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Published on: September 13, 2019
Unusual outer ear swelling: Childhood auricular rhabdomyosarcoma
M Chirat1, L Dainese2, S Fasola3
1Service d'ORL et de chirurgie cervico-faciale, hôpital universitaire Necker Enfants Malades, Paris V University, 149, rue de Sèvres, 75015 Paris, France.
Insights
Rhabdomyosarcoma (RMS) is a rare pediatric cancer. This study highlights successful treatment of three children with rare outer ear RMS, emphasizing early diagnosis and multimodal therapy for good outcomes.
Area of Science:
- Pediatric Oncology
- Surgical Oncology
- Radiation Oncology
Background:
- Rhabdomyosarcoma (RMS) is the most common pediatric soft-tissue sarcoma, typically affecting head and neck sites.
- Outer ear RMS is exceptionally rare, posing diagnostic challenges.
- Presents three pediatric cases of auricular RMS.
Purpose of the Study:
- To report on the diagnosis and management of rare cases of Rhabdomyosarcoma in the outer ear of children.
- To highlight the effectiveness of multimodal treatment strategies in achieving disease control.
Main Methods:
- Three pediatric patients (ages 6-14) with auricular RMS were treated.
- Treatment involved neoadjuvant chemotherapy followed by surgery or radiation therapy, and adjuvant chemotherapy.
Main Results:
- All three patients achieved complete disease control with the implemented treatment protocols.
- Favorable prognosis was observed, attributed to accessible surgical intervention.
- The study underscores the importance of considering RMS in cases of unusual ear swelling.
Conclusions:
- Multimodal treatment, including chemotherapy, surgery, and/or radiation, is effective for auricular RMS in children.
- Early diagnosis and prompt intervention are crucial for favorable outcomes.
- Auricular Rhabdomyosarcoma, though rare, should be considered in the differential diagnosis of pediatric ear masses.
Introduction:
Rhabdomyosarcoma (RMS) is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. The main head and neck locations are the base of the skull, nasopharynx, nasal cavity and orbit. An outer ear location is considered extremely rare. We present 3 cases of children, aged 6 to 14 years, presenting with auricular RMS.
Case Reports:
The first child, aged 6, was managed by 4 chemotherapy cycles followed by surgical resection of the tumor bed, completed by 5 further cycles of chemotherapy. The second, aged 14, was managed by 4 chemotherapy cycles followed by external radiation therapy of the tumor bed and lymph node areas, completed by 5 further cycles of chemotherapy. The third, aged 13, was managed by 4 chemotherapy cycles followed by surgery, completed by 5 further cycles of chemotherapy.
Discussion:
In these 3 patients, the treatment program achieved complete disease control. Prognosis was good, thanks to good surgical access. Diagnosis should be considered in case of unusual progressive swelling in the outer ear.
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