Upper Esophageal Fetal Rhabdomyoma: A Case Series of 4 Children and Review of Literature.

Objective

The aims of the present study were to provide objective evidence for identifying fetal rhabdomyoma (RM) in the upper esophagus of children, enhance clinical understanding of the diagnosis and treatment of this condition, and optimize the treatment strategy for fetal RM.

Methods

The clinical medical records of 4 children with upper esophageal fetal RM were retrospectively collected, and were admitted to the Department of Otolaryngology, Head and Neck Surgery at our hospital between July 2016 and July 2022. Their clinical, histological, and therapeutic characteristics were analyzed in combination with the literature.

Results

Four children diagnosed with upper esophageal tumors were included and all of them underwent resection of the upper esophageal tumor with esophageal-pharyngeal reconstruction, and 2 of them underwent prophylactic tracheotomy due to recurrent laryngeal nerve adhesion. Preoperative biopsy was performed in 2 cases (case 2 and case 4), while intraoperative frozen section analysis was conducted in the other 2 (case 1 and case 3), with pathological results consistent with fetal RM. Patients were followed up for 25 to 96 months after the surgery. So far, only 1 patient has experienced a recurrence of fetal RM and underwent a second surgical resection to remove the tumor.

Conclusion

Fetal RM is a benign tumor prone to recurrence, and complete excision is the preferred optimal treatment. Clinicians need to understand and master the management algorithm for fetal RM to standardize its diagnosis and treatment.