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Rabdomyosarkóm klivu – kazuistika


Authors: T. Haličková 1,2;  M. Sičák 1;  A. Kališ 3;  K. Obtulovičová 1
Published in: Otorinolaryngol Foniatr, 74, 2025, No. 2, pp. 153-158.
Category: Case Reports
doi: https://doi.org/10.48095/ccorl2025153

Overview

Rhabdomyosarkóm je vysoko malígny mezenchymálny nádor, ktorý vyrastá z primitívneho mezenchýmu, ktorý sa normálne diferencuje prevažne na priečne pruhované kostrové svalstvo. Ide o najčastejší mäkkotkanivový sarkóm v detskom veku s preferovaným výskytom v oblasti hlavy a krku. Multimodálny prístup v liečbe zahŕňa chirurgiu, chemoterapiu a rádioterapiu. Predstavujeme prípad 6-ročného dievčaťa s anamnézou nosovej obštrukcie a hlienovej sekrécie viac na ľavej strane, so stratou čuchu a bolesťou hlavy nad ľavým okom. Pri klinickom vyšetrení bol zistený ružový nádor v ľavej nosovej dutine. Doplnené boli CT a MR vyšetrenia, ktoré ukázali nádorovú masu vypĺňajúcu celý nosohltan, s expanziou do ľavej klinovej dutiny, ku klivu a do prevertebrálneho priestoru. Nádorová masa kompletne uzatvárala obe choány. Pacientka podstúpila endoskopickú endonazálnu resekciu nádoru v oblasti prednej bázy lebky s využitím CT navigácie. Bol potvrdený embryonálny rhabdomyosarkóm. V pooperačnom období pacientka podstúpila adjuvantnú onkologickú liečbu, kontrolné biopsie 2 a 16 mesiacov po operácii boli negatívne. Pacientka je momentálne 3 roky po operácii – klinicky bez známok recidívy nádoru. Záver: Rhabdomyosarkóm, podobne ako iné nádory v oblasti prednej bázy lebky, predstavuje výzvu pre chirurga vzhľadom na lokalizáciu, v ktorej je úplná resekcia nádoru niekedy veľmi náročná alebo nemožná. Dôkladná predoperačná analýza zobrazovacích vyšetrení ako aj intraoperačné využitie CT/MR navigácie to umožňujú. Kompletné odstránenie nádoru zvyšuje šancu pacienta na úspešnú liečbu.

Klíčová slova:

rhabdomyosarkom – nádor prednej bázy lebky – endonazálna endoskopická resekcia


Sources

1. Haussler SM, Stromberg C, Olze H et al. Head and neck rhabdomyosarcoma in children: a 20-year retrospective study at a tertiary referral centre. J Cancer Res Clip Once 2018; 144 (2): 371–379. Doi: 10.1007/s00432-017-2544-x.

2. Reilly BK, Kim A, Pena MT et al. Rhabdomyosarcoma of the head and neck in children: review and update. Int J Pediatry Otorhinolaryngol 2015; 79 (9): 1477–1483. Doi: 10.1016/ j.ijporl.2015.06.032.

3. Janeček D, Bartoňková K, Vogazianos E et al. Differential Diagnostics of Expansive Processes at the Child age in ORL Area. Otorinolaryngol Foniatr 2008; 57 (2): 59–64.

4. Radzikowska J, Kukwa W, Kukwa A et al. Management of pediatric head and neck rhabdomyosarcoma: a case-series of 36 patients. Once Lett 2016; 12 (5): 3555–3562. Doi: 10.3892/ol. 2016.5072.

5. Ma X, Huang D, Zhao W et al. Clinical characteristics and prognosis of childhood rhabdomyosarcoma: a ten year retrospective multicenter study. Int J Chin Exp Med 2015; 8 (10): 17196–17205.

6. Shern JF, Yohe ME, Khan J. Pediatric Rhabdomyosarcoma. Crit Rev Oncog 2015; 20 (3–4): 227–243. Doi: 10.1615/critrevoncog.2015013800

7. Shern JF, Chen L, Chmielecki J et al. Comprehensive genomic analysis of rhabdomyosarcoma reveals a landscape of alterations affecting a common genetic axis in fusion-positive and fusion-negative tumors. Cancer Discov 2014; 4 (2): 216–231. Doi: 10.1158/2159-8290.CD-13-0639.

8. Dombrowski ND, Wolter NE, Robson CD et al. Role of surgery in rhabdomyosarcoma of the head and neck in children. Laryngoscope 2020, 131 (3): E984–E992. Doi: 10.1002/lary.28785.

9. Rudzinski ER, Anderson JR, Hawkins DS et al. The World Health Organization Classification of Skeletal Muscle Tumors in Paediatric Rhabdomyosarcoma. A Report From the Children’s Oncology Group. Arch Pathol Lab Med 2015; 139 (10): 1281-1287. Doi: 10.5858/arpa.2014-0475-OA.

10. Fletcher CDM, Bridge JA, Hogendoorn PCW et al. WHO Classification of Tumors of Soft Tissue and Bone. 4th ed. Lyon, France: International Agency for Research on Cancer; 2013. World Health Organization Classification of Tumors; vol. 5.

11. Maurer HM, Beltangady M, Gehan EA et al. The Intergroup Rhabdomyosarcoma Study, I: a final report. Cancer 1988; 61 (2): 209–220. Doi: 10.1002/1097-0142 (19880115) 61: 2<209:: aid-cncr2820610202>3.0.co; 2- l.

12. Maurer HM, Gehan EA, Beltangady M et al. The Intergroup Rhabdomyosarcoma Study, II: Cancer 1993; 71 (5): 1904-1922. Doi: 10.1002/10970142 (19930301) 71: 5<1904:: aid-cncr2820710530>3.0.co; 2-x.

13. Darwish C, Shim T, Sparks AD et al. Pediatric head and neck rhabdomyosarcoma: An analysis of treatment and survival in the United States (1975–2016). Int J Pediatr Otorhinolaryngol 2020; 139: 110403. Doi: 10.1016/ j.ijporl.2020.110403.

14. Kazanowska B, Reich A, Reich M et al. Remaining problems and controversies in the management of childhood head and neck soft tissue sarcomas: Retrospective (national) Multicenter Study of the Polish Pediatric Solid Tumors Group. Pediatr Hematol Oncol 2004; 21 (4): 349–363. Doi: 10.1080/08880010490440491.

15. Raney RB, Maurer HM, Anderson JR et al. The Intergroup Rhabdomyosarcoma Study Group (IRSG): Major Lessons From the IRS-I Through IRS-IV Studies as Background for the Current IRS-V Treatment Protocols. Sarcoma 2001; 5 (1): 9–15. Doi: 10.1080/13577140120048890.

16. Wharam MD, Beltangady MS, Heyn RM et al. Pediatric orofacial and laryngopharyngeal rhabdomyosarcoma: an Intergroup Rhabdomyosarcoma Study report. Arch Otolaryngol Head Neck Surg 1987; 113 (11): 1225–1227. Doi: 10.1001/archotol.1987.01860110091014.

17. Daya H, Chan HSL, Sirkin W et al. Pediatric Rhabdomyosarcoma of The Head and Neck Is There a Place for Surgical Management? Arch Otolaryngol Head Neck Surg 2000; 126 (4): 468–472. Doi: 10.1001/archotol.126.4.468.

18. Smith LM, Anderson JR, Qualman SJ et al. Which patients with microscopic disease and rhabdomyosarcoma experience relapse after therapy? A report from the soft tissue sarcoma commitee of the children’s oncology group. J Clin Oncol 2001; 19 (20): 4058–4064. Doi: 10.1200/JCO.2001.19.20.4058.

19. Matoušek P, Lipina R, Paleček T et al. Endo- scopic Transnasal Surgery of Sellar Region. Otorinolarynol Foniatr 2012; 61 (3): 172–177.

20. Iatrou I, Theologie-Lygidakis N, Schoinohoriti O et al. Rhabdomyosarcoma of the maxillofacial region in children and adolescents: report of 9 cases and literature review. J Craniomaxillofac Surg 2017; 45 (6): 831–838. Doi: 10.1016/j.jcms.2017.03.005.

21. Wolden SL, Anderson JR, Crist WM et al. Indications for radiotherapy and chemotherapy after complete resection in rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Studies I to III. J Clin Oncol 1999; 17 (11): 3468–3475. Doi: 10.1200/JCO.1999.17.11.3468.

22. Ray A, Huh WW. Current state-of-the-art systemic therapy for pediatric soft tissue sarcomas. Curr Oncol Rep 2012; 14 (4): 311–319. Doi: 10.1007/s11912-012-0243-y.

23. Lawrence W Jr, Anderson J, Gehan EA et al. Pretreatment TNM staging of childhood rhabdomyosarcoma: a report of the Intergroup Rhabdomyosarcoma Study Group. Cancer 1997; 80 (6): 1165–1170.

24. FaR – RMS. An overarching study for children and adults with Frontline and Relapsed RhabdoMyoSarcoma Version 1.0b, 11th November 2019: 44. Table 3: Risk Group Assignment.

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