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Orbital rhabdomyosarcoma: Clinicopathologic correlation, management and follow-up in two newborns. A preliminary
Barbara Spahn1, Maja Nenadov-Beck
1Department of Ophthalmology, Hôpital Jules Gonin, Lausanne, 1004, Switzerland. barbara.spahn@ophtal.vd.ch
Insights
Orbital rhabdomyosarcoma (RMS) is rare in newborns. This study details two cases of infant orbital RMS, highlighting challenges in diagnosis and treatment, including chemotherapy resistance and the need for radiotherapy.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Radiology
Background:
- Rhabdomyosarcoma (RMS) is the most common primary malignant orbital tumor in children, typically diagnosed around age seven.
- Orbital rhabdomyosarcoma in newborns is exceptionally rare, presenting unique diagnostic and therapeutic challenges.
Purpose of the Study:
- To present the clinical and radiological findings of two newborns diagnosed with orbital rhabdomyosarcoma.
- To review the literature on infantile orbital rhabdomyosarcoma.
- To discuss the therapeutic approach and outcomes in these rare cases.
Main Methods:
- Case presentation of two newborns with histopathologically confirmed orbital rhabdomyosarcoma.
- Clinical and radiological evaluation including CT-scan and MRI.
- Histological confirmation via open sky biopsy.
- Literature review on infantile orbital rhabdomyosarcoma.
Main Results:
- Both infants presented with unilateral proptosis as the sole initial symptom.
- Chemotherapy resulted in incomplete tumor resolution in both cases after 9-11 months.
- Radiotherapy was administered due to incomplete response to chemotherapy and parental refusal of exenteration.
Conclusions:
- Orbital rhabdomyosarcoma in newborns is a rare entity requiring prompt diagnosis and tailored treatment strategies.
- Chemotherapy may show limited efficacy in very young patients, necessitating consideration of radiotherapy.
- Multidisciplinary management is crucial for optimizing outcomes in infantile orbital rhabdomyosarcoma.
Abstract:
Rhabdomyosarcoma (RMS) is the most common primary malignant tumor of the orbit in childhood. The average age of onset is around seven years. In the present paper, the authors present the clinical and radiological findings in two newborns with histopathologically confirmed orbital rhabdomyosarcoma. A review of the literature was also carried out. In both children, the first sign leading to a visit was a unilateral proptosis, without symptoms or any other sign. Both underwent a CT-scan and an MRI. Confirmation of the histology was made by an open sky biopsy. The children were first treated by chemotherapy but had incomplete resolution of the tumor after more than 11 months for the first case and 9 months for the second. Since the parents of both children refused exenteration, radiotherapy had to be resorted to because of the incomplete response to chemotherapy. Our therapeutic approach and the outcome of these two cases are discussed.
