Sarcoma Botryoides: Optimal Therapeutic Management and Prognosis of an Unfavorable Malignant Neoplasm of Female Children

Embryonal rhabdomyosarcoma (ERMS) is a rare malignancy and occurs primarily in the first two decades of life. Botryoid rhabdomyosarcoma is an aggressive subtype of ERMS that often manifests in the genital tract of female infants and children. Due to its rarity, the optimal treatment approach has been a matter of debate. We conducted a search in the PubMed database and supplemented it with a manual search to retrieve additional papers eligible for inclusion. We retrieved 13 case reports and case series, from which we summarized that the current trend is to approach each patient with a personalized treatment plan. This consists of a combination of local debulking surgery and adjuvant or neoadjuvant chemotherapy (NACT). Effort is made in every approach to avoid radiation for the sake of preserving fertility. Radical surgeries and radiation still have a role to play in extensive disease and in cases of relapse. Despite the rarity and aggressiveness of this tumor, disease-free survival and overall prognosis is excellent, especially when it is diagnosed early, compared with other subtypes of rhabdomyosarcoma (RMS). We conclude that the practice of a multidisciplinary approach is appropriate, with favorable outcomes; however, larger-scale studies need to be organized to have a definite consensus on optimal management.

Automated summary

Embryonal rhabdomyosarcoma (ERMS) is a rare malignancy primarily affecting individuals in the first two decades of life. Botryoid rhabdomyosarcoma, an aggressive subtype of ERMS, commonly presents in the genital tract of female infants and children. Treatment typically involves personalized approaches, combining local debulking surgery with adjuvant or neoadjuvant chemotherapy, while effort is made to preserve fertility and avoid radiation. Radical surgeries and radiation may still be necessary in cases of extensive disease or relapse. Despite its rarity and aggressiveness, ERMS has excellent disease-free survival and overall prognosis, especially when diagnosed early. A multidisciplinary approach is deemed appropriate for optimal management, but larger-scale studies are needed to establish a consensus.