress, and a limited number of studies have provided few details on the management of PILMS. The treatment of choice for PILMS is aggressive multimodal treatment based on total tumor resection and radiotherapy. Moreover, systemic treatment with chemotherapy and targeted therapy, such as olaratumab, as well as further research still needs to be performed as many questions are left unanswered. To our knowledge, this is the first report on a case of PILMS secondary to glioblastoma, which might serve as a potential reference for clinicians and clinical studies.

A Necroptosis-Related lncRNA Signature Predicts Prognosis and Indicates the Immune Microenvironment in Soft Tissue Sarcomas

“…STS is a group of rare and aggressive malignancies originating from mesenchymal tissue, including various pathological subtypes ( Dotan et al, 2006 ). A multidisciplinary manner composed of surgery, radiotherapy, and chemotherapy is the routine treatment for STS ( Zhao L. et al, 2021 ). Generally, the 5-years survival rate of patients with localized STS can reach 55.5–56.5%, but these traditional treatments have unsatisfactory curative effects on patients with postoperative metastasis and recurrence ( Hu et al, 2020 ).…”

Epstein-Barr virus–associated primary intracranial leiomyosarcoma in an immunocompetent patient: illustrative case

“… 11 The tumorigenesis of PILMS is unclear, and tumors are thought to arise from the pluripotent mesenchymal cells of the dura mater or cerebral blood vessel epithelium, although the majority of the evidence suggests dural origin. 11 , 16 The clinical presentation of PILMS varies with tumor location, but common symptoms include headache, limb weakness, vomiting, and vision changes. 17 PILMSs have a poor overall prognosis and high rates of local recurrence and metastases.…”

Giant primary intracranial multi-fossa leiomyosarcoma involving the frontal sinus, ethmoid air cells, anterior fossa, middle fossa, and intraventricular space: A case report and literature review

“…[ 11 , 39 ] The demographic distribution of intracranial LMS is skewed toward males with a wide age range reported, from teenagers to the elderly. [ 2 , 25 , 41 , 44 , 46 ] Risk factors for intracranial LMS include a history of immunocompromised state, such as HIV infection, and previous radiation exposure. [ 39 , 41 , 44 , 46 ] The etiology of intracranial LMS is not well understood, but it is believed to arise from the mesenchymal cells of the dura mater or cerebral blood vessels.…”

“…[ 2 , 25 , 41 , 44 , 46 ] Risk factors for intracranial LMS include a history of immunocompromised state, such as HIV infection, and previous radiation exposure. [ 39 , 41 , 44 , 46 ] The etiology of intracranial LMS is not well understood, but it is believed to arise from the mesenchymal cells of the dura mater or cerebral blood vessels. Intracranial LMS traditionally presents in the left lateral ventricle or originates from the skull.…”

“…[ 2 , 25 , 44 ] Common symptoms vary with the location and size of the tumor but often include persistent headaches, nausea, vomiting, decreased appetite, weight loss, and lethargy. [ 2 , 18 , 25 , 39 , 41 , 44 , 46 ] Management of intracranial LMS is challenging due to its rarity. However, gross total resection has been found to be a significantly favorable factor for increased progression-free survival and overall survival.…”