Alveolar Rhabdomyosarcoma of Nasopharynx and Paranasal Sinuses in Children Diagnosis and Treatment—Review of the Literature and Case Report
Alveolar rhabdomyosarcoma (aRMS) is a rare pediatric malignant tumor with a poor prognosis, particularly when located in the rhinopharynx and sphenoidal floor, which complicates diagnosis and increases the risk of misclassification as benign growths. The specific genotype of aRMS is associated with...
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2025-01-01
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| author | Cristian Mârțu Ștefan Dragoș Tîrnovanu Ioana Mârțu Dan Ferariu Dan Mârțu Alexandra Jităreanu Luminița Rădulescu |
| author_facet | Cristian Mârțu Ștefan Dragoș Tîrnovanu Ioana Mârțu Dan Ferariu Dan Mârțu Alexandra Jităreanu Luminița Rădulescu |
| author_sort | Cristian Mârțu |
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| description | Alveolar rhabdomyosarcoma (aRMS) is a rare pediatric malignant tumor with a poor prognosis, particularly when located in the rhinopharynx and sphenoidal floor, which complicates diagnosis and increases the risk of misclassification as benign growths. The specific genotype of aRMS is associated with a worse clinical outcome. In young children, especially those aged 4 to 12 years, rhinopharyngeal masses are often attributed to chronic adenoiditis; however, other benign (e.g., angiofibroma in boys) and malignant tumors may also be present. Initial symptoms frequently include nasal obstruction, muco-purulent nasal discharge, serous otitis media, sinusitis, and epistaxis. Rhabdomyosarcoma is the second most common ENT neoplasm in children, following lymphoma, with an incidence of approximately 6 cases per 1,000,000 annually. This report presents the case of an 8-year-old boy diagnosed with aRMS, accompanied by a literature review. Alveolar rhabdomyosarcoma should be suspected in children presenting with a vegetative tumor in the rhinopharynx or paranasal sinuses. Combined treatment approaches (surgery, radiotherapy, and chemotherapy) should be tailored to tumor characteristics. Neuronavigation-guided functional endoscopic sinus surgery (FESS) is an effective option for achieving complete tumor excision, depending on tumor size and extent. The prognosis remains reserved and is contingent upon accurate evaluation and timely intervention. Rigorous follow-up, including endoscopic and imagistic investigation, is crucial for early detection of recurrences, thereby improving treatment outcomes. |
| format | Article |
| id | doaj-art-472b68e5a7d24dc7bfd9b5c8c7f756e3 |
| institution | Kabale University |
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| language | English |
| publishDate | 2025-01-01 |
| publisher | MDPI AG |
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| series | Medicina |
| spelling | doaj-art-472b68e5a7d24dc7bfd9b5c8c7f756e32025-01-24T13:40:30ZengMDPI AGMedicina1010-660X1648-91442025-01-016118010.3390/medicina61010080Alveolar Rhabdomyosarcoma of Nasopharynx and Paranasal Sinuses in Children Diagnosis and Treatment—Review of the Literature and Case ReportCristian Mârțu0Ștefan Dragoș Tîrnovanu1Ioana Mârțu2Dan Ferariu3Dan Mârțu4Alexandra Jităreanu5Luminița Rădulescu6ENT Clinic Department, “Grigore T. Popa” University of Medicine and Pharmacy Iasi, Universitatii Street 16, 700115 Iasi, RomaniaDepartment of Orthopedics and Traumatology, Faculty of Medicine, “Grigore T. Popa” University of Medicine and Pharmacy, 700115 Iasi, RomaniaDepartment of Oral Implantology, Removable Dentures and Technology, “Grigore T. Popa” University of Medicine and Pharmacy Iasi, Universitatii Street 16, 700115 Iasi, RomaniaDepartment of Pathology, Regional Institute of Oncology, 700483 Iasi, RomaniaENT Clinic Department, “Grigore T. Popa” University of Medicine and Pharmacy Iasi, Universitatii Street 16, 700115 Iasi, RomaniaDepartment of Toxicology, Faculty of Pharmacy, “Grigore T. Popa” University of Medicine and Pharmacy, 700115 Iasi, RomaniaENT Clinic Department, “Grigore T. Popa” University of Medicine and Pharmacy Iasi, Universitatii Street 16, 700115 Iasi, RomaniaAlveolar rhabdomyosarcoma (aRMS) is a rare pediatric malignant tumor with a poor prognosis, particularly when located in the rhinopharynx and sphenoidal floor, which complicates diagnosis and increases the risk of misclassification as benign growths. The specific genotype of aRMS is associated with a worse clinical outcome. In young children, especially those aged 4 to 12 years, rhinopharyngeal masses are often attributed to chronic adenoiditis; however, other benign (e.g., angiofibroma in boys) and malignant tumors may also be present. Initial symptoms frequently include nasal obstruction, muco-purulent nasal discharge, serous otitis media, sinusitis, and epistaxis. Rhabdomyosarcoma is the second most common ENT neoplasm in children, following lymphoma, with an incidence of approximately 6 cases per 1,000,000 annually. This report presents the case of an 8-year-old boy diagnosed with aRMS, accompanied by a literature review. Alveolar rhabdomyosarcoma should be suspected in children presenting with a vegetative tumor in the rhinopharynx or paranasal sinuses. Combined treatment approaches (surgery, radiotherapy, and chemotherapy) should be tailored to tumor characteristics. Neuronavigation-guided functional endoscopic sinus surgery (FESS) is an effective option for achieving complete tumor excision, depending on tumor size and extent. The prognosis remains reserved and is contingent upon accurate evaluation and timely intervention. Rigorous follow-up, including endoscopic and imagistic investigation, is crucial for early detection of recurrences, thereby improving treatment outcomes.https://www.mdpi.com/1648-9144/61/1/80alveolar rhabdomyosarcomarhinopharynxparanasal sinusespediatric |
| spellingShingle | Cristian Mârțu Ștefan Dragoș Tîrnovanu Ioana Mârțu Dan Ferariu Dan Mârțu Alexandra Jităreanu Luminița Rădulescu Alveolar Rhabdomyosarcoma of Nasopharynx and Paranasal Sinuses in Children Diagnosis and Treatment—Review of the Literature and Case Report Medicina alveolar rhabdomyosarcoma rhinopharynx paranasal sinuses pediatric |
| title | Alveolar Rhabdomyosarcoma of Nasopharynx and Paranasal Sinuses in Children Diagnosis and Treatment—Review of the Literature and Case Report |
| title_full | Alveolar Rhabdomyosarcoma of Nasopharynx and Paranasal Sinuses in Children Diagnosis and Treatment—Review of the Literature and Case Report |
| title_fullStr | Alveolar Rhabdomyosarcoma of Nasopharynx and Paranasal Sinuses in Children Diagnosis and Treatment—Review of the Literature and Case Report |
| title_full_unstemmed | Alveolar Rhabdomyosarcoma of Nasopharynx and Paranasal Sinuses in Children Diagnosis and Treatment—Review of the Literature and Case Report |
| title_short | Alveolar Rhabdomyosarcoma of Nasopharynx and Paranasal Sinuses in Children Diagnosis and Treatment—Review of the Literature and Case Report |
| title_sort | alveolar rhabdomyosarcoma of nasopharynx and paranasal sinuses in children diagnosis and treatment review of the literature and case report |
| topic | alveolar rhabdomyosarcoma rhinopharynx paranasal sinuses pediatric |
| url | https://www.mdpi.com/1648-9144/61/1/80 |
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