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Published on: August 2, 2024
The Giant Infantile Fibrosarcoma of Fetal Oropharynx and Anterior Neck
Tugba Sarac Sıvrıkoz1, Lutfiye Selcuk Uygu1, Çiğdem Kunt İşgüder1
1Department of Obstetrics and Gynecology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
Insights
Infantile fibrosarcoma (IFS) can occur in the prenatal period. An EXIT procedure facilitated early diagnosis and treatment of a giant oropharyngeal IFS.
Area of Science:
- Pediatric Oncology
- Fetal Surgery
- Neonatal Care
Background:
- Infantile fibrosarcoma (IFS) is a rare soft tissue tumor typically presenting in infants within the first year of life.
- Oropharyngeal and prenatal presentations of IFS are exceptionally uncommon.
- Early detection and intervention are crucial for managing neonatal airway obstruction.
Observation:
- A giant solid mass was identified in the fetal oropharynx and anterior neck at 24 weeks gestation via ultrasound and fetal MRI.
- An EXIT (Ex-utero intrapartum treatment) procedure was successfully performed for intrapartum intubation and stabilization.
- Postpartum diagnosis confirmed infantile fibrosarcoma, with the lesion responding to neoadjuvant chemotherapy.
Findings:
- Infantile fibrosarcoma can manifest as early as 24 weeks of gestation.
- The EXIT procedure enabled immediate postnatal management and diagnosis.
- Neoadjuvant chemotherapy proved effective in treating the diagnosed infantile fibrosarcoma.
Implications:
- This case highlights the possibility of early-onset IFS, even in the prenatal period.
- The successful application of an EXIT procedure demonstrates its value in managing complex fetal neck masses.
- Early diagnosis and multimodal treatment strategies, including chemotherapy, are vital for improving outcomes in rare infantile fibrosarcoma cases.
Abstract:
Introduction: Infantile fibrosarcoma (IFS) usually arises in the extremities during the first 12 months of life and responds well to surgery. It is unusual in the oropharynx or the prenatal period. Case report: A giant solid mass was first detected in the oropharynx and anterior neck at 24 weeks of gestation by ultrasound and fetal MRI. An EXIT procedure with intrapartum intubation with appropriate supportive therapy was successful. The diagnosis of IFS was made postpartum, and the lesion responded to neoadjuvant chemotherapy. Conclusion: IFS may arise as early as 24 weeks of gestation. In this case, an EXIT procedure allowed postpartum diagnosis with subsequent treatment.
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