Ovarian Rhabdomyosarcoma in Children.

Area of Science:

• Pediatric Oncology
• Surgical Oncology
• Gynecologic Oncology

Background:

• Rhabdomyosarcoma is the most common pediatric soft tissue sarcoma.
• Ovarian involvement is rare, potentially arising from stromal or endometriotic tissue.
• Often presents in young children with nonspecific symptoms and advanced stage.

Purpose of the Study:

• To analyze clinical characteristics and treatment outcomes of ovarian rhabdomyosarcoma.
• To evaluate the efficacy of multimodal treatment in pediatric patients.
• To highlight the importance of fertility-sparing approaches when feasible.

Main Methods:

• Retrospective analysis of ovarian rhabdomyosarcoma cases over 25 years.
• Inclusion of six female patients aged five months to 13 years.
• Review of clinical stages, histology, treatment modalities (chemotherapy, surgery, radiotherapy), and outcomes.

Main Results:

• Four patients presented with stage IV metastatic disease; two were low-risk.
• Embryonal histology was predominant; no fusion genes were detected.
• Multimodal treatment, including surgery and chemotherapy, was administered to all patients. Radiotherapy was used in select cases.

Conclusions:

• Ovarian rhabdomyosarcoma is a rare entity with nonspecific presentation.
• Immunohistochemistry is crucial for accurate diagnosis.
• Multimodal therapy demonstrates significant survival benefits, exceeding 87 months even in metastatic cases.