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Clinical-pathologic profile of head and neck rhabdomyosarcoma in children: a systematic review
Ludimila Lemes Moura1, Beatriz Della Terra Mouco Garrido2, Nelson Leonel Del Hierro Polanco1
1Department of Surgery, Stomatology, Pathology and Radiology, Bauru School of Dentistry, University of São Paulo, Bauru, Brazil.
Insights
This review analyzed pediatric head and neck rhabdomyosarcoma, finding that smaller tumors, no metastasis, and complete resection with adjuvant therapies improve prognosis. MYOD1 expression indicates a poor outlook for spindle cell/sclerosing types.
Area of Science:
- Pediatric Oncology
- Cancer Research
- Pathology
Background:
- Head and neck rhabdomyosarcoma is a rare but aggressive malignancy in children.
- Understanding its clinicopathological features and prognostic factors is crucial for effective treatment.
Purpose of the Study:
- To systematically review and analyze the clinicopathological profile of pediatric head and neck rhabdomyosarcoma.
- To identify key prognostic factors influencing patient outcomes.
Main Methods:
- A comprehensive literature search was conducted across major databases (PubMed, Lilacs, Embase, Scopus, Web of Science).
- Three studies were included for qualitative analysis after assessing data extraction and risk of bias using STROBE guidelines.
Main Results:
- Embryonal and alveolar rhabdomyosarcoma were the most common subtypes.
- MYOD1 expression correlated with spindle cell/sclerosing rhabdomyosarcoma, associated with a poor prognosis.
- Tumor size <5 cm, absence of metastasis, complete resection, and adjuvant therapies (chemotherapy, radiotherapy) predicted a better prognosis.
Conclusions:
- Clinicopathological factors and specific molecular markers significantly impact the prognosis of pediatric head and neck rhabdomyosarcoma.
- Early detection, complete surgical resection, and multimodal adjuvant therapies are vital for improving survival rates in affected children.
Abstract:
This systematic review aimed to analyze the clinicopathological profile and relevant prognostic factors of head and neck rhabdomyosarcoma in pediatric patients. The search was carried out in the electronic search portals PubMed, Lilacs, Embase, Scopus, and Web of Science. The search yielded studies that were then analyzed regarding study topic, data extraction, and risk of bias using the STROBE (Strengthening the Reporting of Observational Studies) guidelines. Finally, three studies were included for qualitative analysis. Most of the cases involved embryonic and alveolar rhabdomyosarcoma. Expression of MYOD1 was highly correlated with diagnosis of spindle cell/sclerosing rhabdomyosarcoma, which appears to have a poor prognosis in children. Furthermore, tumor size <5 cm and absence of metastasis accompanied by complete resection and administration of adjuvant therapies such as chemotherapy and radiotherapy favored a better prognosis.
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