Oral Embryonal Rhabdomyosarcom in an Adult Addict Man: a case report and review of literature

Document Type : Case Review


1 School of Dentistry, Tehran University of Medical Sciences, Tehran, Iran Dental Research Center, Dentistry Research Institute, Tehran University of Medical Sciences, Tehran, Iran The Academy of Medical Sciences, Tehran, Iran

2 Oral & Maxillofacial Medicine Specialist, Chalus, Iran

3 School of Dentistry, Tehran University of Medical Sciences, Tehran, Iran



Background: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, but rare in adults, especially those in the middle age and older. Embryonal rhabdomyosarcoma in the head and neck region is relatively common, but rarely found intra-orally. A clear etiologic risk factor for the neoplastic growth of this malignancy has not been identified yet.
Method: Here, we report a case of an adult addict patient, with locally extensive embryonal rhabdomyosarcoma (ERMS) of tuberosity and palate, who received chemotherapy and radiotherapy, but died due to the progression of the disease 15 months after the diagnosis.
We also carried out an overview of previous articles reporting this sarcoma in the oral cavity through searching keywords on the following online databases, without any date limitations: Pubmed, Scopous, MEDLINE, Google Scholar, and MD-Consult.
Our search revealed only 8 articles reporting the occurrence of embryonal RMS in the maxilla among adults and none of them referred to any addict patient.
Conclusion: The occurrence of oral ERMS in adults is a rare phenomenon associated with poor prognosis. Furthermore, addiction can contribute to more rapid tumor growth, treatment resistance, and reduced survival through mutagenic induction, immunosuppression, and angiogenesis.


  1. Parham DM. Pathologic classification of rhabdomyosarcomas and correlations with molecular studies. Mod Pathol 2001; 14(5):506-14. doi: 10.1038/modpathol.3880339.
  2. Ognjanovic S, Linabery AM, Charbonneau B, Ross JA. Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975‐2005. Cancer 2009; 115(18):4218-26. doi: 10.1002/cncr.24465.
  3. Anderson GJ, Tom LW, Womer RB, Handler SD, Wetmore RF, Potsic WP. Rhabdomyosarcoma of the head and neck in children. Arch Otolaryngol Head Neck Surg 1990; 116(4):428-31. doi: 10.1001/archotol.1990.01870040050012.
  4. Fletcher CD, Bridge JA, Hogendoorn PC, Mertens F. WHO classification of tumors. Pathology and genetics. 4th Geneva: IARC Publication. https://ci.nii.ac.jp/naid/10026635818/
  5. Johanna CA, Setiawan GB. Retromolar embryonal rhabdomyosarcoma: a case report. Jurnal Bedah Nasional 2017; 1(2):47-52.
  6. Chen SY, Thakur A, Miller AS, Harwick RD. Rhabdomyosarcoma of the oral cavity: Report of four cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995; 80(2):192-201. doi: 10.1016/s1079-2104(05)80202-1.
  7. Stout AP. Rhabdomyosarcoma of the skeletal muscles. Ann Surg 1946; 123(3):447-72.
  8. Chigurupati R, Alfatooni A, Myall RW, Hawkins D, Oda D. Orofacial rhabdomyosarcoma in neonates and young children: a review of literature and management of four cases. Oral Oncol 2002; 38(5):508-15. doi: 10.1016/s1368-8375(01)00087-2.
  9. Sultan I, Qaddoumi I, Yaser S, Rodriguez-Galindo C, Ferrari A. Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: an analysis of 2,600 patients. J Clin Oncol 2009; 27(20):3391-7. doi: 10.1200/JCO.2008.19.7483.
  10. Geiger S, Czernobilsky B, Marshak G, Geiger B. Embryonal rhabdomyosarcoma: immunohistochemical characterization. Oral Surg Oral Med Oral Pathol 1985; 60(5):517-23. doi: 10.1016/0030-4220(85)90241-5.
  11. Suresh D, Manjula M, Kumuda P, Aruna P. Embryonal rhabdomyosarcoma of oral cavity–A case report. J Res Adv Dent 2013; 2(2):78-81.
  12. Sadeghi EM, Gingrass DJ, Surwillo EJ, Anderson T, Tang TT. Embryonal rhabdomyosarcoma. Int J Oral Maxillofac Surg 1988; 17(3):198-200. doi: 10.1016/s0901-5027(88)80032-8.
  13. Roberts SS, Chou AJ, Cheung NK. Immunotherapy of childhood sarcomas. Front Oncol 2015; 5:181. doi: 10.3389/fonc.2015.00181.
  14. Little DJ, Ballo MT, Zagars GK, Pisters PW, Patel SR, El‐Naggar AK, et al. Adult rhabdomyosarcoma. Cancer 2002; 95(2):377-88. doi: 10.1002/cncr.10669.
  15. Arul AS, Verma S, Arul AS, Verma R. Oral rhabdomyosarcoma-embryonal subtype in an adult: A rarity. J Nat Sci Biol Med 2014; 5(1):222-5. doi: 10.4103/0976-9668.127347.
  16. Ward E, DeSantis C, Robbins A, Kohler B, Jemal A. Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 2014; 64(2):83-103. doi: 10.3322/caac.21219.
  17. Yasuda T, Perry KD, Nelson M, Bui MM, Nasir A, Goldschmidt R, et al. Alveolar rhabdomyosarcoma of the head and neck region in older adults: geneticcharacterization and a review of the literature. Hum Pathol 2009; 40(3):341-8.
  18. Pavithran K, Doval DC, Mukherjee G, Kannan V, Kumaraswamy SV, Bapsy PP. Rhabdomyosarcoma of the oral cavity: report of eight cases. Acta Oncol 1997; 36(8):819-21. doi: 10.3109/02841869709001364.
  19. Iatrou I, Theologie-Lygidakis N, Schoinohoriti O, Tzermpos F, Vessala AM. Rhabdomyosarcoma of the maxillofacial region in children and adolescents: Report of 9 cases and literature review. J Cranio-Maxillo Surg 2017; 45(6):831-8. doi: 10.1016/j.jcms.2017.03.005.
  20. Sood N, Sehrawat N. Adult onset sinonasal rhabdomyosarcoma-a rare case report with cytohistological features. Malaysian J Patho 2016; 38(2):169-73.
  21. Esnaola NF, Rubin BP, Baldini EH, VasudevanN, Demetri GD, Fletcher CD, et al. Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma. Annal Surg 2001; 234(2):215-23. doi: 10.1097/00000658-200108000-00012.
  22. Fatusi OA, Ajike SO, Olateju SO, Adebayo AT, Gbolahan OO, Ogunmuyiwa SA. Clinico-epidemiological analysis of orofacial rhabdomyosarcoma in a Nigerian population. Int J Oral Maxillofac Surg 2009; 38(3):256-60. doi: 10.1016/j.ijom.2008.12.002.
  23. O'Day RA, Soule EH, Gores RJ. Embryonal rhabdomyosarcoma of the oral soft tissues. Oral Surg Oral Med Oral Pathol 1965; 20(1):85-93. doi: 10.1016/0030-4220(65)90271-9.
  24. Nakhleh RE, Swanson PE, Dehner LP. Juvenile (embryonal and alveolar) rhabdomyosarcoma of the head and neck in adults. Cancer 1991; 67(4):1019-24. doi: 10.1002/1097-0142(19910215)67:4<1019::aid-cncr2820670426>3.0.co;2-7.
  25. Coindre JM, de Mascarel A, Trojani M, de Mascarel I, Pages A. Immunohistochemical study of rhabdomyosarcoma. Unexpected staining with S100 protein and cytokeratin. J Pathol 1988; 155(2):127-32. doi: 10.1002/path.1711550209.
  26. França CM, Caran EM, Alves MT, Barreto AD, Lopes NN. Rhabdomyosarcoma of the oral tissues--two new cases and literature review. Med Oral Patol Oral Cir Bucal 2006; 11(2):E136-40.
  27. Wu PX, Liang YF, Zeng JC, Ruan JB, Kang DP, Chen C, et al. Embryonal rhabdomyosarcoma of the paranasal sinuses: a case report and review of literature. Int J Clin Exp Med 2014; 7(8):2369-72.
  28. Maurer HM, Crist W, Lawrence W, Ragab AH, Raney RB, Webber B, et al. The intergroup rhabdomyosarcoma study ‐I. A final report. Cancer 1988; 61(2):209-20. doi: 10.1002/1097-0142(19880115)61:2< 209 ::aid-cncr2820610202>3.0.co;2-l.
  29. Rodary C, Rey A, Olive D, Flamant F, Quintana E, Brunat‐Mentigny M, et al. Prognostic factors in 281 children with nonmetastatic rhabdomyosarcoma (RMS) at diagnosis. Med Pediatr Oncol 1988; 16(2):71-7. doi: 10.1002/mpo.2950160202.
  30. Heyn RM, Holland R, Newton WA, Tefft M, Breslow N, Hartmann JR. The role ofcombined chemotherapy in the treatment of rhabdomyosarcoma in children. Cancer 1974; 34(6):2128-45. doi: 10.1002/1097-0142(197412)34:6< 2128::aid-cncr2820340638>3.0.co;2-m.
  31. Shen X, Kosten TR. Immunotherapy for drug abuse. CNS Neurol Disord Drug Targets 2011; 10(8):876-9. doi: 10.2174/187152711799219352.
  32. Nasrollahzadeh D, Kamangar F, Aghcheli K, Sotoudeh M, Islami F, Abnet C, et al. Opium, tobacco, and alcohol use in relation to oesophageal squamous cell carcinoma in a high-risk area of Iran. Br J Cancer 2008; 98(11):1857-63. doi: 10.1038/sj.bjc.6604369.
  33. Ahamadi Mousavi MR, Damghani MA, Haghdoust AA, Khamesipour A. Opium and risk of laryngeal cancer. Laryngoscope 2003; 113(11):1939-43. doi: 10.1097/00005537-200311000-00016.
  34. Randall D, Degenhardt L, Vajdic CM, Burns L, Hall WD, Law M, et al. Increasing cancer mortality among opioid‐dependent persons in Australia: a new public health challenge for a disadvantaged population. Aust N Z J Public Health 2011; 35(3):220-5. doi: 10.1111/j.1753-6405.2011.00682.x.
  35. Hewer T, Rose E, Ghadirian P, Castegnaro M, Malaveille C, Bartsch H, et al. Ingestedmutagens from opium and tobacco pyrolysis products and cancer of the oesophagus. Lancet 1978; 312(8088):494-6. doi: 10.1016/s0140-6736(78)92218-3.
  36. Malekzadeh MM, Khademi H, Pourshams A, Etemadi A, Poustchi H, Bagheri M, et al. Opium use and risk of mortality from digestive diseases: a prospective cohort study. Am J Gastroenterol 2013; 108(11):1757-65. doi: 10.1038/ajg.2013.336.
  37. Sacerdote P, Bianchi M, Gaspani L, Manfredi B, Maucione A, Terno G, et al. The effects of tramadol and morphine on immune responses and pain after surgery in cancer patients. Anesth Analg 2000; 90(6):1411-4. doi: 10.1097/00000539-200006000-00028.
  38. Vaghari BA, Ahmed OI, Wu CL. Regional anesthesia-analgesia: relationship to cancer recurrence and infection. Anesthesiol Clin 2014; 32(4):841-51. doi: 10.1016/j.anclin.2014.08.004.
  39. Zhu W, Ma Y, Bell A, Esch T, Guarna M, Bilfinger TV, et al. Presence of morphine in rat amygdala: Evidence for the mu3 opiate receptor subtype via nitric oxide release in limbic structures. Med Sci Monit 2004; 10(12):BR433-9.
  40. Belcheva MM, Haas PD, Tan Y, Heaton VM, Coscia CJ. The fibroblast growth factor receptor is at the site of convergence between μ-opioid receptor and growth factor signaling pathways in rat C6 glioma cells. J Pharmacol Exp Ther 2002; 303(3):909-18. doi: 10.1124/jpet.102.038554.
  41. Rahim RT, Meissler JJ, Adler MW, Eisenstein TK. Splenic macrophages and B cells mediate immunosuppression following abrupt withdrawal from morphine. J Leukoc Biol 2005; 78(6):1185-91. doi: 10.1189/jlb.0304123.
  42. Williams BA, Williams KM, Doyle J, Stephens D, Greenberg M, Malkin D, et al. Metastatic rhabdomyosarcoma: a retrospective review of patients treated at the hospital for sick children between 1989 and 1999. J Pediatr Hematol Oncol 2004; 26(4):243-7. doi: 10.1097/00043426-200404000-00006.
  43. Dumont SN, Araujo DM, Munsell MF, Salganick JA, Dumont AG, RaymondKA, et al. Management and outcome of 239 adolescent and adult rhabdomyosarcoma patients. Cancer Med 2013; 2(4):553-63. doi: 10.1002/cam4.92.
  44. Pap GS. Rhabdomyosarcoma: Report of a case with involvement of the angle of the mandible. Int J Oral Surg 1980; 9(6):491-3. doi: 10.1016/s0300-9785(80)80083-4.
  45. Yamamoto H, Kozawa Y, Takagi M, Otake S. Rhabdomyosarcoma of the left mandible. J Oral Maxillofac Surg 1984; 42(9):613-8. doi: 10.1016/0278-2391(84)90094-6.
  46. Peters E, Cohen M, Altini M, Murray J. Rhabdomyosarcoma of the oral and paraoral region. Cancer 1989; 63(5):963-6. doi: 10.1002/1097-0142(19890301)63:5<963::aid-cncr2820630529>3.0.co;2-0.
  47. Lazzaro B, Schwartz D, Lewis J, Weiss W. Rhabdomyosarcoma involving the oral cavity, mandible, and roots of the third molar: a clinical-pathologic correlation and review of literature. J Oral Maxillofac Surg 1990; 48(1):72-7. doi: 10.1016/0278-2391(90)90185-5.
  48. Doval DC, Kannan V, Acharya RS, Mukherjee G, Shenoy AM, Bapsy PP. Rhabdomyosarcoma of the tongue. Br J Oral Maxillofac Surg 1994; 32(3):183-6. doi: 10.1016/0266-4356(94)90107-4.
  49. Möller P, Perrier M. Dento-maxillofacial sequelae in a child treated for a rhabdomyosarcoma in the head and neck: A case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endo 1998; 86(3):297-303. doi: 10.1016/s1079-2104(98)90175-5.
  50. Al-Khateeb T, Bataineh AB. Rhabdomyosarcoma of the oral and maxillofacial region in Jordanians: a retrospective analysis. Oral Surg Oral Med Oral Pathol Oral Radiol Endo 2002; 93(5):580-5. doi: 10.1067/moe.2002.123860.
  51. Loducca SV, Mantesso A, de Oliveira EM, de Araújo VC. Intraosseous rhabdomyosarcoma of the mandible: a case report. Int J Surg Pathol 2003; 11(1):57-60. doi: 10.1177/106689690301100115.
  52. Seth T, Kempert P. Embryonal rhabdomyosarcoma of lower lip. Indian Pediatr 2004; 41(8):858-9.
  53. Durães GV, Jham BC, Mesquita AT, dos Santos CR, Miranda JL. Oral embryonal rhabdomyosarcoma in a child: A case report with immunohistochemical analysis. Oral Oncology Extra 2006; 42(3):105-8. doi: 10.1016/j.ooe.2005.09.011.
  54. Chi AC, Barnes JD, Budnick S, Agresta SV, Neville B. Rhabdomyosarcoma of the maxillary gingiva. J Periodontol. 2007 Sep;78(9):1839-45. doi: 10.1902/jop.2007.060454. PMID: 17760557.
  55. Chen SC, Bee YS, Lin MC, Sheu SJ. Extensive alveolar-type paranasal sinus and orbit rhabdomyosarcoma with intracranial invasion treated successfully. J Chin Med Assoc 2011; 74(3):140-3. doi: 10.1016/j.jcma.2011.01.031.
  56. Miloglu O, Altas SS, Buyukkurt MC, Erdemci B, Altun O. Rhabdomyosarcoma of the oral cavity: a case report. Eur J Dent 2011; 5(3):340-3.
  57. Sahni P, Singhvi A, Nayak MT, Deora SS. Gingival rhabdomyosarcoma in an adult: a unique entity. Turk Patoloji Derg 2015; 31(2):153-7. doi: 10.5146/tjpath.2013.01194.
  58. Nema SK, Singh A, Bhasker H, Bhambani P, Sharma HS, Julka K. Alveolar rhabdomyosarcoma in children with histomorphological review. Int J Res Med Sci 2014; 2(2):775-8. doi: 10.5455/2320-6012.ijrms20140580.
  59. Shrutha SP, Vinit GB. Rhabdomyosarcoma in a pediatric patient: A rare case report. Contemp Clin Dent 2015; 6(1):113-5. doi: 10.4103/0976-237X.149305.
  60. Lav R, Heera R, Cherian LM, Ravikumar V, Sood A. Embryonal rhabdomyosarcoma of maxilla–A clinical rarity lending glimpses into embryonal myogenesis. J Oral Maxillofac Surg Med Pathol 2015; 27(5):733-6. doi: 10.1016/j.ajoms.2015.01.002.
  61. Ananthaneni A, Kuberappa PH, Srinivas GV, Kiresur MA. Alveolar rhabdomyosarcoma of maxilla. J Oral Maxillofac Pathol 2016; 20(1):164. doi: 10.4103/0973-029X.180987.
  62. Datta S, Ray JG, Deb T, Patsa S. Embryonal rhabdomyosarcoma: A rare oral tumor. J Oral Maxillofac Pathol 2016; 20(3):527-31. doi: 10.4103/0973-029X.190959.
  63. McInturff M, Adamson A, Donaldson C, Nelson BL. Embryonal rhabdomyosarcoma of the oral cavity. Head Neck Pathol 2017; 11(3):385-8. doi: 10.1007/s12105-016-0761-2.
  64. Radzikowska J, Kukwa W, Kukwa A, Czarnecka AM, Kawecki M, Lian F, et al. Managementof pediatric head and neck rhabdomyosarcoma: A case-series of 36 patients. Oncol Lett 2016; 12(5):3555-62. doi: 10.3892/ol.2016.5072.
  65. Mungan S, Arslan S, Küçüktülü E, Ersöz Ş, Çobanoğlu B. Pleomorphic rhabdomyosarcoma arising from true vocal fold of larynx: report of a rare case and literature review. Case Rep Otolaryngol 2016; 2016:8135967. doi: 10.1155/2016/8135967.
  66. Sepulveda I, Vera P, Platin E, Spencer ML, Klaassen V, Hidalgo C, et al. Sinonasal alveolar rhabdomyosarcoma in an adult patient: a case report and review of the literature. J Cancer Treat Research 2017; 5(2):11-4. doi: 10.11648/j.jctr.20170502.12.
  67. Vhriterhire R, Ugbaje B, Ngbea JA, Ojo BA, Ogbeifun OJ, Eke BA. Maxillary embryonal rhabdomyosarcoma with oral and nasal extension in an adult: a case report and review of literature. Asian J Med Health 2017; 3(4):1-6. doi: 10.9734/AJMAH/2017/31524.
  68. 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. doi: 10.1007/s12105-017-0818-x.
  69. Silva Cunha JL, De Assis Almeida Lima-Júnior F, Gonçalves Júnior WD, De Santana Santos T, Da Silveira EJ, De Sousa SF, et al. Embryonal rhabdomyosarcoma (botryoid subtype) affecting the buccal mucosa. Head Neck Pathol 2019; 13(4):671-6. doi: 10.1007/s12105-018-0957-8.
  70. Motallebnejad M, Aminishakib P, Derakhshan S, Karimi A. Rhabdomyosarcoma of the maxillary gingiva. Dent Res J (Isfahan) 2018; 15(1): 80-83. doi: 10.4103/1735-3327.223619.
  71. Kusafuka K, Kawasaki T, Onitsuka T, Fuke T, Nakamura T, Minato H, et al. Sclerosing rhabdomyosarcoma of the oral floor mimicking a salivary gland tumor: A case report with immunohistochemical and genetic analyses. Human Pathol: Case Reports 2018; 13:39-44. doi: 10.1016/j.ehpc.2018.03.006.
  72. Amtha R, Hartanto FK, Gautama W, Hussaini HM. Pleomorphic rhabdomyosarcoma of the tongue in adult mimicking oral squamous cell carcinoma. Scien Dent J 2019; 3(2):70-4. doi: 10.4103/SDJ.SDJ_10_19.
  73. Joy T, Tupkari JV, Hanchate AV, Siwach P. Oral rhabdomyosarcoma in an adult male: A rare case report. J Oral Maxillofac Pathol 2018; 22(2):285. doi: 10.4103/jomfp.JOMFP_11_17.
  74. De Albuquerque RF, Muniz YVS, Dos Anjos RS, Do Nascimento ML, Valente RODH, Salgado MRT, Silva IHM: Embryonal Rhabdomyosarcoma of the Maxilla: a Case Report. Oral Surg, Oral Med, Oral Pathol Oral Radiol 2018; 126(3):e75. doi: 10.1016/j.oooo.2018.02.207.