Embryonal rhabdomyosarcoma presenting as edema of the soft palate and necrosis of the uvula: a case report and review of the literature
DOI:
https://doi.org/10.18203/issn.2454-5929.ijohns20242044Keywords:
RMS, Embryonal RMS, Sarcoma, Soft palate, UvulaAbstract
Rhabdomyosarcoma (RMS) is a malignant tumor that affects skeletal muscles, typically found in the head and neck region of children. There have been a few reported cases of RMS occurring in the oral cavity. The prognosis of a patient with this condition depends on many clinical and biological factors, including age, the location of the tumor, histopathological subtype, and whether it has spread at the time of diagnosis. This case report and literature review focus on an unusual presentation of embryonal RMS in the uvula. The report described a case of a young Mexican child presented with symptoms such as edema, swelling of the soft palate, and necrosis of the uvula. The authors discuss the characteristics, management, and treatment of this neoplasm, providing valuable insights on the prognosis and therapeutic approaches for this rare condition and considering that the head and neck region can be a challenging site to treat due to the young age of the patient and the critical anatomy of this area.
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References
Zhang Y, Zhang WL, Huang DS, Wang YZ, Hu HM, Mei YY, et al. Prognostic factors in children with head and neck rhabdomyosarcoma: a 12-year retrospective study. Brain Behav. 2020;10:e01697.
Radzikowska J, Kukwa W, Kuwka A, Czarnecka A, Kreski A. Rhabdomyosarcoma of the head and neck in children. Contemp Oncol. 2015;19(2):98-107.
Casey DL, Wolden SL. Rhabdomyosarcoma of the head and neck: A multimodal approach. J Neurol Surg B 2018;79:58-64.
Obrebowski A, Kaczmarek-Kanold M, Kuczkowski A. Embryonal rhabdomyosarcoma of the uvula in a 5-year-old child. Otolaryngol Pol. 1991;45(5):386-90.
Vieta LJ, Guraieb SR, Obregon MA. Rhabdomyosarcoma-type botryoides of the uvula and soft palate. Arch Otolaryngol. 1962;75:248-53.
Saigal RK, Khurana C, Khanna SD. Pleomorphic rhabdomyosarcoma of the uvula--report of a case and review of the literature. Indian J Cancer. 1973;10(2):243-5.
Anik Y, Corapcioglu F, Aydin O. Rhabdomyosarcoma of the uvula. Pediatr Hematol Oncol. 2007;24(8):639-40.
Kim HJ, Moammar A. Botryoid rhabdomyosarcoma of uvula-A case report. Pathology. 2018;50(1):S76-7.
Datta S, Ray JG, Deb T, Patsa S. Embryonal rhabdomyosarcoma: A rare oral tumor. J Oral Maxillofacial Pathol. 2016;20(3):527-31.
Anwar Sadat AM, Shaikh MH, Badruddoza M. Rhabdomyosarcoma of a 7-year-old girl from Bangladesh: A case report with review of literature. Oral Maxillofacial Surg Cases. 2020;6(4):10.
Seigel R, Kimberly DM, Nikita SW, Ahmedin J. Statistics adapted from the American Cancer Society website. Additional source was: Cancer Statistics 2023. CA Can J Clin. 2023;73(1):17-48.
Komatsu Y, Kawai T, Miura S, Takeda Y, Yamada H. Rhabdomyosarcoma in the maxillary gingiva of a child patient. J Surg Case Rep. 2021;2021(7):rjab322.
Frankart AJ, Breneman JC, Pater LE. Radiation Therapy in the Treatment of Head and Neck Rhabdomyosarcoma. Cancers. 2021;13(14):3567.
Shrutha SP, Vinit GB. Rhabdomyosarcoma in a pediatric patient: A rare case report. Contemp Clin Dent. 2015;6:113-5.
Wosho AA, Huang SC, Chen S, Kashikar S, Estilo CL, Wolden SL, et al. A clinicopathologic study of head and neck rhabdomyosarcomas showing FOXO1 fusion-positive alveolar and MYOD1-mutant sclerosing are associated with unfavorable outcomes. Oral Oncol. 2016;61:89-97.
Haduong JH, Heske CM, Allen-Rhoades W, Xue W, Teot LA, Rodeberg DA, et al. An update on rhabdomyosarcoma risk stratification and the rationale for current and future Children's Oncology Group clinical trials. Pediatr Blood Cancer. 2022;69(4):e29511.
