Ovarian Rhabdomyosarcoma in Children

Rhabdomyosarcoma is the most common soft tissue sarcoma in children. It predominantly arises in parameningeal and periorbital regions, as well as in other areas that do not typically contain striated muscle. Ovarian involvement is rare and may be attributed to the presence of ovarian stromal fibroblasts, or endometriotic stroma. Ovaries may be considered a favorable site for rhabdomyosarcoma. The disease typically presents in children under three years of age, often at an advanced stage and with nonspecific symptoms. Management requires a multimodal treatment approach.

This was a retrospective analysis of ovarian rhabdomyosarcoma cases over the past 25 years at a national cancer referral center in Peru.

Six female patients, aged between five months and 13 years, were included in the study. Four of them were classified as clinical stage and group IV due to the presence of distant metastases, while the remaining two were categorized as low-risk. The majority had embryonal histology, and none tested positive for fusion genes. All patients underwent chemotherapy and surgery. Surgical approaches varied according to disease extent, with an emphasis on fertility preservation. These ranged from unilateral adnexectomy with ovarian-sparing staging in cases of suspected localized disease, to primary cytoreduction without hysterectomy or contralateral adnexectomy in cases presenting with severe symptoms due to mass effect from advanced tumors. Chemotherapy regimens were selected based on risk stratification and aligned with international treatment protocols. Three patients received intensity-modulated abdominopelvic radiotherapy at a total dose of 2,400 cGy, delivered in 16 sessions for peritoneal sarcomatosis, two due to persistent disease following chemotherapy and surgery, and one due to high-risk histology. One low-risk patient achieved a survival of up to 94 months.

Ovarian rhabdomyosarcoma is rare. Its clinical and radiological manifestations are nonspecific. The use of immunohistochemistry is essential for diagnosis. Advanced disease with embryonal histology is predominantly observed. Multimodal treatment has achieved survival exceeding 87 months even in cases of metastatic stages.