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Management of neonatal retro-auricular embryonal rhabdomyosarcoma - Case report
M Jean-Christophe Roubaud1, Julien Prevot1, Jean-Christophe Leclere1
1CHRU de Brest, Department of Otolaryngology, Head & Neck Surgery, Brest, France.
Insights
Rhabdomyosarcoma (RMS) is a rare pediatric soft-tissue sarcoma. Early diagnosis and treatment in infants with retro-auricular RMS led to complete disease control and good prognosis in two cases.
Area of Science:
- Pediatric Oncology
- Skeletal Muscle Biology
- Cancer Genetics
Background:
- Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children, originating from mesenchymal cells.
- It presents in two main histological forms: embryonal and alveolar.
- Retro-auricular location is an exceptionally rare presentation for pediatric RMS.
Observation:
- Two infants diagnosed with left retro-auricular RMS at a very early postnatal stage were treated.
- Management involved multi-step surgical resection and eight chemotherapy cycles.
- Both patients were followed for over six years post-diagnosis and remained recurrence-free.
Findings:
- Complete disease control was achieved in both pediatric patients.
- Early diagnosis and the specific anatomical location contributed to a favorable prognosis.
- Treatment protocols integrating surgery and chemotherapy were effective.
Implications:
- This case series highlights the importance of considering RMS in infants with unusual head and neck presentations.
- Prompt diagnosis via imaging (CT/MRI) and biopsy is crucial for effective management.
- Aggressive treatment strategies can lead to excellent outcomes even in rare RMS locations.
Introduction:
Rhabdomyosarcoma (RMS) arises from mesenchymal cells committed to skeletal muscle. It is the most frequent soft-tissue sarcoma in children and makes up 5% of all pediatric malignant tumors. In this population, there are two main histological forms, which are the embryonal or the alveolar RMS. The retro auricular location is extremely rare. We present 2 cases of children with left retro-auricular RMS occurring at a very early stage of post natal development.
Case Reports:
Two children were included in the RMS 2005 Protocol. The first child, aged 5-days, was managed by surgical resection in two steps after a previous incomplete resection which was followed by 8 chemotherapy cycle. The second, aged 3-days, was managed by surgical resection of the tumor bed, completed by 8 chemotherapy cycle. On regular follow up for over 6 years after the diagnosis, both patients are free of recurrence.
Discussion:
RMS is a fast-growing malignant and aggressive tumor originating from skeletal muscle. It occurs in the first decade of life and is associated with genetic conditions. With an unusual clinical presentation and anatomical location, both benign and malignant tumors can be suspected. Diagnosis can be performed with CT-scan or MRI and confirmed by biopsy. The treatment is based on chemotherapy followed by radiotherapy or surgical resection. In our two patients, the treatment program achieved complete disease control, with good prognosis especially because of anatomical location as well as early diagnosis.
