Abstract
ABSTRACT Introduction and importance: Rhabdomyosarcoma is the most common pediatric soft tissue sarcoma. It constitutes 4 – 6% of all malignancies in childhood. The botryoid variant is a type of ERMS that is the most common malignancy in the pediatric female genitourinary tract. ERMS of the vagina usually occurs in early childhood, whereas ERMS of the uterus occurs later in age. Since 1959, only 7 cases of uterine botryoid ERMS during infancy have been reported. Case presentation: A 15-months-old girl was admitted to our ED for three reddish, smooth, glistening, polypoidal masses protruding from her vaginal introitus for 9 days. The lesions had a suspicious “grape-like” appearance and tended to come in and out through the vaginal ostium. A pelvic US examination on the patient showed an inhomogeneous aspect of the uterus, which appeared to be occupied and stretched out by an iso-hypoechogenic solid tissue. Additionally, there were some hyperechogenic spots inside as well as evidence of vascular signals on color doppler. A surgical biopsy revealed a malignant mesenchymal tumor consistent with the diagnosis of embryonal rhabdomyosarcoma (ERMS), botryoid variant. Clinical Discussion: The patient completed her first cycle of chemotherapy with ifosfamide, vincristine, and dactinomycin (IVA). When she was 18 months old, she underwent a hysterectomy, which removed the entire mass with a Pfannenstiel approach. The postoperative course presented with no complications. Follow-up visits were scheduled quarterly. Conclusion: Botryoid ERMS of the uterus is a diagnosis not to be missed in an infant. Since rapid diagnosis and treatment are necessary, clinicians should be aware of its presentation to assure optimal management. Highlights:
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