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DOI: 10.1055/s-0045-1807989
The impact of location on the prognosis of patients with embryonal rhabdomyosarcoma: a systematic review
Authors
Introduction: Rhabdomyosarcoma (RMS) is a malignant soft tissue tumor originating from embryonic mesenchymal cells that differentiate into skeletal muscle tissue. It is the most common sarcoma in childhood, accounting for over 50% of pediatric sarcoma cases. RMS exhibits extensive local dissemination and a high propensity for metastasis. It is frequently found in the head, neck, genitourinary system, and extremities. However, its primary occurrence in the larynx is rare.
Objective: This study aims to conduct a systematic literature review on the relationship between the anatomical location of embryonal rhabdomyosarcoma and its impact on the prognosis of pediatric patients, considering advancements and therapeutic challenges.
Methodology: A systematic review of the literature was carried out on the SciELO, PubMed, BVS and Google Scholar databases, covering the period from 2014 to 2024. The descriptors “Embryonal rhabdomyosarcoma”, “rhabdomyosarcoma” and “pediatric” were used, including full and free articles.
Results: RMS is divided into embryonic, alveolar and pleomorphic subtypes. The embryonic type is the most common in the head and neck and has a generally better prognosis, with no metastases. The analyzed articles reported atypical cases of RMS in different sites such as the larynx, biliary tree, liver, and conjunctiva. Symptoms vary according to the location of the tumor, and may include hoarseness, dysphagia, dyspnea, and obstructive jaundice. And these diverse clinical manifestations can lead to complex and delayed diagnoses. Diagnosis is based on clinical, radiological, and histopathological examinations. Treatment involves chemotherapy and radiotherapy, with variation in response and prognosis. The 5-year survival rate for embryonal rhabdomyosarcoma is approximately 22.6%. Metastasis to lymph nodes is a significant prognostic factor, associated with lower survival if the metastasis is positive.
Conclusion: Embryonal rhabdomyosarcoma, although it is the most common type in childhood and generally has a more favorable prognosis, shows significant variations according to the location of the tumor. The integration of personalized therapeutic approaches is essential to improve patients' quality of life and survival. Imaging tests are crucial for diagnosis and appropriate therapeutic management, which includes chemotherapy and radiotherapy.
Corresponding author: Larissa Maria Moraes Rodrigues de Souza (e-mail: larissammoraesrsouza@gmail.com).
No conflict of interest has been declared by the author(s).
Publication History
Article published online:
06 May 2025
© 2025. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution 4.0 International License, permitting copying and reproduction so long as the original work is given appropriate credit (https://creativecommons.org/licenses/by/4.0/)
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Larissa Maria Moraes Rodrigues de Souza, Sandy da Silva Ribeiro, Stephanie Zarlotim Jorge, Marcella Silvestre Xavier, Felipe Rodrigues Paganini, Melissa Araújo Martins, Isabela Lima Hitzschky, Yasmin Mendes Rodrigues dos Santos, Beatriz Cruz de Paula, Lorrany Eduarda Pereira de Moraes, Heleonara das Graças de Souza Ferraz, Carolina Trujilo, David Lana Silva, Sabrina Jutkoski, Nícolas Freitas dos Santos, Kethlen Torres Cavinato, Beatriz da Silva Martins, Renata Mendes de Almeida, Duana Xavier Dantas Rodrigues, Grazielle Suhett. The impact of location on the prognosis of patients with embryonal rhabdomyosarcoma: a systematic review. Brazilian Journal of Oncology 2025; 21.
DOI: 10.1055/s-0045-1807989