Primary Intracranial Leiomyosarcoma Research Articles

Primary Intracranial Leiomyosarcoma- Report of a Rare Case with Literature Review

A 29 years old man presented with a H/O headache and vomiting for 4 months, blurring of vision and gait ataxia for 2 months. A contrast MRI of brain indicated the lesion arose from the dura over left cerebellar hemisphere. A posterior fossa craniotomy was performed with a provisional diagnosis of meningioma. Histopathology and immunohistochemistry revealed Leiomyosarcoma. Further evaluation including Contrast CT and PET scan failed to identify any primary site. Primary intracranial leiomyosarcoma is very rare and only few cases were previously reported. The prognosis for primary intracranial leiomyosarcoma is poor with the longest reported survival being 32 months. Bang. J Neurosurgery 2023; 13(1): 48-50

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

Primary intracranial leiomyosarcomas (PILMSs) are extremely rare tumors arising from smooth muscle connective tissue. PILMSs have been shown to be associated with Epstein-Barr virus (EBV). Thus far, EBV-associated PILMS has been exclusively described in immunocompromised patients. A 40-year-old male presented with a 2-year history of left-sided headaches, nausea, and vomiting. Magnetic resonance imaging demonstrated a large, heterogeneously enhancing, lobulated, dura-based mass arising from the left middle cranial fossa with associated edema and mass effect. The patient underwent an uncomplicated resection of suspected meningioma; neuropathology revealed the exceedingly rare diagnosis of EBV-associated PILMS. Follow-up testing for human immunodeficiency virus (HIV) and other immunodeficiencies confirmed the patient's immunocompetent status. Primary intracranial smooth muscle tumors are often misdiagnosed as meningiomas due to their similar appearance on imaging. PILMSs have a poor prognosis and gross total resection is the mainstay of treatment in the absence of clear recommendations for management. Prompt diagnosis and resection are important; therefore, these tumors should be included in the differential of dura-based tumors, especially among immunocompromised patients. Although EBV-associated PILMSs usually occur in immunocompromised individuals, their presence cannot be ruled out in immunocompetent patients.

RARE-11. PRIMARY INTRACRANIAL LEIOMYOSARCOMA IN A PATIENT WITH NEUROFIBROMATOSIS TYPE 1

Primary intracranial leiomyosarcoma (LMS) is very rare, with only a few reported cases. Only one prior case report of intracranial LMS in a patient with neurofibromatosis type 1 (NF1) was identified. We report a case of primary intracranial LMS in a patient with known history of NF1. Our patient is a 17-years-old female without history of immunocompromise presenting with severe headaches representative of right frontal hemorrhagic tumor found to be primary intracranial LMS. In prior reported cases, most primary intracranial LMS were treated with sarcoma-based chemotherapy and radiation therapy. Our patient underwent multiple resections, as well as focal radiation. Her chemotherapy initially included ifosfamide, carboplatin, and etoposide, but when she failed etoposide twice due to severe allergic reactions, she completed treatment successfully with the combination of ifosfamide and doxorubicin. She continues to be doing well with no evidence of disease at 41 months post-treatment.

Primary intracranial leiomyosarcoma presenting with frontal bone mass: a case report

Primary intracranial mesenchymal neoplasms are rare tumors. These tumors are usually metastatic disease from other primary sites. We presented a 31-year-old man with a 6-month history of gradually enlarging frontal mass and positional headache. There was no other symptom demonstrating other organs’ involvement. The patient underwent an uncomplicated craniotomy with clear surgical margins. The pathology review and the immunohistochemistry staining confirmed leiomyosarcoma grade II. We prescribed radiation therapy with tumor dose of 60 Gy in 30 fractions with conformal treatment planning to the tumor bed. As this disease has a high potency for metastasis, we advised four courses of single agent doxorubicin chemotherapy 75 mg/m2 every 4 weeks starting one month after the end of radiotherapy. In the last follow-up visit 34 months later, the patient was disease free in physical exam and imaging findings.

Surgical management and long-term outcomes of primary intracranial leiomyosarcoma: a case series and review of literature.

Primary intracranial leiomyosarcomas (PILMSs) are extremely rare neoplasms, and their management remains unclear. The authors aimed to enunciate the radiological features and design a treatment protocol based on previously published cases combined with our series. Clinical data from all 12 cases of PILMS treated at their institute between 2008 and 2018 were reviewed. Meanwhile, they searched the Ovid MEDLINE, Embase, PubMed, Web of Science and Cochrane databases using the keywords "leiomyosarcoma" and "intracranial," "central nervous system," "cerebral," or "brain" Previously published data were processed and used according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The authors' cohort included 4 males (33.3%) and 8 females (66.7%) ranging in age from 13 to 67 years with a mean of 44.1 ± 5.0 years. Gross total resection (GTR), subtotal resection, and partial resection were achieved in 8 (66.7%), 3 (25%), and 1 (8.3%) patient, respectively, and only four patients (33.3%) received adjuvant therapy after surgery. After a mean follow-up of 30.5 ± 7.6 months, 6 patients (50%) experienced recurrences, and all of them died at the final. Twenty-seven patients (15 were male and 12 were female), in the literature cases, were identified between January 1980 and November 2019, presenting with PILMS. GTR and non-GTR were achieved in 21 (77.8%) and 6 (22.2%) patients, respectively. Postoperative radiotherapy was administrated in 17 patients (63.0%), and postoperative chemotherapy was administrated in 9 patients (33.3%), After a mean follow-up of 22.2 ± 4.1 months, recurrence and death occurred in 8 (36.4%) and 4 (14.8%) cases, respectively. In the pooled cases, the univariate analysis model revealed that only GTR was a significantly favorable factor for increased progression-free survival (hazard ratio 0.270, 95% confidence interval = 0.093-0.787, p = 0.016) and overall survival (hazard ratio 0.255, 95% confidence interval = 0.073-0.890, p = 0.032). GTR was recommended as an optimal treatment; meanwhile, postoperative radiation was also a choice to help increase the survival of patients of PILMS.

Primary intracranial leiomyosarcoma in an immunocompetent patient

Rationale:Primary intracranial leiomyosarcoma (LMS) is an extremely rare tumor in the central nervous system (CNS), and usually seen in immunocompromised individuals. Only a few cases of primary intracranial LMS have been documented in the literature and no study focused on their MRI findings. We reported a case of primary intracranial leiomyosarcoma in a immunocompetent patient and review its imaging features.Patient concerns:A 20-year-old female was admitted to our hospital, complaining with nausea, weight loss and progressive headache in recent 2 years.Diagnosis:The magnetic resonance imaging scan of the brain revealed a large well-defined extra-cerebral mass adherent to left temporal meninges. The mass was iso-intense on T1-weighted images (T1WI), lightly iso to hypointense on T2-weighted images (T2WI) and enhanced uniformly with contrast medium. The preoperative diagnosis is meningioma.Interventions:A left craniotomy was performed for the complete resection of the mass.Outcomes:The lesion was diagnosed via surgical histopathology and immunochemistry as leiomyosarcoma. No evidence of recurrence and complications were found in the following 13 months.Lessions:Primary intracranial leiomyosarcoma which has some special MR imaging features should be considered in the differential diagnosis of intracranial tumor.

Primary Intracranial Leiomyosarcoma - Case Report and Principles for Treatment

Primary Intracranial Leiomyosarcoma - Case Report and Principles for Treatment

Primary Intracranial Thalamic Leiomyosarcoma: Clinical Report of a Case and Review of the Literature

Purpose:The incidence of the primary intracranial leiomyosarcoma is extremely rare, and few cases have been previously reported worldwide to date. This report was to clarify the potential role of radiotherapy in the management of primary intracranial leiomyosarcoma. Methods and Materials:This report presented a 49-year old man with a 3-month history of a progressively headache and walking unsteadily. The diagnosis was confirmed with thalamic leiomyosarcomaof high-grade malignancyaccording to the pathologic examination after neurosurgical biopsy. The patient didnt undergo surgical resection because of a high risk death. After biopsy, radiotherapy using 3D-CRT technique to the mass site with 55.8Gy/31f/43d was given accordingly. Results: The mass didnt reduce much at the end of radiotherapy. The patient refused systemic chemotherapy, he was alive without signs of local relapse and brain side-effectswith 6 month follow-up. After living eleven months and three weeks after radiotherapy, he died of local progression. Conclusions: Through literature review, the current therapeutic approaches including surgery, radiotherapy as well as chemotherapy appear to have limited effect, but could be beneficious of patients in tumor local control and improvement of the life quality.

Primary Intracranial Leiomyosarcoma- Case Report and Review of the Literature

Primary Intracranial Leiomyosarcoma- Case Report and Review of the Literature

Primary intracranial leiomyosarcoma of the torcular Herophili associated with Fanconi anemia and allogenic stem cell transplantation

Fanconi anemia is associated with a high risk for developing malignant tumors. The occurrence of primary intracranial leiomyosarcoma, however, which in general has a poor prognosis, has not been described thus far. The purpose of this study is to report on management and outcome of leiomyosarcoma of the torcular Herophili associated with Fanconi anemia in a pediatric patient. A 12-year-old girl with Fanconi anemia presented with a primary intracranial leiomyosarcoma arising from the torcular Herophili and infiltrating the adjacent venous sinuses after previous allogenic hematopoietic stem cell transplantation. Radical tumor resection followed by radiotherapy resulted in tumor-free survival and good outcome at a 2-year follow-up. Despite occurrence of leiomyosarcoma in a site thought unfavorable for surgery, combined tumor resection and radiosurgery may yield excellent outcome.

Primary intracranial β-human chorionic gonadotropin–producing leiomyosarcoma in a 2-year-old immunocompetent child

The authors present a rare case of primary intracranial leiomyosarcoma (LMS) in a young, immunocompetent boy. The patient presented with an expanding right forehead mass. Diagnostic workup revealed multiple large intracranial tumors. The largest mass was resected, and pathological analysis revealed LMS. Given the poor prognosis of this tumor, the family declined further care, and the child died 3 months later. Primary LMSs are extremely rare tumors in the pediatric population, especially in patients who are not immunocompromised.

Primary intracranial leiomyosarcoma in an immunocompetent patient

Primary intracranial leiomyosarcoma in an immunocompetent patient

Primary Intracranial Leiomyosarcoma in an Immunocompetent Adult

Primary Intracranial Leiomyosarcoma in an Immunocompetent Adult

Primary intracranial leiomyosarcoma in an immunocompetent patient: case report

We report a case of intracranial leiomyosarcoma (LMS) arising after resection of neurofibroma at the cerebellopontine angle. A 45-year-old immunocompetent woman presented with recurrence of a tumor 9 years after resection performed in another hospital. Magnetic resonance imaging demonstrated a heterogeneously enhancing, dura-based mass at the left cerebellopontine angle. The tumor was subtotally removed via lateral suboccipital craniotomy. LMS was diagnosed based on histological and immunohistochemical findings. Postoperatively, although the patient was treated using local radiotherapy, she died due to rapid regrowth of the tumor. Reevaluation of the specimen obtained in the first operation led to a diagnosis of neurofibroma. Both LMS and neurofibroma rarely occur intracranially. LMS is generally thought to arise from smooth muscle cells of the blood vessels or pluripotent mesenchymal cells. In this case, LMS might also have originated from smooth muscle cells of the vessels in the neurofibroma, possibly associated with mechanical and/or heat stimulation during the previous surgery.

Primary intracranial leiomyosarcoma in an HIV-infected patient

Primary intracranial leiomyosarcomas are rare tumors arising from the mesenchymal cells of the dura matter or the cerebral blood vessels. Only 14 cases of primary intracranial leiomyosarcoma are reported in the literature. We report a case of primary intracranial leiomyosarcoma in an human immunodeficiency virus-positive patient with a CD4 count of 14cells/μL. Additionally, in-situ hybridization of Epstein-Barr virus (EBV) early RNA stained sections highlighted the tumor cells, consistent with the presence of EBV. Review of the literature strongly suggests an association between AIDS, EBV, and primary intracranial leiomyosarcoma. Given the paucity of information in the literature, we review possible chemotherapeutic agents for treatment of primary intracranial leiomyosarcoma in patients refractory to surgical resection and radiation therapy.

Primary intracranial leiomyosarcoma: a case report and review of the literature

Malignant tumours of the central nervous system (CNS) represent the second most common cancer type in the paediatric population of developed countries, and mortality caused by CNS tumours is the highest among paediatric cancers. Tumours of mesenchymal origin occurring either as primary or secondary lesions of the central nervous system are very rare in children. This paper describes the case of a 5-year-old non-immunocompromised male who presented with a right frontal primary intracranial leiomyosarcoma associated with large subdural collections. Following surgical excision and adjuvant chemoradiotherapy, the patient has remained disease-free for 18 months. The literature is reviewed and the possible association of the chronic subdurals to the later development of the leiomyosarcoma explored.

Primary intracranial leiomyosarcoma associated with beta-HCG elevation in a 2 year-old child

Primary intracranial leiomyosarcoma associated with beta-HCG elevation in a 2 year-old child

Primary intracranial leiomyosarcoma associated with beta‐HCG elevation in a 2 year‐old child

Primary sarcomas, especially leiomyosarcomas (LMS), are rare intracranial tumors. Recently LMS have been described in immunocompromised patients, including HIV infection, in association with latent EBV infection. Herein, we describe a previously healthy, HIV seronegative, 2 year-old boy with a history of craniomegaly who presented with a right frontal scalp lesion. CT and MR imaging studies revealed apparently multiple intracranial enhancing solid and partially cystic masses with extracranial extension. No radiological abnormalities were detected in other organs. Screening of the serum revealed an elevated beta-HCG level of greater than 400 U/L, raising clinical suspicion for a germ cell tumor. Histological examination of the partially resected intracranial mass revealed highly cellular spindled and rhabdoid areas showing immunoreactivity to smooth muscle actin, vimentin, glial fibrillary acidic protein, and beta-HCG antibodies. No absence of the INI1 protein was detected. Cytogenetic studies revealed no chromosomal abnormalities. Ultrastructural findings included features typical of smooth muscle differentiation. The interpretation was that of a high-grade sarcoma, primarily LMS, with immunohistochemical support for divergent differentiation. The source of the serum beta-HCG elevation was attributed to the LMS, an association previously rarely reported in some sarcomas, including LMS.

Primary Intracranial Leiomyosarcoma: Report of a Case and Review of the Literature

A 26-year old man presented with a 3-month history of a progressively enlarging palpable parieto-occipital mass. A CT scan indicated the lesion arose from the dura with bony destruction. A stealth assisted craniotomy was performed with the provisional diagnosis of osteoblastic meningioma. Further histopathologic analysis of the intracranial mass was consistent with leiomyosarcoma. Staging evaluation, including CT and PET scans, demonstrated no other sites of disease. Despite complete surgical resection and radiotherapy to the resection site, the disease recurred locally and systematically 5 months later. Primary intracranial mesenchymal tumors are rare and few cases have been previously reported. Outcomes have been universally poor and current therapeutic approaches appear to have only limited benefit.

Primary Intracranial Leiomyosarcoma: A Case Report.

Primary Intracranial Leiomyosarcoma: A Case Report.