Three-dimensional modelling and printing for preoperative planning in the resection of an ischiorectal epithelioid sarcoma: a case report

Introduction

Epithelioid sarcomas are rare malignant mesenchymal tumours, accounting for approximately 3–4 % of pediatric soft-tissue sarcomas, and present unique surgical challenges due to their infiltrative nature and proximity to critical anatomical structures. While three-dimensional modelling has emerged as a valuable adjunct in preoperative planning for complex tumours, its use in pediatric soft-tissue sarcomas is rare, with no described cases involving epithelioid sarcomas.

Case presentation

A 13-year-old girl presented with a year-long history of perineal pain and a palpable mass. Imaging revealed a tumour involving the rectum and external sphincter, abutting the posterior vaginal wall. Biopsy confirmed epithelioid sarcoma and staging showed indeterminate pulmonary nodules. She underwent neoadjuvant chemoradiotherapy. Two surgical options were considered: abdominoperineal resection with hysterectomy or vaginal preservation with increased risk of residual disease. A three-dimensional model with 2cm margins was created to aid decision-making. After multidisciplinary review and model consultation, the patient opted for vaginal preservation. She underwent abdominoperineal resection with VRAM flap reconstruction. Final pathology confirmed negative margins. Nearly three years later, she developed a pancreatic tail metastasis and progressive pulmonary lesions, which were treated with distal pancreatectomy, splenectomy, radiotherapy, and cryoablation. Additional metastases in the stomach, scalp, retroperitoneum, and flank are being managed with palliative radiotherapy.

Conclusion

Three-dimensional printed models can be helpful in the preoperative planning for the resection of tumours of the ischiorectal region.