{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,2]],"date-time":"2025-11-02T11:03:07Z","timestamp":1762081387718,"version":"build-2065373602"},"reference-count":57,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2022,12,21]],"date-time":"2022-12-21T00:00:00Z","timestamp":1671580800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Maria Sklodowska-Curie National Research Institute of Oncology in Warsaw"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Leiomyomas are common lesions that are usually located in the genitourinary and gastrointestinal tracts. Primary leiomyomas at the skull base are uncommon. They are composed of well-differentiated smooth muscle cells without cellular atypia. The diagnosis of a leiomyoma has to be confirmed by immunohistochemistry. The tumor tissue is immunoreactive for SMA, S100 and cytokeratin. Leiomyomas mainly occur in immunocompromised patients. Most tumor tissues are positive for EBV. The presented case is that of a 56-year-old immunocompetent woman with a tumor on the clivus. The radiological images suggested chordoma or fibrous dysplasia. Transnasal transsphenoidal surgery was performed. The tumor tissue consisted of well-differentiated smooth muscle cells with elongated nuclei. Immunohistochemistry revealed a positive reaction for desmin, SMA and h-Caldesmon and a negative reaction for S100, beta-catenin, PGR and Ki67. The leiomyoma diagnosis was subsequently established. To the best of our knowledge, the case of a primary leiomyoma on the clivus of an immunocompetent patient is the first to be described. We also extensively reviewed the literature on the immunohistopathological and radiological differential diagnosis of clival lesions.<\/jats:p>","DOI":"10.3390\/diagnostics13010009","type":"journal-article","created":{"date-parts":[[2022,12,21]],"date-time":"2022-12-21T05:42:53Z","timestamp":1671601373000},"page":"9","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["A Rare Case of a Primary Leiomyoma of the Clivus in an Immunocompetent Patient and a Review of the Literature Regarding Clival Lesions"],"prefix":"10.3390","volume":"13","author":[{"given":"Jacek","family":"Kunicki","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9228-3014","authenticated-orcid":false,"given":"Natalia","family":"Rzewuska","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland"},{"name":"Department of Gynecological Endocrinology, Medical University of Warsaw, 00-315 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Micha\u0142","family":"Kunicki","sequence":"additional","affiliation":[{"name":"INVICTA Fertility and Reproductive Center, 00-019 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Piotr","family":"Wi\u015bniewski","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Diagnostics, Maria Sklodowska-Curie National Research Institute of Oncology, 02-781 Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,12,21]]},"reference":[{"key":"ref_1","unstructured":"Perry, A., and Brat, D.J. (2018). 14-Mesenchymal Tumors of the Central Nervous System. Practical Surgical Neuropathology: A Diagnostic Approach, Elsevier. [2nd ed.]."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1016\/j.jocn.2012.07.001","article-title":"Two patients with primary sellar leiomyomas, a rare entity","volume":"20","author":"Ko","year":"2013","journal-title":"J. Clin. Neurosci."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Colagrande, A., Cazzato, G., Fedele, S., Andriola, V., Ingravallo, G., Resta, L., and Vincenti, L. (2022). A Unique Case of the Transformation of a Hepatic Leiomyoma into Leiomyosarcoma with Pancreatic Metastases: Review of the Literature with Case Presentation. Reports, 5.","DOI":"10.3390\/reports5010002"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1002\/ijgo.13102","article-title":"Epidemiology and management of uterine fibroids","volume":"149","author":"Giuliani","year":"2020","journal-title":"Int. J. Gynaecol. Obstet."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1037","DOI":"10.1289\/ehp.5787","article-title":"Etiology and pathogenesis of uterine leiomyomas: A review","volume":"111","author":"Flake","year":"2003","journal-title":"Environ. Health Perspect."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1055\/s-0037-1607264","article-title":"Uterine fibroids: Burden and unmet medical need","volume":"35","author":"Myers","year":"2017","journal-title":"Semin. Reprod. Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1093\/humupd\/7.1.8","article-title":"Leiomyomata: Heritability and cytogenetic studies","volume":"7","author":"Ligon","year":"2001","journal-title":"Hum. Reprod. Update."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1","DOI":"10.4172\/2329-6895.1000102","article-title":"Primary Intracranial Leiomyoma in Immunocompetent Patients: Case Report, Review of Literature, and Treatment Recommendations","volume":"1","author":"Fornoff","year":"2013","journal-title":"J. Neurol. Disord."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1016\/j.wneu.2020.05.157","article-title":"Intracranial Leiomyoma Associated with Epstein-Barr Virus: A cerebellopontine angle mass presenting with trigeminal neuralgia","volume":"141","author":"Sadeh","year":"2020","journal-title":"World Neurosurg."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"e13","DOI":"10.1016\/j.wneu.2016.01.088","article-title":"Primary Intraventricular Leiomyoma in an Immunocompetent Patient: First Case Report and Review of the Literature","volume":"90","author":"Garces","year":"2016","journal-title":"World Neurosurg."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"189","DOI":"10.3171\/jns.1968.29.2.0189","article-title":"Primary intrasellar leiomyoma. Case report","volume":"29","author":"Kroe","year":"1968","journal-title":"J. Neurosurg."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"72","DOI":"10.4103\/ijri.IJRI_250_18","article-title":"Primary intracranial leiomyoma\u2014A case report and literature review","volume":"29","author":"Jehendran","year":"2019","journal-title":"Indian J. Radiol. Imaging."},{"key":"ref_13","first-page":"679","article-title":"Primary intracranial intraventricular leiomyoma: A literature review","volume":"44","author":"Yamaner","year":"2020","journal-title":"Neurosurg. Rev."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"283","DOI":"10.3171\/2018.3.PEDS17609","article-title":"Synchronous Epstein-Barr virus-associated skull base and adrenal smooth-muscle tumors in an 8-year-old girl with recent Epstein-Barr virus infection","volume":"22","author":"Brown","year":"2018","journal-title":"J. Neurosurg. Pediatr."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"701","DOI":"10.3171\/jns.2000.93.4.0701","article-title":"Multicentric intracranial smooth-muscle tumor in a woman with human immunodeficiency virus. Case report","volume":"93","author":"Citow","year":"2000","journal-title":"J. Neurosurg."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"3923","DOI":"10.1007\/s00381-021-05173-0","article-title":"Primary intracranial smooth muscle tumor associated with Epstein-Barr virus in immunosuppressed children: Two cases report and review of literature","volume":"37","author":"Andaur","year":"2021","journal-title":"Childs Nerv. Syst."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"907","DOI":"10.1016\/j.clineuro.2009.08.016","article-title":"Primary intracranial leiomyomas: Report of two cases and review of the literature","volume":"111","author":"Hua","year":"2009","journal-title":"Clin. Neurol. Neurosurg."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1007\/s00590-011-0874-y","article-title":"Intraosseous leiomyoma of the distal femur: A case report and review of literatures","volume":"22","author":"Sung","year":"2012","journal-title":"Eur. J. Orthop. Surg. Traumatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1025","DOI":"10.1016\/j.humpath.2005.07.004","article-title":"Desmoplastic fibroma of bone: An immunohistochemical study including beta-catenin expression and mutational analysis for beta-catenin","volume":"36","author":"Hauben","year":"2005","journal-title":"Hum. Pathol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1093\/ajcp\/aqw159.011","article-title":"Spindle Cell Hemangioma of the Bone: A Case Report and Review of Literature","volume":"146","author":"Hotchandani","year":"2016","journal-title":"Am. J. Clin. Pathol."},{"key":"ref_21","first-page":"ZD19","article-title":"Rare case of spindle cell haemangioma","volume":"9","author":"Chavva","year":"2015","journal-title":"J. Clin. Diagn. Res."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"281","DOI":"10.3171\/2019.3.JNS182968","article-title":"Primary intraosseous meningioma: Clinical, histological, and differential diagnostic aspects","volume":"133","author":"Butscheidt","year":"2019","journal-title":"J. Neurosurg."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"e15737","DOI":"10.1097\/MD.0000000000015737","article-title":"Solitary fibrous tumor of the pineal region with delayed ectopic intracranial metastasis: A case report and review of the literature","volume":"98","author":"Wang","year":"2019","journal-title":"Medicine"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"127","DOI":"10.14791\/btrt.2015.3.2.127","article-title":"Solitary Fibrous Tumor of Central Nervous System: A Case Report","volume":"3","author":"Kim","year":"2015","journal-title":"Brain Tumor Res. Treat."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1007\/s10143-013-0463-0","article-title":"Solitary nonchordomatous lesions of the clival bone: Differential diagnosis and current therapeutic strategies","volume":"36","author":"Gagliardi","year":"2013","journal-title":"Neurosurg. Rev."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"S42","DOI":"10.14639\/0392-100X-suppl.1-41-2021-04","article-title":"Clivus pathologies from diagnosis to surgical multidisciplinary treatment. Review of the literature","volume":"41","author":"Pagella","year":"2021","journal-title":"Acta. Otorhinolaryngol. Ital."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1007\/s00701-009-0229-1","article-title":"Clivus metastases: Report of seven patients and literature review","volume":"151","author":"Pallini","year":"2009","journal-title":"Acta Neurochir."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1016\/S0303-8467(03)00002-7","article-title":"Benign metastasizing leiomyoma to skull base and spine: A report of two cases","volume":"105","author":"Alessi","year":"2003","journal-title":"Clin. Neurol. Neurosurg."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1007\/s10143-013-0503-9","article-title":"Endoscopic endonasal approach in the management of skull base chordomas\u2014Clinical experience on a large series, technique, outcome, and pitfalls","volume":"37","author":"Chibbaro","year":"2013","journal-title":"Neurosurg. Rev."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"1835","DOI":"10.1007\/s00701-016-3032-9","article-title":"Surgical resection of skull-base chordomas: Experience in case selection for surgical approach according to anatomical compartments and review of the literature","volume":"159","author":"Shimony","year":"2017","journal-title":"Acta Neurochir."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1007\/s40134-018-0275-7","article-title":"State-of-the-Art Imaging in Human Chordoma of the Skull Base","volume":"6","author":"Santegoeds","year":"2018","journal-title":"Curr. Radiol. Rep."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1295","DOI":"10.1016\/j.crad.2014.07.010","article-title":"Benign and malignant diseases of the clivus","volume":"69","author":"Neelakantan","year":"2014","journal-title":"Clin. Radiol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/j.nec.2015.03.008","article-title":"The Endoscopic Endonasal Approach for Removal of Petroclival Chondrosarcomas","volume":"26","author":"Prevedello","year":"2015","journal-title":"Neurosurg. Clin. N. Am."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"803","DOI":"10.3389\/fendo.2019.00803","article-title":"Co-administration of Systemic and Intralesional Zoledronic Acid in a Case of Fibrous Dysplasia: A Potentially Novel Therapy","volume":"10","author":"Bhadada","year":"2019","journal-title":"Front. Endocrinol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"1234","DOI":"10.1016\/j.humpath.2011.09.012","article-title":"The diagnostic utility of the GNAS mutation in patients with fibrous dysplasia: Meta-analysis of 168 sporadic cases","volume":"43","author":"Lee","year":"2012","journal-title":"Hum. Pathol."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"omz039","DOI":"10.1093\/omcr\/omz039","article-title":"Fibrous dysplasia of occipital and temporal bone. A case report","volume":"5","author":"Neromyliotis","year":"2019","journal-title":"Oxf. Med. Case Rep."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"E4","DOI":"10.1177\/014556131409301202","article-title":"Clival Fibrous Dysplasia: Case Series and Review of the Literature","volume":"93","author":"Boyer","year":"2014","journal-title":"Ear Nose Throat J."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1227\/01.NEU.0000043694.77162.6E","article-title":"Fibrous dysplasia of the clivus","volume":"52","author":"Adadam","year":"2003","journal-title":"Neurosurgery"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1016\/j.radcr.2020.11.019","article-title":"Fibrous dysplasia of the clivus-A case study and literature review","volume":"16","author":"Butt","year":"2020","journal-title":"Radiol. Case Rep."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"190","DOI":"10.1016\/j.wneu.2021.11.105","article-title":"Metastatic Lesions of the Clivus: A Systematic Review","volume":"158","author":"Jozsa","year":"2020","journal-title":"World Neurosurg."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"1180","DOI":"10.1016\/j.clineuro.2012.10.028","article-title":"Epidural intracranial metastasis from benign leiomyoma: A case report with literature review","volume":"115","author":"Joo","year":"2013","journal-title":"Clin. Neurol. Neurosurg."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"509","DOI":"10.5468\/ogs.2018.61.4.509","article-title":"Aberrant uterine leiomyomas with extrauterine manifestation: Intravenous leiomyomatosis and benign metastasizing leiomyomas","volume":"61","author":"Kim","year":"2018","journal-title":"Obstet. Gynecol. Sci."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"343","DOI":"10.1016\/j.ccm.2004.01.014","article-title":"Benign metastasizing leiomyoma and lymphangioleiomyomatosis: Sex-specific diseases?","volume":"25","author":"Pitts","year":"2004","journal-title":"Clin. Chest Med."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"2409","DOI":"10.2217\/fon-2021-0316","article-title":"Solitary plasmacytoma of jaw bone: A viewpoint on treatment modalities","volume":"17","author":"Choudhari","year":"2021","journal-title":"Future Oncol."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"573","DOI":"10.4103\/1793-5482.148788","article-title":"Plasmacytoma of the clivus","volume":"12","author":"Amita","year":"2017","journal-title":"Asian J. Neurosurg."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"418","DOI":"10.3171\/2017.9.JNS171057","article-title":"Solitary fibrous tumor\/hemangiopericytoma: Treatment results based on the 2016 WHO classification","volume":"130","author":"Sung","year":"2019","journal-title":"J. Neurosurg."},{"key":"ref_47","unstructured":"Louis, D.N., Ohgaki, H., Wiestler, O.D., and Cavenee, W.K. (2016). Mesenchymal, non-meningothelial tumours. WHO Classification of Tumours of the Central Nervous System, IARC. [4th ed.]."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1097\/COC.0000000000000146","article-title":"CNS Hemangiopericytoma: A Systematic Review of 523 Patients","volume":"40","author":"Ghose","year":"2017","journal-title":"Am. J. Clin. Oncol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1578","DOI":"10.1016\/j.jocn.2009.02.039","article-title":"Solitary fibrous tumor","volume":"16","author":"Yilmaz","year":"2009","journal-title":"J. Clin. Neurosci."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"414","DOI":"10.1016\/j.wneu.2016.02.103","article-title":"Prognostic Factors in Patients with Primary Hemangiopericytomas of the Central Nervous System: A Series of 103 Cases at a Single Institution","volume":"90","author":"Zhu","year":"2016","journal-title":"World Neurosurg."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1007\/s11060-018-2746-3","article-title":"Efficacy of adjuvant radiotherapy in the intracranial hemangiopericytoma","volume":"137","author":"Jeon","year":"2018","journal-title":"J. Neuro-Oncol."},{"key":"ref_52","first-page":"60","article-title":"Primary Lymphomas of the Skull Base from a Neurosurgical Perspective: Review of the Literature and Personal Experience","volume":"78","author":"Pesce","year":"2017","journal-title":"J. Neurol. Surg. A Cent. Eur. Neurosurg."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1007\/s00701-011-1124-0","article-title":"Primary lymphoma of the cranial vault: Case report and a systematic review of the literature","volume":"154","author":"Akhaddar","year":"2012","journal-title":"Acta Neurochir."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1007\/s00256-018-3085-3","article-title":"CT, conventional, and functional MRI features of skull lymphoma: A series of eight cases in a single institution","volume":"48","author":"Xing","year":"2019","journal-title":"Skelet. Radiol."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"e755","DOI":"10.1097\/SCS.0000000000005843","article-title":"Clivus Intraosseous Meningioma Mimicking Chordoma","volume":"30","author":"Abuzayed","year":"2019","journal-title":"J. Craniofacial Surg."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.nec.2015.11.011","article-title":"Primary Intraosseous Meningioma","volume":"27","author":"Chen","year":"2016","journal-title":"Neurosurg. Clin. N. Am."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"124","DOI":"10.1159\/000114536","article-title":"Endoscopic endonasal surgery for clival chordoma and chondrosarcoma","volume":"70","author":"Zhang","year":"2008","journal-title":"ORL J. Otorhinolaryngol. Relat. Spec."}],"container-title":["Diagnostics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-4418\/13\/1\/9\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:45:18Z","timestamp":1760147118000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-4418\/13\/1\/9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12,21]]},"references-count":57,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2023,1]]}},"alternative-id":["diagnostics13010009"],"URL":"https:\/\/doi.org\/10.3390\/diagnostics13010009","relation":{},"ISSN":["2075-4418"],"issn-type":[{"type":"electronic","value":"2075-4418"}],"subject":[],"published":{"date-parts":[[2022,12,21]]}}}