Introduction Sebaceous carcinomas arising from ovarian dermoid cysts are exceedingly rare. We present a review of the literature and add a case of our own, which is to date the only case identified in Singapore’s largest women’s hospital.

Methods PubMed and Embase electronic databases were searched systematically on 30 April 2024. Keywords used were sebaceous carcinoma, ovary, and dermoid cyst. This revealed 15 other cases of ovarian sebaceous carcinoma arising from dermoid cysts, excluding ours.

Results We present the case of a 60-year-old Chinese woman who presented with lower abdominal pain and a large pelvic mass. She underwent total abdominal hysterectomy, bilateral salpingo-oophorectomy, pelvic and para-aortic lymph node dissection, infragastric omentectomy and peritoneal washings. Histology showed an ovarian carcinoma with a population of cells showing ample vacuolated cytoplasm indicating sebaceous differentiation. Postoperative recovery was uneventful and she remained disease-free after 20-months. The mean age was 57.9±13.5 years. FIGO stage at diagnosis ranged from IA to IIIC, with majority presenting with early stage disease with unilateral tumours. Mean gross tumour size was 17.6±6.70cm. Salpingo-oophorectomy was performed in all cases, hysterectomy in 87.5%, and staging or debulking surgery in 12.5%. 12.5% of patients underwent chemotherapy. Median disease-free interval was 19.5 (IQR: 4.75-33) months. One case recurred 5 months post-surgery. Loss of MMR protein expression was identified in 3 cases. Findings from our literature review are summarised in table 1.

Conclusion/Implications Clinical behaviour and optimal management strategies for sebaceous carcinoma arising from dermoid cysts remain poorly defined. More data is needed to better manage this rare phenomenon.