Rhabdomyosarcoma in the Oral Cavity of A Pediatric Patient: A Rare Case

Abstract

Dear Editor, Rhabdomyosarcoma (RMS) is a rare, aggressive, and malignant neoplasm with rapid growth composed of primitive mesenchymal cells that exhibit skeletal muscle differentiation and mainly affects children and adolescents (60%) [1]. RMS is the most common soft tissue sarcoma with a rate of 50-60% in pediatric patients and ranks third among pediatric extracranial solid tumors, following Wilms tumor and neuroblastoma at a rate of 4-5% [2]. Head and neck localizations constitute 35–40% of cases, with oral lesions being extremely rare [3]. RMS has four well-defined subtypes: embryonal, alveolar, spindle cell/sclerosing, and pleomorphic. Embryonal RMS comprises 70-75% of all RMS cases [4], which are mostly sporadic. However, an increase has occurred in the number of recent studies on RMS, and the conclusions of these studies have shown that the children detected with defects in the RAS or Hedgehog pathways, as well as those with predisposing familial syndromes, carry a higher risk for developing embryonal RMS. In addition, PAX3-FOX01 and PAX7-FOXO1 fusions have been identified in alveolar RMS and been accepted as diagnostic markers by many researchers [4-7]. The literature describes the clinical signs and symptoms of oral RMS as rapidly growing swelling, facial asymmetry, paresthesia, trismus, and difficulty swallowing [1,8].

Keywords

• Rhabdomyosarcoma
• pathology
• oncology
• pediatrics

References

1. Komatsu Y, Kawai T, Miura S, Takeda Y, Yamada H. Rhabdomyosarcoma in the maxillary gingiva of a child patient. Journal of Surgical Case Reports 2021;7:1-4. DOI: 10.1093/jscr/ rjab322 google scholar
2. Demellawy DE, McGowan-Jordan J, De Nanassy J, Chernetsova E, Nasr A. Update on molecular findings in rhabdomyosarcoma. Pathology 2017;49(3):238-246. DOI: 10.1016/j.pathol.2016.12.345 google scholar
3. Smith MH, Atherton D, Reith JD, Islam NM, Bhattacharyya I, Cohen DM. Rhabdomyosarcoma, Spindle Cell/Sclerosing Variant: A Clinical and Histopathological Examination of this Rare Variant with Three New Cases from the Oral Cavity. Head Neck Pathol 2017;11(4):494-500. DOİ: 10.1007/s12105-017-0818 google scholar
4. Candaner I, Ocak GA. Rabdomiyosarkomlar. Turkiye Klinikleri J Med Pathol-Special Topics 2017;2(2):165-72. google scholar
5. Clay MR. Embryonal rhabdomyosarcoma. PathologyOutlines.com website. Accessed January 1st, 2023. Available from: https://www. pathologyoutlines.com/topic/softtissueembryonalrhabdo.html. google scholar
6. Ekmekci S. Tümörlerde moleküler değişimler: Mezenkimal tümörler. Türkiye Klinikleri Tıbbi Onkoloji-Özel Sayı 2021;1: 83-8. google scholar
7. A lmazan-Moga A, Zarzosa P, Vidal I, Molist C, Giralt I, Navarro N, et al. Hedgehog Pathway Inhibition Hampers Sphere and Holoclone Formation in Rhabdomyosarcoma. Stem Cells Int. 2017;1-14. DOİ: 10.1155/2017/7507380 google scholar
8. Chi AC, Barnes JD, Budnick S, Agresta SV, Neville B. Rhabdomyosarcoma of the maxillary gingiva. J Periodontol 2007;78(9):1839-45. DOİ: 10.1902/jop.2007.060454 google scholar

9. Peters SM, Kunkle T, Perrino MA, Philipone EM, Yoon AJ. Mandibular embryonal rhabdomyosarcoma with cartilaginous metaplasia: report of a case and review of literature. Oral Surg Oral Med Oral Pathol Oral Radiol 2017;124(6):288-293. DOİ: 10.1016/j. oooo.2017.08.014. google scholar
10. Batra S, Srivastava A, Singh AK, Krishnan A. Oral rhabdomyosarcoma of mandibular region: A case report. Natl J Maxillofac Surg. 2023 May-Aug;14(2):330-333. DOİ: 10.4103/njms.njms_55_22. google scholar
11. Shrutha SP, Vinit GB. Rhabdomyosarcoma in a pediatric patient: A rare case report. Contemp Clin Dent 2015;6(1):113-5. DOİ: 10.4103/0976-237X.149305 google scholar
12. Alfazaz A, Assoumane I, Adakal O, Adamou H, Magagi IA, Baare I. Oropharyngeal Rhabdomyosarcoma with cranial nerve paralysis in a limited resource setting: a case report and review of literature. Pan Afr Med J. 2019;34:51. DOİ: 10.11604/pamj.2019.34.51.20255. google scholar
13. D^ez-Montiel A, Antunez-Conde R, Navarro Cuellar C, Tousidonis Rial M, Salmeron JI, Bonsfills N, Pujol CA, Serrano FA, Ochandiano S. Embryonal Rhabdomyosarcoma of the Tongue in Adults. Life (Basel). 2023 May 25;13(6):1255. DOİ: 10.3390/life13061255. google scholar
14. Glosli H, Bisogno G, Kelsey A, Chisholm JC, Gaze M, Kolb F, et al. Non-parameningeal head and neck rhabdomyosarcoma in children, adolescents, and young adults: Experience of the European paediatric Soft tissue sarcoma Study Group (EpSSG)-RMS2005 study. Eur J Cancer 2021;151:84-93. DOİ: 10.1016/j. ejca.2021.04.007 google scholar