Surgical treatment of primary intracranial and extracranial communicating leiomyosarcoma: a case report

Surgical treatment of primary intracranial and extracranial communicating leiomyosarcoma: a case report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Surgical treatment of primary intracranial and extracranial communicating leiomyosarcoma: a case report Kuairong Pu, Tianhong Wang, Zhe Li, Xiwen Lin, Jun Wu, Dongchuan Shao, and 1 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-4458582/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract 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. Primary intracranial tumor Leiomyosarcoma Clinical features Surgical removal Case report Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Background Primary intracranial leiomyosarcoma, a rare smooth muscle sarcoma originating within the cranial cavity, accounts for a mere 0.1–0.2% of all intracranial tumors [ 1 ]. This condition can affect individuals of any age and gender, with clinical presentation varying based on tumor location and potentially encompassing symptoms such as memory loss, hemiparesis, and seizures [ 2 ]. Imaging studies typically reveal an irregular mass with heterogeneous enhancement on MRI, frequently originating from the dura mater but also potentially arising in the cerebrum [ 3 ]. Histologically, primary intracranial leiomyosarcoma exhibits similarities to soft tissue leiomyosarcoma, characterized by interwoven bundles of spindle-shaped cells, occasionally displaying a fence-like or perivascular epithelioid cell tumor-like arrangement [ 4 ]. The cells are densely packed and may demonstrate fibrosis and myxoid alterations. Larger tumors frequently exhibit regions of hyalinization and coagulative necrosis [ 5 ]. The precise etiology of primary intracranial leiomyosarcoma remains elusive, but associations with immunosuppression and viral infections have been reported [ 6 ]. Due to its rarity, this neoplasm is generally considered aggressive with a poor prognosis. Treatment strategies for primary intracranial leiomyosarcoma are typically determined based on the patient's age, tumor location, pathological characteristics, and the presence of distant metastases [ 7 ]. These may include surgical resection, radiotherapy, chemotherapy, and biological therapy, with surgical resection serving as the primary treatment modality [ 8 ]. Currently, there are only over 30 reports on surgical treatment. This case report discusses the clinical characteristics and treatment experience of a rare primary intracranial-extracranial communicating leiomyosarcoma. The patient underwent surgical resection in 2015, experienced a recurrence after 2 years and 4 months, and was treated with an expanded resection. As of 2023, the patient has shown no signs of recurrence, highlighting the efficacy of surgical intervention as the main treatment approach. Case presentation A 37-year-old married male patient was admitted to our hospital on May 10, 2015, with a subcutaneous mass on the left frontal vertex that had been present for 1 month and was gradually enlarging. Examination revealed a subcutaneous mass approximately 4 cm x 5 cm on the left frontal vertex. The mass was pliable, non-tender upon palpation, with a clear texture, well-defined borders, and no tenderness, elevated about 0.7 cm above the normal skin margin. CT indicated local lytic changes in the left frontal bone, with spindle-shaped hypo-to isodense shadows seen on the inner and outer aspects of the skull. The partial brain tissue was slightly compressed and shifted inward, with uneven density within and a CT value of about 38 HU. Uneven enhancement was observed at the edges. MRI revealed a spindle-shaped lesion with equal T1 and long T2 signals in the left frontal vertex bone, measuring approximately 2.2 cm x 3.5 cm x 4.0 cm, growing centered on the skull bone, with corresponding bone absorption and thinning. Bone destruction within presented as uneven low signals and abnormal uneven enhancement was noted at that location after contrast enhancement, with significant enhancement on delayed scanning. The adjacent brain parenchyma was slightly compressed and shifted inward (Fig. 1 ). During surgery, a mass in the left frontal area was observed to have a fish-flesh appearance, firm texture, and no significant adhesion to the scalp. An intraoperative frozen section examination was performed. After fully exposing the extracranial tumor, the skull was drilled, and the bone was bitten along the tumor. A circular incision was made in the dura mater. Due to the lack of adhesion between the tumor and the brain, the entire tumor was removed (Fig. 2 ). Following the intraoperative frozen section examination, which suggested a malignant tumor, the scope was expanded, and approximately 2 cm of surrounding tissue was removed. A range of about 8 cm x 8 cm of normal skull and dura mater was excised, and a phase-one artificial dura mater and titanium mesh repair were performed. The pathological diagnosis of leiomyosarcoma was made based on the presence of spindle-shaped and round-like irregularly arranged cells within the tumor, with abnormally enlarged nuclei that were deeply stained and significantly atypical. A few vacuolated cells were seen, with abundant acidophilic cytoplasm and visible mitosis, diffusely forming plaques and some interstitial mucoid degeneration. Immunohistochemistry results showed silver staining (+), B-cell lymphoma-2 (Bcl-2, +), CD34 (+), FLI-1 (+), FN (+), SMA (+), CD68 (scattered), and Ki-67 (30%). The specimen's margins were normal tissue (Fig. 3 ), which observed tumor cells arranged in irregular spindle shapes, with enlarged nuclei and some cells displaying a vacuolar appearance. The patient was followed up 9 days after discharge. As the tumor was completely excised and was not sensitive to radiotherapy and chemotherapy, no such treatments were administered. Routine follow-ups for 2 years and 3 months until August 2017 showed no recurrence, and PET-CT scans during this period did not reveal any recurrence or distant metastasis. At 2 years and 4 months postoperatively, a subcutaneous mass appeared on the left frontotemporal vertex, considered to be a recurrence of the leiomyosarcoma. The patient was admitted again on September 27, 2017, due to a lump found on the left frontotemporal vertex for 20 days. Examination revealed the original surgical scar on the left frontotemporal vertex and a subcutaneous mass approximately 3 cm in diameter, with a pliable texture, no tenderness upon touch, clear boundaries, no redness or skin ulceration, no noticeable movement, and elevated about 0.5 cm above the normal skin margin. No vascular murmur was heard within the mass. Enlarged lymph nodes were palpable on both sides of the neck, with the largest being about 3 cm in diameter. CT indicated an absence of bone substance in the left frontotemporal vertex, with a broad-based segment of low-density shadow visible, clear boundaries, measuring approximately 3.2 cm × 1.3 cm, and a CT value of about 39 HU. MRI showed a mass and post-surgical change in the left frontal vertex, with an irregular abnormal lesion seen in the inner table of the left parietal bone, presenting as equal T1 and slightly long T2 signals, measuring about 3.5 cm × 1.6 cm × 3.5 cm in size, with uneven enhancement after contrast, and significant mass effect (Fig. 4 ). During the surgery, a mass of approximately 3 cm × 4 cm was observed attached to the three-dimensional titanium mesh on the left frontal vertex, with no clear demarcation from the surrounding tissue. The mass was removed in sections, and after removing the titanium mesh, a mass with a diameter of 4 cm was seen on the left parietal vertex. The excised mass had a fish-flesh appearance and clear demarcation. The artificial dura mater, tumor tissue, and affected skull in that area were excised. The tumor originated from the dura mater at the vertex, and an additional 3 cm of skull, dura mater, periosteum, and subcutaneous tissue towards the vertex were removed. Towards the frontotemporal area, 4 cm away from the tumor, proliferative tissue, artificial dura mater, brain tissue, and skull were excised, along with a skin flap measuring 2 cm × 5 cm at the tumor site. The surgical procedure involved a phase-one repair with artificial dura mater and titanium mesh. The secondary pathological diagnosis still showed leiomyosarcoma (WHO Grade III). Within the tumor, spindle-shaped and round-like irregularly arranged cells were observed, with nuclei that were deeply stained and enlarged. HE showed significant pleomorphism, accompanied by a few vacuolated cells, and conspicuous mitotic figures diffusely distributed in sheets, with some interstitial mucoid degeneration (Fig. 5 ). Immunohistochemistry studies revealed silver immersion staining (+) and SMA (+). No tumors were found in the surrounding tissues, and neck lymph node needle aspiration cytology biopsy showed no tumor cells. The patient was discharged 10 days after surgery. Considering the recurrence of leiomyosarcoma without metastasis, no radiotherapy or chemotherapy was administered before or after the surgery. The patient regularly returned for check-ups after discharge, and as of June 1, 2020, had been followed up for over 2 years and 8 months, with no significant complications arising, and follow-up is ongoing. Discussion and conclusion Leiomyosarcoma is a rare malignant tumor arising from mesodermal tissues, commonly found in the uterus, gastrointestinal tract, and retroperitoneum. Head and neck leiomyosarcomas account for 1% to 4% of all leiomyosarcomas [9, 10]. Primary intracranial-extracranial communicating leiomyosarcomas are exceedingly rare, frequently originating from the meningeal interstitium and unrelated to meningeal epithelial cells [11]. These tumors can occur at any age, with no significant gender difference, and are typically seen in individuals with compromised immune function [12], especially in patients positive for HIV carrying the EB virus [13]. Intracranial sarcomas are mostly found supratentorially, very rarely in the cerebellum or spinal cord, with symptoms and signs varying depending on the tumor location. Extracranial leiomyosarcomas often present as subcutaneous masses, while intracranial leiomyosarcomas commonly cause symptoms such as headaches, vomiting, and secondary epilepsy. In this case, the patient presented with a subcutaneous mass without related intracranial symptoms. Currently, clinical diagnosis relies on the imaging characteristics of the tumor and the results of pathological immunohistochemistry. On CT, the appearance is usually that of a medium-sized soft tissue mass with clear boundaries, with a small part showing infiltrative growth and blurred boundaries; on MRI, the appearance is generally uneven signals, with some parts accompanied by cystic changes and necrosis, and the edges showing ring-like enhancement after contrast [13]. In this case, the sarcoma was clearly demarcated from the normal brain tissue, with only mild edema at the edges, uneven signals within, and obvious uneven enhancement after MRI contrast, with non-enhanced flaky areas visible inside. Pathological examination revealed that intracranial-extracranial communicating leiomyosarcomas, like soft tissue leiomyosarcomas at other locations, are mostly arranged in irregular spindle cell bundles, with some showing perivascular epithelioid cell tumor-like structures under the microscope, significant pleomorphism, elongated and variable nuclei, deep staining, easily visible mitotic figures, acidophilic or pale cytoplasm, and common vacuolated appearance. Immunohistochemistry is typically diffusely strongly positive for SMA, calponin, and desmin, with Ki-67 about 30% positive [14]. Primary intracranial-extracranial communicating leiomyosarcomas need to be differentiated from related intracranial and extracranial lesions, such as benign meningiomas, intracranial hemangiopericytomas, eosinophilic granulomas of the skull, osteosarcomas of the skull, gliosarcomas, and fibrosarcomas [15, 16]. Currently, the treatment of leiomyosarcoma still primarily involves surgical excision [17], removing the tumor visible to the naked eye, ensuring that the margins are pathologically negative intraoperatively, and then expanding the excision to include more than 2 cm of normal tissue [18]. Late-stage tumors have a higher degree of malignancy, with a short disease-free survival period, typically between 6 to 24 months [19], and surgery is difficult to achieve complete removal, so radiotherapy may be added before or after surgery [20]. Studies have shown that bevacizumab can effectively treat certain soft tissue sarcomas [21], but there is a lack of clinical data to support this for primary intracranial-extracranial communicating leiomyosarcomas. In this case, the patient had bone destruction, and the first expanded excision surgery was effective. After recurrence, two more surgeries were performed to further expand the excision range by 3 to 4 cm. Follow-up until June 1, 2020, showed no recurrence, and the patient has survived for 5 years since the first surgery, which indicated that surgical excision remains the main treatment method for this tumor. Currently, there is no uniform standard for the range of surgical excision, and it is recommended that even if intraoperative rapid pathological examination shows negative margins, the excision range should still be expanded by more than 2 cm. Conclusion The symptoms and signs of leiomyosarcoma vary depending on the tumor location and are mostly present with subcutaneous masses. Primary intracranial-extracranial communicating leiomyosarcoma needs to be differentiated from related intracranial and extracranial lesions to improve diagnostic accuracy. At present, the diagnosis of primary intracranial-extracranial leiomyosarcoma is mainly based on the imaging manifestations and pathological immunohistochemical results of the tumor in clinical practice. Furthermore, the treatment of leiomyosarcoma still relies mainly on surgical resection, which removes visible tumors and highlights the efficacy of surgical intervention as the main treatment method. Abbreviations MRI: Magnetic resonance imagingm; CT: Computed tomography; T 1 WI: T 1 -weighted imaging; T 2 WI: T 2 -weighted imaging; Bcl-2: B-cell lymphoma-2; SMA: Smooth muscle actin; HE: Haematoxylin and eosin; HIV: Human immunodeficiency virus; EB: Epstein-Barr Declarations Acknowledgements The authors thank the patient for his contributions. Author contributions K P wrote the manuscript. T W, Z L, and X L contributed to the acquisition of data and played a significant role in the analysis and interpretation of the results. Both authors of J W and D S performed the surgery and/or perioperative patient management. N Z supervised the work and reviewed the final manuscript. Funding This work was supported by the Yunnan Provincial Science and Technology Plan Project (202301AY070001-286), Yunnan Provincial Department of Education Scientific Research Fund Project (2024J0374), and Kunming Municipal Health Research Project (2023-04-04-004). Availability of data and materials There are no additional data to disclose. Ethics approval and consent to participate No applicable. Consent for publication Written informed consent was obtained from the patient for publication of the case report and accompanying images. Competing interests The authors declare that they have no competing interests. References Cohen N, Fedewa S, Chen AY. Epidemiology and demographics of the head and neck cancer population. Oral and Maxillofacial Surgery Clinics. 2018;30(4):381-95. Adelman MR. The Morcellation Debate: The History and the Science. Clinical obstetrics and gynecology. 2015;58(4):710-7. Zhao L, Jiang Y, Wang Y, Bai Y, Sun Y, Li Y. Primary Intracranial Leiomyosarcoma Secondary to Glioblastoma: Case Report and Literature Review. Frontiers in oncology. 2021;11:642683. Marko J, Wolfman DJ. Retroperitoneal Leiomyosarcoma From the Radiologic Pathology Archives. Radiographics : a review publication of the Radiological Society of North America, Inc. 2018;38(5):1403-20. Singh DK, Singh N, Parihar A, Singh R. Craniopharyngioma and epidermoid tumour in same child: a rare association. BMJ case reports. 2013;2013. Li XL, Ren J, Niu RN, Jiang X, Xu GH, Zhou P, et al. Primary intracranial leiomyosarcoma in an immunocompetent patient: Case report with emphasis on imaging features. Medicine (Baltimore). 2019;98(17):e15269. Canisius J, Wagner A, Bunk EC, Spille DC, Stögbauer L, Grauer O, et al. Expression of decitabine-targeted oncogenes in meningiomas in vivo. Neurosurgical review. 2022;45(4):2767-75. Zhu J, Wang H, Huang YQ, Song W, Li YF, Wang WJ, et al. Comprehensive analysis of a long non-coding RNA-associated competing endogenous RNA network in glioma. Oncology letters. 2020;20(4):63. Cohen N, Fedewa S, Chen AY. Epidemiology and Demographics of the Head and Neck Cancer Population. Oral and maxillofacial surgery clinics of North America. 2018;30(4):381-95. Workman AD, Farquhar DR, Brody RM, Parasher AK, Carey RM, Purkey MT, et al. Leiomyosarcoma of the head and neck: A 17-year single institution experience and review of the National Cancer Data Base. Head & neck. 2018;40(4):756-62. Rizzo A, Nigro MC, Ramponi V, Gallo C, Perrone AM, De Iaco P, et al. Skull Metastasis From Uterine Leiomyosarcoma, a Rare Presentation for a Rare Tumor: A Case Report and Review of the Literature. Frontiers in oncology. 2020;10:869. Gallagher SJ, Rosenberg SA, Francis D, Salamat S, Howard SP, Kimple RJ. Primary intracranial leiomyosarcoma in an immunocompetent patient: Case report and review of the literature. Clinical Neurology and Neurosurgery. 2018;165:76-80. Sivendran S, Vidal CI, Barginear MF. Primary intracranial leiomyosarcoma in an HIV-infected patient. International journal of clinical oncology. 2011;16(1):63-6. Gaetke-Udager K, McLean K, Sciallis AP, Alves T, Maturen KE, Mervak BM, et al. Diagnostic Accuracy of Ultrasound, Contrast-enhanced CT, and Conventional MRI for Differentiating Leiomyoma From Leiomyosarcoma. Academic radiology. 2016;23(10):1290-7. Wang N, Osswald M. Meningiomas: Overview and New Directions in Therapy. Seminars in neurology. 2018;38(1):112-20. Shankar JJS, Hodgson L, Sinha N. Diffusion weighted imaging may help differentiate intracranial hemangiopericytoma from meningioma. Journal of neuroradiology. 2019;46(4):263-7. Alshawwa K, Hijazi J, Jaber B, AlHassan H, Abu-Amer T, Salahaldeen R, et al. Resection of Primary Renal Leiomyosarcoma Involving the Inferior Vena Cava (IVC) with IVC Resection and Reconstruction. Case reports in surgery. 2022;2022:6037890. Kadouri Y, Lachkar S, Dergamoun H, El Sayegh H, Benslimane L, Nouini Y. Management of the Uncommon Bladder Cancers: A Single-Center Experience over 10 Years. Advances in urology. 2020;2020:7563703. Hussain S, Nanda A, Fowler M, Ampil FL, Burton GV. Primary intracranial leiomyosarcoma: report of a case and review of the literature. Sarcoma. 2006;2006:52140. Boșoteanu M, Vodă RI, Așchie M, Bosoteanu LA, Bălțătescu GI. Morphological and Ancillary Features of Uterine Leiomyosarcoma: Case Report. Clinical pathology (Thousand Oaks, Ventura County, Calif). 2022;15:2632010x221105224. Agulnik M, Yarber JL, Okuno SH, von Mehren M, Jovanovic BD, Brockstein BE, et al. An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas. Annals of oncology : official journal of the European Society for Medical Oncology. 2013;24(1):257-63. Additional Declarations No competing interests reported. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-4458582","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":310063462,"identity":"b0a000b9-0320-4ef9-bc78-d63e6f6c92fe","order_by":0,"name":"Kuairong Pu","email":"","orcid":"","institution":"Department of Neurosurgery, First People’s Hospital of Kunming","correspondingAuthor":false,"prefix":"","firstName":"Kuairong","middleName":"","lastName":"Pu","suffix":""},{"id":310063463,"identity":"8384d012-9ed4-4903-8289-71b1ed75fcf0","order_by":1,"name":"Tianhong Wang","email":"","orcid":"","institution":"Department of Jinning District People’s Hospital","correspondingAuthor":false,"prefix":"","firstName":"Tianhong","middleName":"","lastName":"Wang","suffix":""},{"id":310063464,"identity":"5efcedaa-6aac-4d91-af03-9863916cab42","order_by":2,"name":"Zhe Li","email":"","orcid":"","institution":"Department of Neurosurgery, First People’s Hospital of Kunming","correspondingAuthor":false,"prefix":"","firstName":"Zhe","middleName":"","lastName":"Li","suffix":""},{"id":310063465,"identity":"69d6723c-a73a-4b1b-933f-774908a70402","order_by":3,"name":"Xiwen Lin","email":"","orcid":"","institution":"Department of Neurosurgery, First People’s Hospital of Kunming","correspondingAuthor":false,"prefix":"","firstName":"Xiwen","middleName":"","lastName":"Lin","suffix":""},{"id":310063466,"identity":"142d879a-8c7f-443e-bda4-053fb70fc2e3","order_by":4,"name":"Jun Wu","email":"","orcid":"","institution":"Department of Neurosurgery, First People’s Hospital of Kunming","correspondingAuthor":false,"prefix":"","firstName":"Jun","middleName":"","lastName":"Wu","suffix":""},{"id":310063467,"identity":"213bc18e-0a7a-49b0-8b6b-79792d56f407","order_by":5,"name":"Dongchuan Shao","email":"","orcid":"","institution":"Department of Neurosurgery, First People’s Hospital of Kunming","correspondingAuthor":false,"prefix":"","firstName":"Dongchuan","middleName":"","lastName":"Shao","suffix":""},{"id":310063468,"identity":"89f0b77f-af99-412e-b90c-06f6b81a340c","order_by":6,"name":"Nan Zhao","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA4UlEQVRIiWNgGAWjYBACNvb+jw8YG8BsxgcMBkRo4eM5YGwA1cJsQJQWOYkEMwmoFjYJ4hwmkZBs+HOHnZw5+9ljlT8K7sgzsJ89gF8Lz4ODDyTPJBtb9uSl3eYxeGbYwJOXgF8Le2KzgWEbc+KGAzlmtxkMDjM2SPDg9xEbQzKbRGJbfeKG82/MCn8YHLYnrIUjjU3iYNvhxA03cswYeAwOJxLWwnOG2bCx7bixwY03xtJALcltPDn4tci39zA+/NlWLWdwPsfw448/h2372c8QEzuovhsFo2AUjIJRQDEAAGQERAfnxB+ZAAAAAElFTkSuQmCC","orcid":"","institution":"Department of Neurosurgery, First People’s Hospital of Kunming","correspondingAuthor":true,"prefix":"","firstName":"Nan","middleName":"","lastName":"Zhao","suffix":""}],"badges":[],"createdAt":"2024-05-22 06:18:49","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-4458582/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-4458582/v1","draftVersion":[],"editorialEvents":[],"editorialNote":"","failedWorkflow":false,"files":[{"id":58233203,"identity":"ca4c30c2-cc1d-4efd-a601-58e381f2897e","added_by":"auto","created_at":"2024-06-12 20:02:16","extension":"jpg","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":47249,"visible":true,"origin":"","legend":"\u003cp\u003eThe imaging findings before and after the first surgery. (\u003cstrong\u003ea\u003c/strong\u003e) Preoperative CT showed local cranial bone with moth-eaten changes and a spindle-shaped to hypodense shadow on the lateral side of the skull, with uneven density inside. (\u003cstrong\u003eb\u003c/strong\u003e) Preoperative MRI (T\u003csub\u003e1\u003c/sub\u003eWI) displays an uneven signal density. (\u003cstrong\u003ec\u003c/strong\u003e) Preoperative MRI (T\u003csub\u003e2\u003c/sub\u003eWI) appears as an isointense to hyperintense signal. (\u003cstrong\u003ed\u003c/strong\u003e) Preoperative MRI enhancement shows ring-like enhancement. (\u003cstrong\u003ee\u003c/strong\u003e) A one-week postoperative follow-up cranial CT indicates complete tumor resection.\u003c/p\u003e","description":"","filename":"1.jpg","url":"https://assets-eu.researchsquare.com/files/rs-4458582/v1/f1b2c90c43d00741cf047628.jpg"},{"id":58233200,"identity":"0ffc548c-3fb5-430c-933e-e361409ae4e4","added_by":"auto","created_at":"2024-06-12 20:02:16","extension":"jpg","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":53425,"visible":true,"origin":"","legend":"\u003cp\u003e(\u003cstrong\u003ea\u003c/strong\u003e) Leiomyosarcoma tissue excised during the first surgery. (\u003cstrong\u003eb\u003c/strong\u003e) Affected dural surface. (\u003cstrong\u003ec\u003c/strong\u003e) The inner surface of the skull.\u003c/p\u003e","description":"","filename":"2.jpg","url":"https://assets-eu.researchsquare.com/files/rs-4458582/v1/7fe077b683b3460fb429c825.jpg"},{"id":58233204,"identity":"39292f1a-03cd-43c0-a32b-638491d75e04","added_by":"auto","created_at":"2024-06-12 20:02:16","extension":"jpg","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":83824,"visible":true,"origin":"","legend":"\u003cp\u003eImmunohistochemical staining (original magnification, x 400). (\u003cstrong\u003ea\u003c/strong\u003e) SMA (+). (\u003cstrong\u003eb\u003c/strong\u003e) Ki-67 (+).\u003c/p\u003e","description":"","filename":"3.jpg","url":"https://assets-eu.researchsquare.com/files/rs-4458582/v1/e5fa52211a984b11f04effa6.jpg"},{"id":58233558,"identity":"90439003-2bd0-41e4-a47a-fa10e2a43a7a","added_by":"auto","created_at":"2024-06-12 20:10:16","extension":"jpg","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":48236,"visible":true,"origin":"","legend":"\u003cp\u003eThe imaging findings before and after the second surgery. (\u003cstrong\u003ea\u003c/strong\u003e)Preoperative CT reveals a low-density lesion with a wide base and clear boundary. (\u003cstrong\u003eb\u003c/strong\u003e) Preoperative MRI (T\u003csub\u003e1\u003c/sub\u003eWI) shows the soft tissue as a lower-density shadow. (\u003cstrong\u003ec\u003c/strong\u003e) Preoperative MRI (T\u003csub\u003e2\u003c/sub\u003eWI) presents as an isointense to hyperintense signal. (\u003cstrong\u003ed\u003c/strong\u003e) Preoperative MRI\u003csub\u003e \u003c/sub\u003eenhancement demonstrates uneven enhancement. (\u003cstrong\u003ee\u003c/strong\u003e) A one-week postoperative follow-up cranial CT indicates complete tumor resection.\u003c/p\u003e","description":"","filename":"4.jpg","url":"https://assets-eu.researchsquare.com/files/rs-4458582/v1/a9d77f46e536480fb5731d04.jpg"},{"id":58233559,"identity":"96f11f9c-e1ac-4663-a33a-122c6cd5ead8","added_by":"auto","created_at":"2024-06-12 20:10:16","extension":"jpg","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":131266,"visible":true,"origin":"","legend":"\u003cp\u003eThe tumor cells were under the microscope after the second surgery, with deeply stained\u003c/p\u003e","description":"","filename":"5.jpg","url":"https://assets-eu.researchsquare.com/files/rs-4458582/v1/fcbd569ceff19057f49a70f4.jpg"},{"id":63102062,"identity":"06972039-6a24-476e-94d7-d7fcd3fdf31a","added_by":"auto","created_at":"2024-08-23 07:06:41","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":668870,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-4458582/v1/ff5dcd5c-4610-48f0-b27d-9f55737ac9d8.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"Surgical treatment of primary intracranial and extracranial communicating leiomyosarcoma: a case report","fulltext":[{"header":"Background","content":"\u003cp\u003ePrimary intracranial leiomyosarcoma, a rare smooth muscle sarcoma originating within the cranial cavity, accounts for a mere 0.1\u0026ndash;0.2% of all intracranial tumors [\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e]. This condition can affect individuals of any age and gender, with clinical presentation varying based on tumor location and potentially encompassing symptoms such as memory loss, hemiparesis, and seizures [\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e]. Imaging studies typically reveal an irregular mass with heterogeneous enhancement on MRI, frequently originating from the dura mater but also potentially arising in the cerebrum [\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e].\u003c/p\u003e \u003cp\u003eHistologically, primary intracranial leiomyosarcoma exhibits similarities to soft tissue leiomyosarcoma, characterized by interwoven bundles of spindle-shaped cells, occasionally displaying a fence-like or perivascular epithelioid cell tumor-like arrangement [\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e]. The cells are densely packed and may demonstrate fibrosis and myxoid alterations. Larger tumors frequently exhibit regions of hyalinization and coagulative necrosis [\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e]. The precise etiology of primary intracranial leiomyosarcoma remains elusive, but associations with immunosuppression and viral infections have been reported [\u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e]. Due to its rarity, this neoplasm is generally considered aggressive with a poor prognosis.\u003c/p\u003e \u003cp\u003eTreatment strategies for primary intracranial leiomyosarcoma are typically determined based on the patient's age, tumor location, pathological characteristics, and the presence of distant metastases [\u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e]. These may include surgical resection, radiotherapy, chemotherapy, and biological therapy, with surgical resection serving as the primary treatment modality [\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e]. Currently, there are only over 30 reports on surgical treatment. This case report discusses the clinical characteristics and treatment experience of a rare primary intracranial-extracranial communicating leiomyosarcoma. The patient underwent surgical resection in 2015, experienced a recurrence after 2 years and 4 months, and was treated with an expanded resection. As of 2023, the patient has shown no signs of recurrence, highlighting the efficacy of surgical intervention as the main treatment approach.\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eA 37-year-old married male patient was admitted to our hospital on May 10, 2015, with a subcutaneous mass on the left frontal vertex that had been present for 1 month and was gradually enlarging. Examination revealed a subcutaneous mass approximately 4 cm x 5 cm on the left frontal vertex. The mass was pliable, non-tender upon palpation, with a clear texture, well-defined borders, and no tenderness, elevated about 0.7 cm above the normal skin margin.\u003c/p\u003e \u003cp\u003eCT indicated local lytic changes in the left frontal bone, with spindle-shaped hypo-to isodense shadows seen on the inner and outer aspects of the skull. The partial brain tissue was slightly compressed and shifted inward, with uneven density within and a CT value of about 38 HU. Uneven enhancement was observed at the edges.\u003c/p\u003e \u003cp\u003eMRI revealed a spindle-shaped lesion with equal T1 and long T2 signals in the left frontal vertex bone, measuring approximately 2.2 cm x 3.5 cm x 4.0 cm, growing centered on the skull bone, with corresponding bone absorption and thinning. Bone destruction within presented as uneven low signals and abnormal uneven enhancement was noted at that location after contrast enhancement, with significant enhancement on delayed scanning. The adjacent brain parenchyma was slightly compressed and shifted inward (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eDuring surgery, a mass in the left frontal area was observed to have a fish-flesh appearance, firm texture, and no significant adhesion to the scalp. An intraoperative frozen section examination was performed. After fully exposing the extracranial tumor, the skull was drilled, and the bone was bitten along the tumor. A circular incision was made in the dura mater. Due to the lack of adhesion between the tumor and the brain, the entire tumor was removed (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). Following the intraoperative frozen section examination, which suggested a malignant tumor, the scope was expanded, and approximately 2 cm of surrounding tissue was removed. A range of about 8 cm x 8 cm of normal skull and dura mater was excised, and a phase-one artificial dura mater and titanium mesh repair were performed.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eThe pathological diagnosis of leiomyosarcoma was made based on the presence of spindle-shaped and round-like irregularly arranged cells within the tumor, with abnormally enlarged nuclei that were deeply stained and significantly atypical. A few vacuolated cells were seen, with abundant acidophilic cytoplasm and visible mitosis, diffusely forming plaques and some interstitial mucoid degeneration.\u003c/p\u003e \u003cp\u003eImmunohistochemistry results showed silver staining (+), B-cell lymphoma-2 (Bcl-2, +), CD34 (+), FLI-1 (+), FN (+), SMA (+), CD68 (scattered), and Ki-67 (30%). The specimen's margins were normal tissue (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e), which observed tumor cells arranged in irregular spindle shapes, with enlarged nuclei and some cells displaying a vacuolar appearance.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eThe patient was followed up 9 days after discharge. As the tumor was completely excised and was not sensitive to radiotherapy and chemotherapy, no such treatments were administered. Routine follow-ups for 2 years and 3 months until August 2017 showed no recurrence, and PET-CT scans during this period did not reveal any recurrence or distant metastasis. At 2 years and 4 months postoperatively, a subcutaneous mass appeared on the left frontotemporal vertex, considered to be a recurrence of the leiomyosarcoma.\u003c/p\u003e \u003cp\u003eThe patient was admitted again on September 27, 2017, due to a lump found on the left frontotemporal vertex for 20 days. Examination revealed the original surgical scar on the left frontotemporal vertex and a subcutaneous mass approximately 3 cm in diameter, with a pliable texture, no tenderness upon touch, clear boundaries, no redness or skin ulceration, no noticeable movement, and elevated about 0.5 cm above the normal skin margin. No vascular murmur was heard within the mass. Enlarged lymph nodes were palpable on both sides of the neck, with the largest being about 3 cm in diameter.\u003c/p\u003e \u003cp\u003eCT indicated an absence of bone substance in the left frontotemporal vertex, with a broad-based segment of low-density shadow visible, clear boundaries, measuring approximately 3.2 cm \u0026times; 1.3 cm, and a CT value of about 39 HU. MRI showed a mass and post-surgical change in the left frontal vertex, with an irregular abnormal lesion seen in the inner table of the left parietal bone, presenting as equal T1 and slightly long T2 signals, measuring about 3.5 cm \u0026times; 1.6 cm \u0026times; 3.5 cm in size, with uneven enhancement after contrast, and significant mass effect (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eDuring the surgery, a mass of approximately 3 cm \u0026times; 4 cm was observed attached to the three-dimensional titanium mesh on the left frontal vertex, with no clear demarcation from the surrounding tissue. The mass was removed in sections, and after removing the titanium mesh, a mass with a diameter of 4 cm was seen on the left parietal vertex. The excised mass had a fish-flesh appearance and clear demarcation. The artificial dura mater, tumor tissue, and affected skull in that area were excised. The tumor originated from the dura mater at the vertex, and an additional 3 cm of skull, dura mater, periosteum, and subcutaneous tissue towards the vertex were removed. Towards the frontotemporal area, 4 cm away from the tumor, proliferative tissue, artificial dura mater, brain tissue, and skull were excised, along with a skin flap measuring 2 cm \u0026times; 5 cm at the tumor site. The surgical procedure involved a phase-one repair with artificial dura mater and titanium mesh.\u003c/p\u003e \u003cp\u003eThe secondary pathological diagnosis still showed leiomyosarcoma (WHO Grade III). Within the tumor, spindle-shaped and round-like irregularly arranged cells were observed, with nuclei that were deeply stained and enlarged. HE showed significant pleomorphism, accompanied by a few vacuolated cells, and conspicuous mitotic figures diffusely distributed in sheets, with some interstitial mucoid degeneration (Fig.\u0026nbsp;\u003cspan refid=\"Fig5\" class=\"InternalRef\"\u003e5\u003c/span\u003e). Immunohistochemistry studies revealed silver immersion staining (+) and SMA (+). No tumors were found in the surrounding tissues, and neck lymph node needle aspiration cytology biopsy showed no tumor cells.\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003eThe patient was discharged 10 days after surgery. Considering the recurrence of leiomyosarcoma without metastasis, no radiotherapy or chemotherapy was administered before or after the surgery. The patient regularly returned for check-ups after discharge, and as of June 1, 2020, had been followed up for over 2 years and 8 months, with no significant complications arising, and follow-up is ongoing.\u003c/p\u003e"},{"header":"Discussion and conclusion","content":"\u003cp\u003eLeiomyosarcoma is a rare malignant tumor arising from mesodermal tissues, commonly found in the uterus, gastrointestinal tract, and retroperitoneum. Head and neck leiomyosarcomas account for 1% to 4% of all leiomyosarcomas [9, 10]. Primary intracranial-extracranial communicating leiomyosarcomas are exceedingly rare, frequently originating from the meningeal interstitium and unrelated to meningeal epithelial cells [11]. These tumors can occur at any age, with no significant gender difference, and are typically seen in individuals with compromised immune function [12], especially in patients positive for HIV carrying the EB virus [13]. Intracranial sarcomas are mostly found supratentorially, very rarely in the cerebellum or spinal cord, with symptoms and signs varying depending on the tumor location. Extracranial leiomyosarcomas often present as subcutaneous masses, while intracranial leiomyosarcomas commonly cause symptoms such as headaches, vomiting, and secondary epilepsy. In this case, the patient presented with a subcutaneous mass without related intracranial symptoms.\u003c/p\u003e\n\u003cp\u003eCurrently, clinical diagnosis relies on the imaging characteristics of the tumor and the results of pathological immunohistochemistry. On CT, the appearance is usually that of a medium-sized soft tissue mass with clear boundaries, with a small part showing infiltrative growth and blurred boundaries; on MRI, the appearance is generally uneven signals, with some parts accompanied by cystic changes and necrosis, and the edges showing ring-like enhancement after contrast\u0026nbsp;[13]. In this case, the sarcoma was clearly demarcated from the normal brain tissue, with only mild edema at the edges, uneven signals within, and obvious uneven enhancement after MRI contrast, with non-enhanced flaky areas visible inside. Pathological examination revealed that intracranial-extracranial communicating leiomyosarcomas, like soft tissue leiomyosarcomas at other locations, are mostly arranged in irregular spindle cell bundles, with some showing perivascular epithelioid cell tumor-like structures under the microscope, significant pleomorphism, elongated and variable nuclei, deep staining, easily visible mitotic figures, acidophilic or pale cytoplasm, and common vacuolated appearance. Immunohistochemistry is typically diffusely strongly positive for SMA, calponin, and desmin, with Ki-67 about 30% positive\u0026nbsp;[14].\u0026nbsp;\u003c/p\u003e\n\u003cp\u003ePrimary intracranial-extracranial communicating leiomyosarcomas need to be differentiated from related intracranial and extracranial lesions, such as benign meningiomas, intracranial hemangiopericytomas, eosinophilic granulomas of the skull, osteosarcomas of the skull, gliosarcomas, and fibrosarcomas\u0026nbsp;[15, 16].\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eCurrently, the treatment of leiomyosarcoma still primarily involves surgical excision [17], removing the tumor visible to the naked eye, ensuring that the margins are pathologically negative intraoperatively, and then expanding the excision to include more than 2 cm of normal tissue [18]. Late-stage tumors have a higher degree of malignancy, with a short disease-free survival period, typically between 6 to 24 months [19], and surgery is difficult to achieve complete removal, so radiotherapy may be added before or after surgery [20]. Studies have shown that bevacizumab can effectively treat certain soft tissue sarcomas [21], but there is a lack of clinical data to support this for primary intracranial-extracranial communicating leiomyosarcomas. In this case, the patient had bone destruction, and the first expanded excision surgery was effective. After recurrence, two more surgeries were performed to further expand the excision range by 3 to 4 cm. Follow-up until June 1, 2020, showed no recurrence, and the patient has survived for 5 years since the first surgery, which indicated that surgical excision remains the main treatment method for this tumor. Currently, there is no uniform standard for the range of surgical excision, and it is recommended that even if intraoperative rapid pathological examination shows negative margins, the excision range should still be expanded by more than 2 cm.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003eThe symptoms and signs of leiomyosarcoma vary depending on the tumor location and are mostly present with subcutaneous masses. Primary intracranial-extracranial communicating leiomyosarcoma needs to be differentiated from related intracranial and extracranial lesions to improve diagnostic accuracy. At present, the diagnosis of primary intracranial-extracranial leiomyosarcoma is mainly based on the imaging manifestations and pathological immunohistochemical results of the tumor in clinical practice. Furthermore, the treatment of leiomyosarcoma still relies mainly on surgical resection, which removes visible tumors and highlights the efficacy of surgical intervention as the main treatment method.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cp\u003eMRI: Magnetic resonance imagingm; CT:\u0026nbsp;Computed tomography;\u0026nbsp;T\u003csub\u003e1\u003c/sub\u003eWI: T\u003csub\u003e1\u003c/sub\u003e-weighted imaging; T\u003csub\u003e2\u003c/sub\u003eWI: T\u003csub\u003e2\u003c/sub\u003e-weighted imaging; Bcl-2: B-cell lymphoma-2; SMA: Smooth muscle actin; HE: Haematoxylin and eosin; HIV: Human immunodeficiency virus; EB: Epstein-Barr\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003e\u003cstrong\u003eAcknowledgements\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors thank the patient for his contributions.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAuthor contributions\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eK P wrote the manuscript. T W, Z L, and X L contributed to the acquisition of data and played a significant role in the analysis and interpretation of the results. Both authors of J W and D S performed the surgery and/or perioperative patient management. N Z supervised the work and reviewed the final manuscript.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis work was supported by the Yunnan Provincial Science and Technology Plan Project (202301AY070001-286), Yunnan Provincial Department of Education Scientific Research Fund Project (2024J0374), and Kunming Municipal Health Research Project (2023-04-04-004).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eAvailability of data and materials\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThere are no additional data to disclose.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics approval and consent to participate\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNo applicable.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent was obtained from the patient for publication of the case report and accompanying images.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCompeting interests\u0026nbsp;\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no competing interests.\u0026nbsp;\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eCohen N, Fedewa S, Chen AY. Epidemiology and demographics of the head and neck cancer population. Oral and Maxillofacial Surgery Clinics. 2018;30(4):381-95.\u003c/li\u003e\n\u003cli\u003eAdelman MR. The Morcellation Debate: The History and the Science. Clinical obstetrics and gynecology. 2015;58(4):710-7.\u003c/li\u003e\n\u003cli\u003eZhao L, Jiang Y, Wang Y, Bai Y, Sun Y, Li Y. Primary Intracranial Leiomyosarcoma Secondary to Glioblastoma: Case Report and Literature Review. Frontiers in oncology. 2021;11:642683.\u003c/li\u003e\n\u003cli\u003eMarko J, Wolfman DJ. Retroperitoneal Leiomyosarcoma From the Radiologic Pathology Archives. Radiographics : a review publication of the Radiological Society of North America, Inc. 2018;38(5):1403-20.\u003c/li\u003e\n\u003cli\u003eSingh DK, Singh N, Parihar A, Singh R. Craniopharyngioma and epidermoid tumour in same child: a rare association. BMJ case reports. 2013;2013.\u003c/li\u003e\n\u003cli\u003eLi XL, Ren J, Niu RN, Jiang X, Xu GH, Zhou P, et al. Primary intracranial leiomyosarcoma in an immunocompetent patient: Case report with emphasis on imaging features. Medicine (Baltimore). 2019;98(17):e15269.\u003c/li\u003e\n\u003cli\u003eCanisius J, Wagner A, Bunk EC, Spille DC, St\u0026ouml;gbauer L, Grauer O, et al. Expression of decitabine-targeted oncogenes in meningiomas in vivo. Neurosurgical review. 2022;45(4):2767-75.\u003c/li\u003e\n\u003cli\u003eZhu J, Wang H, Huang YQ, Song W, Li YF, Wang WJ, et al. Comprehensive analysis of a long non-coding RNA-associated competing endogenous RNA network in glioma. Oncology letters. 2020;20(4):63.\u003c/li\u003e\n\u003cli\u003eCohen N, Fedewa S, Chen AY. Epidemiology and Demographics of the Head and Neck Cancer Population. Oral and maxillofacial surgery clinics of North America. 2018;30(4):381-95.\u003c/li\u003e\n\u003cli\u003eWorkman AD, Farquhar DR, Brody RM, Parasher AK, Carey RM, Purkey MT, et al. Leiomyosarcoma of the head and neck: A 17-year single institution experience and review of the National Cancer Data Base. Head \u0026amp; neck. 2018;40(4):756-62.\u003c/li\u003e\n\u003cli\u003eRizzo A, Nigro MC, Ramponi V, Gallo C, Perrone AM, De Iaco P, et al. Skull Metastasis From Uterine Leiomyosarcoma, a Rare Presentation for a Rare Tumor: A Case Report and Review of the Literature. Frontiers in oncology. 2020;10:869.\u003c/li\u003e\n\u003cli\u003eGallagher SJ, Rosenberg SA, Francis D, Salamat S, Howard SP, Kimple RJ. Primary intracranial leiomyosarcoma in an immunocompetent patient: Case report and review of the literature. Clinical Neurology and Neurosurgery. 2018;165:76-80.\u003c/li\u003e\n\u003cli\u003eSivendran S, Vidal CI, Barginear MF. Primary intracranial leiomyosarcoma in an HIV-infected patient. International journal of clinical oncology. 2011;16(1):63-6.\u003c/li\u003e\n\u003cli\u003eGaetke-Udager K, McLean K, Sciallis AP, Alves T, Maturen KE, Mervak BM, et al. Diagnostic Accuracy of Ultrasound, Contrast-enhanced CT, and Conventional MRI for Differentiating Leiomyoma From Leiomyosarcoma. Academic radiology. 2016;23(10):1290-7.\u003c/li\u003e\n\u003cli\u003eWang N, Osswald M. Meningiomas: Overview and New Directions in Therapy. Seminars in neurology. 2018;38(1):112-20.\u003c/li\u003e\n\u003cli\u003eShankar JJS, Hodgson L, Sinha N. Diffusion weighted imaging may help differentiate intracranial hemangiopericytoma from meningioma. Journal of neuroradiology. 2019;46(4):263-7.\u003c/li\u003e\n\u003cli\u003eAlshawwa K, Hijazi J, Jaber B, AlHassan H, Abu-Amer T, Salahaldeen R, et al. Resection of Primary Renal Leiomyosarcoma Involving the Inferior Vena Cava (IVC) with IVC Resection and Reconstruction. Case reports in surgery. 2022;2022:6037890.\u003c/li\u003e\n\u003cli\u003eKadouri Y, Lachkar S, Dergamoun H, El Sayegh H, Benslimane L, Nouini Y. Management of the Uncommon Bladder Cancers: A Single-Center Experience over 10 Years. Advances in urology. 2020;2020:7563703.\u003c/li\u003e\n\u003cli\u003eHussain S, Nanda A, Fowler M, Ampil FL, Burton GV. Primary intracranial leiomyosarcoma: report of a case and review of the literature. Sarcoma. 2006;2006:52140.\u003c/li\u003e\n\u003cli\u003eBoșoteanu M, Vodă RI, Așchie M, Bosoteanu LA, Bălțătescu GI. Morphological and Ancillary Features of Uterine Leiomyosarcoma: Case Report. Clinical pathology (Thousand Oaks, Ventura County, Calif). 2022;15:2632010x221105224.\u003c/li\u003e\n\u003cli\u003eAgulnik M, Yarber JL, Okuno SH, von Mehren M, Jovanovic BD, Brockstein BE, et al. An open-label, multicenter, phase II study of bevacizumab for the treatment of angiosarcoma and epithelioid hemangioendotheliomas. Annals of oncology : official journal of the European Society for Medical Oncology. 2013;24(1):257-63.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"Primary intracranial tumor, Leiomyosarcoma, Clinical features, Surgical removal, Case report","lastPublishedDoi":"10.21203/rs.3.rs-4458582/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-4458582/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003cp\u003e\u003cstrong\u003eBackground: \u003c/strong\u003ePrimary intracranial-extracranial communicating leiomyosarcomas, capable of invading both the intracranial and extracranial regions and involving complex anatomical structures, are exceedingly rare neoplasms.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eCase presentation:\u003c/strong\u003e 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.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConclusion: \u003c/strong\u003ePrimary 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.\u003c/p\u003e","manuscriptTitle":"Surgical treatment of primary intracranial and extracranial communicating leiomyosarcoma: a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-06-12 20:02:11","doi":"10.21203/rs.3.rs-4458582/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"84cb67d0-3182-4879-aec6-1a3893ea1135","owner":[],"postedDate":"June 12th, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-08-23T07:06:27+00:00","versionOfRecord":[],"versionCreatedAt":"2024-06-12 20:02:11","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-4458582","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-4458582","identity":"rs-4458582","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}